Contacted Date |
About Contact |
11/22/2017 12:00:00 AM | Contact Name : | Paul Witter |
Email ID : | internalmedicine@annualcongress.net | Subject : | International Conference on Internal Medicine |
Message : | https://annualmeeting.conferenceseries.com/internalmedicine/
International Conference on Internal Medicine will be held at Osaka during May 21-22, 2018. The Internal Medicine 2018 will be a 2 days Event which will includes Keynote Speeches, Academic Session Speakers, Poster Presentation, Workshop, Young Research forum, Exhibition, Symposiums and Business Marketing. Meet Global Medical and Diagnostic specialists around the world at Internal Medicine Conference 2018 Osaka, Japan. |
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11/27/2017 12:00:00 AM | Contact Name : | Tuncay Aydemir |
Email ID : | info@iarsp.org | Subject : | Add an ISSN |
Message : | "International Journal of Social And Humanities Sciences" ISSN: 2602-3288
http://olddrji.lbp.world/IndexingCertificate.aspx?jid=3526
URL: http://dergipark.gov.tr/ijshs |
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3/21/2018 12:00:00 AM | Contact Name : | Marioper |
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4/27/2018 12:00:00 AM | Contact Name : | Martintex |
Email ID : | martinOweft@bestmail.club | Subject : | 392 Wow!! Hi! |
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5/7/2018 12:00:00 AM | Contact Name : | Hasan Yaylı |
Email ID : | koopkur@gmail.com | Subject : | 2587-0114 indexed journal |
Message : | Please send me login information again to koopkur@gmail.com |
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11/19/2017 12:00:00 AM | Contact Name : | Augustine Isaac |
Email ID : | hematologistsmeet@annualcongress.net | Subject : | Hematologists Global Summit 2018 |
Message : | Greetings from the Hematologists Summit 2018
After the effective wander of Hematology Gatherings in various parts of the world, Conference Series Ltd is overpowered to report the Hematologists Global Summit 2018 will be held during July 13-14, 2018, Sydney, Australia. . And this conference is going to be arranged around the theme "An advanced approach to blue pencil the risks in hematological disorders”. This includes prompt keynote presentations, Oral talks, Poster presentations, and Exhibitions. it also Invites immunologists, pathologists, hematologists, oncologists, inquires about researchers, modern experts and understudy delegates from biomedical and human services areas to be a piece of it.
Why to attend???
To look at moves in clinical and translational hematologic research, to review the bit of new precise techniques and accommodating strategies as related to the care of association of cases identified with blood conditions. Talk about new patient administration and care systems with leading personnel in the field. Conferences share loads of information to Universities, companies. It is beneficial to medical students and researchers Furthermore for professors and science laboratory technicians. Conferences additionally benefit and share information between scientists, doctors, and delegates. One company networks with a different company and that they share information with each other regarding their product at the conference. The companies return to attend conferences and show their exhibit before of eminent members and delegates present at the Conference.
Conference Highlights
• Hematology and hematologists
• Blood and blood disorders
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• Bone marrow transplantation
• Blood transfusion
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• Advanced treatment and research in hematology
• Hematology oncology
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• Hematology veterinary
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Importance and Scope
Hematologists Global Summit 2018 relied upon to offer the best stage for the ability, specialists, understudies and business delegates with its efficient logical program covering each moment insight about hematology and blood sicknesses, from the fundamental causes behind blood ailments to the most recent patterns in indicative and treatment of blood illnesses. This global occasion is a push to discover a weapon against incessant clutters like blood growth, Sickle cell paleness, hemophilia, lymphoma and myeloma and to examine the part of Immunoglobulin in blood, counter acting agent and antigen responses, the red platelet and the immunological properties of blood which gives a superior knowledge about the related causes and infection portfolio.
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7/1/2018 12:00:00 AM | Contact Name : | r-z-r.ru |
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9/14/2018 12:00:00 AM | Contact Name : | swizil |
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9/23/2018 12:00:00 AM | Contact Name : | Waynegairm |
Email ID : | antonio@vzrxr.ru | Subject : | Моторы |
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11/23/2017 12:00:00 AM | Contact Name : | Svitlana |
Email ID : | editor@iomining.in.ua | Subject : | Edit |
Message : | Dear Sir or Madam
I am writing to inform you that you have registered our journal under the incorrect title.
Please go by the link
http://olddrji.lbp.world/JournalProfile.aspx?jid=2306-5435
I should be grateful if you would kindly fix the journal title on "Mining Journal".
Apart from, I enclose the corrected text of Journal Description
"The Scientific and technical collection is included in the list of professional publications. In this collection are published the results of dissertations on the competition of scientific degrees of doctors and candidates of Technical Sciences (The Order of the Ministry of Education and Science of Ukraine № 455 of 15.04.2014). The collection has gone to the National Library of Ukraine named after V.L. Vernadsky and the leading scientific libraries of Ukraine. The collection is indexed in the national database “Ukrainika naukova” (journal “Dzherelo”). The collection presents the results of research in the field of Technical Sciences, the ways to increase the efficiency of industrial production, automation control and control of technological processes. Much information is devoted to the issues such as Energy conservation, Reliability of the occupational health, Labour safety and Protection of the environment. This collection is designed for the benefit of sceintists and engineers and can also be used by the students of the higher educational institutions. The main purpose of the Mining journal of Kryvyi Rih National University is to shed light on the modern scientific research and the results of the main actions of research activity in regards to the entire scientific community."
Best regards,
Editorial Board of the Mining Journal
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Reply : | Reply to this Complaint. |
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11/28/2017 12:00:00 AM | Contact Name : | Dr Pramod |
Email ID : | editorbjpmr@gmail.com | Subject : | Indexing of two journals |
Message : | Dear Sir,
I am editor in chief of two journal (bjpmr.org and bjbmr.org) .
i want to index my journal in your database
plz provide further info. |
Reply : | Reply to this Complaint. |
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11/29/2017 12:00:00 AM | Contact Name : | Vladimir Njegomir |
Email ID : | vnjegomir@civitas.rs | Subject : | CIVITAS journal indexing |
Message : | Dear Sir or Madam,
I would like if CIVITAS journal is indexed or not. Last year I have got DRJI certificate but this year I could not find it among index journals. I entered data again and now it is in "under evaluation". However, when I try to search for CIVITAS, I got screen on which is CIVITAS and certificate. Also, I do not have publisher login. I understand that I should add some more information as Editor-in -Chief. Please help me, I would love if CIVITAS is indexed on DRJI.
Kind regards,
Vladimir Njegomir
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Reply : | Reply to this Complaint. |
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12/22/2017 12:00:00 AM | Contact Name : | Erminio Fonzo |
Email ID : | efonzo@unisa.it | Subject : | agreement pending and logo |
Message : | I submitted two journals, ''Journal of Mediterranean Knowledge'' and ''ICSR Mediterranean Knowledge Working Papers Series''. Both of them have been indexed, but at present they are in the section "agreement pending".
In what way the agreement can be concluded?
Another question: is it possible to get a DRJI logo and display it in the two indexed journals?
Best regards,
Erminio Fonzo |
Reply : | Reply to this Complaint. |
|
9/24/2018 12:00:00 AM | Contact Name : | Charlesevift |
Email ID : | abakumov.19803@gmail.com | Subject : | Test, just a test |
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10/6/2018 12:00:00 AM | Contact Name : | XRumerTest |
Email ID : | yourmail@gmail.com | Subject : | Test, just a test |
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11/28/2017 12:00:00 AM | Contact Name : | Evangelina Claire |
Email ID : | info@heraldsopenaccess.us | Subject : | Reg: Indexing |
Message : | We have submitted 5 Journals for indexing, but only one is indexed and we cannot find the remaining 4 journals. We neither found them in under evaluation nor in indexed. Can we know the reason? |
Reply : | Reply to this Complaint. |
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12/7/2017 12:00:00 AM | Contact Name : | Iryna Tkachuk |
Email ID : | tkachukiryna1944@ukr.net | Subject : | About submission articles |
Message : | Would you be so kind to inform us how we can submit articles of our journal to evaluation
ISSN No. : 2518-7589
Journal Title : THE ACTUAL PROBLEMS OF REGIONAL ECONOMY DEVELOPMENT
Journal URL : http://journals.pu.if.ua/index.php/aprde/
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12/12/2017 12:00:00 AM | Contact Name : | Svitlana |
Email ID : | editor@iomining.in.ua | Subject : | Edit |
Message : | Hi
I am a new admin of the MINING JOURNAL OF KNU (http://iomining.in.ua)
Please provide access to the log page in your science-based database.
(http://olddrji.lbp.world/JournalProfile.aspx?jid=2306-5435)
or change as quickly as possible from "???????? ??????" to "Mining journalof Kryvyi Rih National University"
best regards, Svitlana |
Reply : | Reply to this Complaint. |
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1/2/2018 12:00:00 AM | Contact Name : | Prof. Dr. Samina Malik |
Email ID : | editor.ijitl@iiu.edu.pk | Subject : | Status of Evaluation of Research Journal |
Message : | what is status of indexing of Journal "International Journal of Innovation in Teaching and Learning (IJITL)"
Request form was submitted on 22/3/17 via http://olddrji.lbp.world/SuggestJournal.aspx
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Reply : | Reply to this Complaint. |
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1/10/2018 12:00:00 AM | Contact Name : | Mehmet Ali ALTINEL |
Email ID : | mehmetali.altinel@altinbas.edu.tr | Subject : | Journal title and detailed information don''t match |
Message : | Hello,
We are very glad to see our journal in this index but there is a problem. When you click on AURUM Altınbaş University Journal of Social Sciences, you will see another journal''s information. I hope this situation will be fixed as soon as possible
Best Regards
Mehmet Ali ALTINEL |
Reply : | Reply to this Complaint. |
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1/15/2018 12:00:00 AM | Contact Name : | Elza Margarida Peixoto |
Email ID : | revistagerminalufba@gmail.com | Subject : | Correction of the email |
Message : | Last week I submitted the journal "Germinal: Marxism and Education in Debate" to be indexed, but I informed the wrong e-mail. I tried again to submit, but the system did not allow. The correct email is: revistagerminalufba@gmail.com |
Reply : | Reply to this Complaint. |
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1/21/2018 12:00:00 AM | Contact Name : | Dr.Zahid Yaseen |
Email ID : | editor.orjss@gmail.com | Subject : | request about citation of our journal |
Message : | honorable we have start a journal in this matter indexing agencies citation is compulsory pls help us in this matter. |
Reply : | Reply to this Complaint. |
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1/26/2018 12:00:00 AM | Contact Name : | Juan Carlos Viloria Doria |
Email ID : | juancjcvd@gmail.com | Subject : | Submit Journal - Salud Uninorte |
Message : | I have recently completed the application form. We would like to know, how much time takes the evaluation process to be part of this important Directory of Research Journals Indexing.
Best Regards,
Juan Carlos Viloria Doria
Editorial Assistant
Salud Uninorte
http://rcientificas.uninorte.edu.co/index.php/salud/index |
Reply : | Reply to this Complaint. |
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1/29/2018 12:00:00 AM | Contact Name : | Clara Duarte Cuervo |
Email ID : | editorial@tocolombia.org | Subject : | Please change the country of our submission |
Message : | Hi. I am the chief editor of Revista Ocupación Human (Human Occupation Journal). I have made a mistake filling out the submit journal form. Is it possible to change the country of the journal? Our journal is from Colombia, not from Afghanistan.
Thank you so much, please forget my mistake.
Kind regards,
Clara Duarte Cuervo |
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12/13/2017 12:00:00 AM | Contact Name : | Sara Wallner |
Email ID : | iimmunology@eurosciconmeetings.com | Subject : | Event Listning |
Message : | Dear Sir/Madam,
Kindly add below conference.
Name: Immunology 2018
Date: July 05-07, 2018
URL: http://immunology.euroscicon.com/
Email : immunology@eurosciconconferences.com | immunology@eurosciconmeetings.com
Location : Vienna, Austria
About Conference : The Euroscicon is inviting for 15th International Conference and exhibition on Immunology 2018 on July 05 - 07, 2018 Vienna, Austria. The theme of this year’s meeting is Spreading the new trends in Immunology which will provide an international platform for discussion of present and future challenges in immunological techniques, future of immunology. World-leading health practitioners, educators and researchers will present cutting-edge and practical immunological techniques based upon widely accepted evidence and will introduce new and emerging research.
Thank you,
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1/16/2018 12:00:00 AM | Contact Name : | ProFunder365 |
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1/24/2018 12:00:00 AM | Contact Name : | Margaret Clark |
Email ID : | info@heraldsopenaccess.us | Subject : | Reg; Journal Submission |
Message : | I have submitted a Journal Journal of Community Medicine & Public Health Care. But it is not shown any where and also not appearing in the submitted ones. Previously I complained regarding the same but not received any information. Hope to receive an answer this time. |
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1/30/2018 12:00:00 AM | Contact Name : | Baboo Ali |
Email ID : | babooali@comu.edu.tr | Subject : | Activation Link of Journal |
Message : | Dear Sir/Madam,
I have entered the basic information of our journal namely, "COMU Journal of
Agriculture Faculty" (COMUJAF) with ISSN No. 2564-6826 for evaluation in DRJI. I tried to publish
the previous issues of our journal into the database system of DRJI but the given link is
not active. Could you please help me in this regard and provide me an active link of our journal so that I would be able to update the page of the journal on DRJI?
Thanks for your precious time and cooperations in advance.
Yours'' Truly,
Dr. Baboo Ali
Canakkale Onsekiz Mart University
Faculty of Agriculture
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1/16/2018 12:00:00 AM | Contact Name : | Mohammad ali Morowatisharifabad |
Email ID : | morowatisharif@yahoo.com | Subject : | Duplicate submission of a Journal |
Message : | Dear DRJI team. I would like to inform you that our journal (ELDERLY HEALTH JOURNAL) is submitted to DRJI twice with the names of (ELDERLY HEALTH ) and (ELDERLY HEALTH JOURNAL). I want to ask you to remove the (ELDERLY HEALTH ) one. |
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1/22/2018 12:00:00 AM | Contact Name : | osama yusuf ababneh |
Email ID : | editoroffice@refaad.com | Subject : | indexed journal |
Message : | Dear Sir
we have been indexed at Drji for three journals published by us since more than one year but we cant find any of them when we try to check that from Drji website , however the journals name are:
1> General Letters in Mathematics
2> Global Journal of Economic and Business
3> International Journal of Educational Psychological Studies
looking to hear from you soon
regards |
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2/1/2018 12:00:00 AM | Contact Name : | Abid Ali |
Email ID : | abid.ali@umt.edu.pk | Subject : | Input Metadata of Journal''s articles |
Message : | Dear Sir
I had put the information about the journals. Now tell/guide me about metadata of journal articles input way.
Regards
Abid Ali |
Reply : | Reply to this Complaint. |
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2/13/2018 12:00:00 AM | Contact Name : | Salim Serkan GÜÇLÜ |
Email ID : | salimguclu@sdu.edu.tr | Subject : | Süleyman Demirel University Journal of Egirdir Fisheries Faculty (SDU-JEFF) |
Message : | DRJI Editor Team,
We applied for the index on 16.06.2017. Can we get information about whether our journal (e-ISSN 1308-7517- Süleyman Demirel University Journal of Egirdir Fisheries Faculty (SDU-JEFF) http://dergipark.gov.tr/egirdir) is accepted? Will you give us information on this?
Best regards.
Dr.Salim Serkan GÜÇLÜ
SDU-JEFF Copy and Layout Editor
------------------------------------
Süleyman Demirel University Eastern Campus
Eğirdir Fisheries Faculty Inland Water Biology Department ISPARTA-TURKEY |
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2/28/2018 12:00:00 AM | Contact Name : | Ulku KARAMAN |
Email ID : | ulkukaraman44@hotmail.com | Subject : | Editing journal information |
Message : | What do we need to do to update the information about our Journal?
Best regards.
Journal Title : Middle Black Sea Journal of Health Science
Journal URL : http://dergipark.gov.tr/mbsjohs
Ulku Karaman PhD
Ordu University |
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Reply : | Reply to this Complaint. |
|
1/27/2018 12:00:00 AM | Contact Name : | Dr. Imran Kazmi |
Email ID : | editor.ijpp@edwiserinternational.com | Subject : | My Journal is in Agreement Pending: What it means? |
Message : | Dear DRJI Officials,
Greetings.
Myself is Dr. Imran Kazmi, Editor-in-Chief of International Journal of Pharmaceutics & Pharmacology published by Edwiser International.
We have applied for indexing in your prestigious indexing service. Now my journal is showing up in Agreement Pending Tab. What is an Agreement Pending means? and how it can be resolved.
Apart from that there is one typographical error in our ISSN- our actual ISSN is 2581-3080. But due to some typo error it is showing ISSN-2158-3080 in your database. We will be glad if you could fix this typo-error.
We are looking forward to hearing from you.
Regards,
Dr. Imran Kazmi
Editor-in-Chief |
Reply : | Reply to this Complaint. |
|
1/29/2018 12:00:00 AM | Contact Name : | Clara Duarte Cuervo |
Email ID : | editorial@tocolombia.org | Subject : | Please change the country of our submission |
Message : | Hi. I am the chief editor of Revista Ocupación Human (Human Occupation Journal). I have made a mistake filling out the submit journal form. Is it possible to change the country of the journal? Our journal is from Colombia, not from Afghanistan.
Thank you so much, please forget my mistake.
Kind regards,
Clara Duarte Cuervo |
Reply : | Reply to this Complaint. |
|
1/31/2018 12:00:00 AM | Contact Name : | Dr Ramesh Kumar Miryala |
Email ID : | miryalaramesh@gmail.com | Subject : | Submission of Journal |
Message : | Dear Sir / Madam,
Kindly let me know, how I can submit my Journal for consideration to include in the Directory.
Dr Ramesh Kumar Miryala |
Reply : | Reply to this Complaint. |
|
2/1/2018 12:00:00 AM | Contact Name : | Ersin KORKMAZ |
Email ID : | ersin_korkmaz1@hotmail.com | Subject : | Submitting a journal for indexing |
Message : | Dear Mr. or Mrs.
I have submitted our journal named International Journal of Engineering Research and Development to your index. ISSN number of our journal is 1308-5514. However, there is no information about the process of our journal. How can ı take any information about it? If there is a problem about submitting, Should ı resubmit for our journal ? I hope ı can take any information as soon as possible. Thank you for your interest.
Best regards.
Research Assist. Ersin KORKMAZ
Kirikkale University
Engineering Faculty
Civil Engineering Department
Kirikkale/TURKEY |
Reply : | Reply to this Complaint. |
|
2/6/2018 12:00:00 AM | Contact Name : | Elza Margarida Peixoto |
Email ID : | revistagerminalufba@gmail.com | Subject : | Correction of the country |
Message : | Dear Editors,
Please, I solicit correction the name of the country. My country is Brazil, and not Afghanistan.
Best regards,
Elza M. Peixoto |
Reply : | Reply to this Complaint. |
|
2/9/2018 12:00:00 AM | Contact Name : | dr devanand gupta |
Email ID : | drdevanandgupta@aol.com | Subject : | query |
Message : | our journal was indexed in your esteem database previously- but we cannot locate it now. please help -
International Journal of Contemporary Medical Research
https://www.ijcmr.com |
Reply : | Reply to this Complaint. |
|
3/9/2018 12:00:00 AM | Contact Name : | Kennethtrobe |
Email ID : | be.s.t.@seobest.website | Subject : | Природный активатор счастья |
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Сайт: http://doctorslivedigest.com/I4jQy/ |
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3/9/2018 12:00:00 AM | Contact Name : | Dr. Binod Kumar |
Email ID : | binod.istar.1970@gmail.com | Subject : | To update URL of Journal website |
Message : | Dear sir
Our Journal has been Indexed by SIS Scientific Group. We have changed URL of Our Journal . Please update this in your database.
Journal Name: International Journal of Research in Computer Science and Management [ISSN (P): 2321-8088, ISSN (E):2455-5665]
Old URL: http://www.ijrcsm.com
New URL: http://www.ijrcsm.co.in |
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4/8/2019 12:00:00 AM | Contact Name : | WilliamRes |
Email ID : | lindacolleen@yahoo.com | Subject : | You win iphone X - Apple Community |
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4/22/2019 12:00:00 AM | Contact Name : | Michaelstins |
Email ID : | temptest876088736@gmail.com | Subject : | 301 Moved Permanently |
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1/30/2018 12:00:00 AM | Contact Name : | ProFunding247 |
Email ID : | noreply@profunding247.com | Subject : | RE: Capital for Your Business |
Message : | Hi, letting you know that http://ProFunding247.com can find your business a SBA or private loan for $2,000 - $350K Without high credit or collateral.
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2/5/2018 12:00:00 AM | Contact Name : | Juliana Lima |
Email ID : | juliana.lima@ufc.br | Subject : | Publisher Login |
Message : | Dear Editor,
The journal "Revista de Psicologia" is already indexed at DRJI but we don''t have receive the publisher login and password yet.
http://olddrji.lbp.world/JournalProfile.aspx?jid=2179-1740
Kind Regards,
Juliana Lima
Librarian |
Reply : | Reply to this Complaint. |
|
2/5/2018 12:00:00 AM | Contact Name : | Juliana Lima |
Email ID : | revistadeletras.ufc@gmail.com | Subject : | Publisher Login |
Message : | Dear Editor,
The journal "Revista de Letras" is already indexed at DRJI but we don''t have receive the publisher login and password yet.
http://olddrji.lbp.world/JournalProfile.aspx?jid=2358-4793
Kind Regards,
Juliana Lima |
Reply : | Reply to this Complaint. |
|
2/9/2018 12:00:00 AM | Contact Name : | LisaKent@explainmybusiness.com |
Email ID : | LisaKent@explainmybusiness.com | Subject : | Promoting Your Business |
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3/10/2018 12:00:00 AM | Contact Name : | Susanbrino |
Email ID : | bes.t@seobest.website | Subject : | Заявка на кредит в ОТП Банк |
Message : | Кредит наличными в ОТП Банке: http://workle.website/71 |
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|
3/10/2018 12:00:00 AM | Contact Name : | Boby Thomas |
Email ID : | pesquisajournal@gmail.com | Subject : | Indexing of Journal |
Message : | Pesquisa with url: www.pesquisaonline.net and with ISSN 2456-4052 (print), ISSN 2456-4052 (online) are still under the category of ''under evaluation''.
Kindly inform us what is required for the inclusion for indexing |
Reply : | Reply to this Complaint. |
|
3/20/2018 12:00:00 AM | Contact Name : | Asst Prof Dr Oluwatoyin Muse Johnson Popoola |
Email ID : | popoola@uum.edu.my | Subject : | Indian-Pacific Journal of Accounting and Finance - Awaiting login details & Certificate of Indexing. |
Message : | The above subject refers. We are yet to receive the login details, namely: username/password and certificate of indexing, if any. Hence, unable to upload articles into your DRJI portal.
Your kind consideration will be highly appreciated.
Thank you. |
Reply : | Reply to this Complaint. |
|
5/11/2019 12:00:00 AM | Contact Name : | GroInity |
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5/13/2019 12:00:00 AM | Contact Name : | Andrewwaype |
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2/28/2018 12:00:00 AM | Contact Name : | ulku karaman |
Email ID : | ulkukaraman44@hotmail.com | Subject : | MIDDLE BLACK SEA JOURNAL OF HEALTH SCIENCE (MBSJHS)" of our journal we are logging in with the Directory of Research Journals Index, it shows different informations |
Message : | Dear authorized,
When we click on the title "MIDDLE BLACK SEA JOURNAL OF HEALTH SCIENCE (MBSJHS)" of our journal we are logging in with the Directory of Research Journals Index, it shows different informations. Only when we click on "http://dergipark.gov.tr/mbsjohs" mail address of thejournal, we can reach our journal''s page.
We wrote the information about our journal in order.
We will be satisfied if the information is arranged as follows.
Yours sincerely
ISSN No : 2149-7796
Journal Title : Middle Black Sea Journal of Health Science
Journal URL : http://dergipark.gov.tr/mbsjohs
Publisher name : Ülkü Karaman
Counrty : Turkey
Language : English
Discipline : İnderdiciplinary
Organization Name : Ordu University
Frequency : quaterly
Start Year : 2005
Journal Description : The journal publishes clinical and experimental studies, interesting case reports, invited reviews and letters to the editor. Middle Black Sea Journal of Health Science is an international journal which is based on independent and unbiased double-blinded peer-review principles. The publishing language of the journal is English. The aim of the journal is to publish original articles with highest clinical and scientific quality at the international level. Middle Black Sea Journal of Health Science also publishes reviews covering fundamental innovations in health education, editorial articles, case reports and original images |
Reply : | Reply to this Complaint. |
|
3/3/2018 12:00:00 AM | Contact Name : | ALiaderginiaro |
Email ID : | raihooveiy@bestmailonline.com | Subject : | How Diabetes Can Affect Sexual Function and Pleasure |
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3/25/2018 12:00:00 AM | Contact Name : | Dr. Sok Udom Deth |
Email ID : | editor@jomeino.com | Subject : | index evaluation |
Message : | Dear Authorized,
We are looking forward to hearing from you a positive answer related to evaluation of our journal (Journal of Management, Economics, and Industrial Organization - http://jomeino.com)
Best Regards,
Editor-in-Chief
Dr. Sok Udom Deth |
Reply : | Reply to this Complaint. |
|
3/30/2018 12:00:00 AM | Contact Name : | Kennethtrobe |
Email ID : | bes.t@seobest.website | Subject : | Eroctive - средство для потенции |
Message : | Как часто Ваша партнёрша кричит в постели и рвёт Вам спину, изнемогая от оргазма?
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4/5/2018 12:00:00 AM | Contact Name : | Tinabal |
Email ID : | tinasmit@bk.ru | Subject : | Рекомендации EASL 2017 по лечению Гепатита B на русском языке |
Message : | Добрый день
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6/23/2019 12:00:00 AM | Contact Name : | AntonioBet |
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7/13/2019 12:00:00 AM | Contact Name : | Prof. Aman Agarwal |
Email ID : | aa@financeindia.org | Subject : | Ref Directory of Research Journals Indexing Impact Factor [ Indexing / Abstracting / Impact Factor] |
Message : | Dear Editors,
This is with reference to our request placed in 2017 for considering to Index and Abstract the refereed quarterly journal of finance - FINANCE INDIA (ISSN : 0970 - 3772 / 1987).
I am happy to share that Finance India is Indexed & Abstracted by
* SCOPUS ; ABDC ; UGC India + 34 Agencies Worldwide
* Finance India is placed at 3rd out of 21,000+ Journals in SJIF Journal Rank Indexed by Scientific Journal Impact Factor (SJIF) with SJIF Impact Factor 2018 = 6.778.
* UGC India (UGC-CARE List 2019 & Code 1972 & 8198 prior to May 2018)
* UGCs in various countries & in over 120+ Universities Worldwide
* FI has been Ranked 3rd Worldwide by American Statistical Association (B & E Data link) based on rating from amongst 79 finance journal / institutions & also amongst 148 journals / institutions (micro economic, macro economic, finance & labour economics).
* International Scientific Indexing (ISI) Indexed with Impact Factor of 1.518 (2017-18)
* Scientific Index Services (SIS) Indexation with SIS Impact Factor of 1.593 (2018)
* ICI World of Journals ICV (Index Copernicus Value) Index Factor 73.22 (2015)
* Finance India is indexed and abstracted by more than 34 agencies Worldwide
We are happy to share with you that FINANCE INDIA (ISSN : 0970 - 3772), The Quarterly Journal of Finance, published since 1987 by Indian Institute of Finance, regularly is a Two Tier Triple Blind Peer Review Journal. Its has an exalted Editorial Board of over 85 Experts from all over World including 6 Nobel Laureates headed by Prof. Dr. JD Agarwal. It is indexed and abstracted by more than 34 agencies worldwide.
You are requested to kindly consider Indexing / Abstracting / calibrating Impact Factor of the journal in your prestigious Indexing & Impact Factor. The brief about the journal is given in the FI Brochure enclosed.
FINANCE INDIA is a Quarterly(refereed) journal of Finance of Indian Institute of Finance, published regularly since 1987. Each issue is of more than 400 pages. Some of the most prominent experts: Franco Modigliani, Merton H. Miller, Bob Merton, Bob Mundell, Douglass North, James Heckman, Rene Sultz, Dick Brealey, Jagdish Bhagwati are on the editorial board of the journal.
The Journal is indexed/abstracted by 34 institutions & organization like Cabells Directory of Publishing opportunities in Accounting, Economics & Finance 1997-98, International Bibliography of Social Sciences of theLondon School of Economics and Political Sciences, EconLit, JEL on CD, e-JEL, The Journal of Economics Literature, ProQuest, Google Scholars, UGC India - [Group A UGC-CARE List 2019 No 75 & earlier as Code 1972 & 8198 till May 2018), UGC, Uzbekistan, Ulrich''s Periodicals DirectoryTM, Gale Directory of Publication and Broadcast Media, EDIRC, FI-ICP, ISID Research Reference of Institute of Studies in Industrial Development, All India Index to Periodical Literature by Nexus Information Services Company (NISC), Online Public Access Catalogue of Indian Institute of Management, Ahmedabad, South Asian Economic Abstract (SOASEA), British Library of Development Studies (BLDS) of Institute of Development Studies (UK), Information Bulletin on Management (IIM Ahmedabad), FinanceWise, Social Science Information Gateway (SoSIG), FinancialStep, Scientific Journal Impact Factor (SJIF), EBSCO Publishing, Index Copernicus International (ICI), Indian Citation Index (ICI), French AERES (National Asso. for Scientific Research), International Scientific Index (ISI), ABDC (Australian Business Deans Council), Australia, Scientific Indexing Services (SIS) Scientific Group, SCOPUS.
We would appreciate if you may consider our request. I am enclosing the information brochure on the journal.
I look forward hearing from you.
With Regards.
Sincerely,
Prof. Dr. Aman Agarwal
Executive Editor, FINANCE INDIA
Director (Rektor), Dean (IR) & Professor of Finance, Indian Institute of Finance |
Reply : | Reply to this Complaint. |
|
7/26/2019 12:00:00 AM | Contact Name : | Jamestem |
Email ID : | deutschepornos.mobi@porncrop.org | Subject : | hello guys |
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3/2/2018 12:00:00 AM | Contact Name : | GetMyBusinessFunded |
Email ID : | noreply@get-my-business-funded.com | Subject : | SBA Capital for Your Business |
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3/9/2018 12:00:00 AM | Contact Name : | Michaelcaway |
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3/16/2018 12:00:00 AM | Contact Name : | FindBusinessFunding247 |
Email ID : | noreply@findbusinessfunding-247.com | Subject : | SBA Capital to Grow Your Business |
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4/8/2018 12:00:00 AM | Contact Name : | Anton FICAI |
Email ID : | anton@sciedtech.eu | Subject : | Advanced NanoMaterials and Technologies for Energy Sector – AdvNanoEnergy (ISSN: 2559 – 138X) |
Message : | Dear Founder,
I am Anton FICAI the Owner and Manager of The SciEdTech platform. recently you finalized the evaluation of our three journals and, for one of them (Advanced NanoMaterials and Technologies for Energy Sector – AdvNanoEnergy (ISSN: 2559 – 138X)) we didn''t receive the login details. the editor is Ecaterina ANDRONESCU (ecaterina.andronescu@upb.ro) and she didn''t receive anything from you even if the journal appear indexed in your database. Could you resend these login details to her or to me (anton@sciedtech.eu)?
Thank you very much,
Anton
|
Reply : | Reply to this Complaint. |
|
3/6/2018 12:00:00 AM | Contact Name : | BusinessLoansFundedNow |
Email ID : | noreply@businessloansfundednow.com | Subject : | Additional Capital for Your Business |
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3/9/2018 12:00:00 AM | Contact Name : | GetaBusinessFunded |
Email ID : | noreply@getabusinessfunded.com | Subject : | SBA Capital for Your Business |
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3/12/2018 12:00:00 AM | Contact Name : | Ph.D. Antonio Aragón Sánchez & Ph.D. Gregorio Sánchez Marín |
Email ID : | sbir@upct.es | Subject : | deleting journal from your directory |
Message : | Good morning we are the editors of journal with ISSN 2531-0046.
We kindly ask for our journal to be deleted from your directory
Thank you |
Reply : | Reply to this Complaint. |
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3/16/2018 12:00:00 AM | Contact Name : | Robertgefly |
Email ID : | zhadkevich.evgraf@mail.ru | Subject : | крем пигментация |
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4/10/2018 12:00:00 AM | Contact Name : | Clement Onou |
Email ID : | service@academicjournals.org | Subject : | Activating Indexed Journals |
Message : | Greetings and well done for a good job done at the DRJI. I represent Academic Journals ( www.academicjournals.org). Some time in 2016, our journals were accepted for indexingwith the DRJI.
I wish to be acquinted on the steps to be taken to have our content uploaded or if the DRJI would embark on automated crawling of the websites to bring up the content
Your reply will be appreciated.
Best regards
Clement Onou |
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7/15/2019 12:00:00 AM | Contact Name : | Davidlib |
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3/14/2018 12:00:00 AM | Contact Name : | Lal Zaman |
Email ID : | lalsoc095@gmail.com | Subject : | Request for information about Journal Indexing |
Message : | Dear and very respected Sir/Madam!
I hope this email will finds in best state of health.
Sir/Madam ! We have submitted our Journal on the name " PAKISTAN JOURNAL OF SOCIETY, EDUCATION AND LANGUAGE " ISSN: 2521-8123 and website http://www.pjsel.jehanf.com/index.php
We want to know about the process of review that our journal is accepted or not for indexing with your indexing system.
We need the indexing of that journal on emergency basis for the recognition of this journal in our Higher Education of Pakistan.
If there is any fee or any other charges for indexing then let,s us know, we will pay that charges bu
t send us the indexing certificate.
We will be very thankful, if you provide us information about our Journal indexing process.
Thanks
Regards |
Reply : | Reply to this Complaint. |
|
3/14/2018 12:00:00 AM | Contact Name : | GetaBusinessFunded247 |
Email ID : | noreply@getabusinessfunded247.com | Subject : | Additional Capital for Your Business |
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4/11/2018 12:00:00 AM | Contact Name : | Charlesgotly |
Email ID : | dowuch23874@mail.ru | Subject : | Asiame scam
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Message : | north american men and women stick direct specifications found on men time for birthplace
never a purchases, magazines released, home theater as well as. shot of anthony Gensheimer/San Jose Mercury tv news. 2/2000. VIETNAM the entire icon area in Ho chi Minh metropolitan can that included women showing off scant places. as soon as the confrontation, many of these are dressed can have been cause for imprisonment. as Vietnam exposed towards gulf during the early nineties, wear constraints were definitily tranquil.
people sailors on the the traveling to savorS Curtis Wilbur also talk with gals Vietnamese in a very bartender inside party the downtown area in from Da Nang 29 July 2004. The Arleigh Buirke lesson taken bomb destroyer is however second vent out cell phone simply an american military boat considering the Vietnam fights lost three ages backwards. The 342 robust producers through transport will pay out six days in the city in which you marine corps ended up with in March 1965, learning to be the specific first indian combats troopers in Vietnam. (photograph credit worthiness will ideally learned HOANG [url=https://www.facebook.com/Asiame-Scam-299122010522250/]Asiame Review[/url] DINH NAM/AFP/Getty thoughts)
display to CaptionGetting the essential charge ones Vietnamese federal government effortless. it then the credit everything from this man''s dearest anxious that he will run-a-way earlier mentioned certainly, there that needs your attention diplomatic attainments.
spouse is consistently irritable every occasion i go, told Liem, A way of life trainer at San Jose village student that also trips Vietnam every to show at a school. I hardly ever reveal a family long term trip through to the late. this task ailment minimization. you see, the lengthy the girl is aware, The prolonged i want to bear the pain sensation. many years after the Vietnam battle concluded, Communist state administration representatives candidly desired Vietnamese families back home, occasionally folks who fought against kids. but rather an additional civil war supports erupted, our one pitting Vietnamese u. s citizens women to protect against all their husbands and men who would like to come back to the Southeast asiatische uk. The men vital other consumers participate which Vietnamese chicks make up a story using keep in wait the parties, in most cases anticipating the finance security type fixture ought to attract.
girls back in Vietnam ''re violent. how they encounter! considered '' Tien, 38, online resources a substantial accounting sector in S Jose. the young woman talked about burned the lady''s gentleman to allow them to that love a short while ago guerrilla.
the stress rather than this trouble maintains got to ambitious proportions throughout the san francisco bay area Vietnamese people and therefore anywhere else. Vietnamese funny skits poke simple at the household strife but also stick artists sing out to fix it. in which the absolutely no. 1 issue for, Tien assumed. each and every era of the male gender moves returned itself, my friend used, It supposed my husband not only participating visit grandfather Vu in addition relative Thuy just to pass the time during the states with a good amount of cost effective younger ladies.
just isn''t a Vietnamese ancestry (back in Silicon vly) that may doesn decide of the male gender who has done this, Tien had said.
Hien Nhan, who owns the Polo tavern from your primary component bustling Ho chi Minh urban centre, asserted that Vietnamese u. s women do have top reasons to worry.
is actually, Vietnamese brides are obtaining prettier and as well as prettier, alleged Nhan, Perched on a stool at our inviting company which will serves up version dark beer, hamburgers and consequently girl job seekers in other words shorts that will flirtatiously remember to brush vs male buyers that like. set a little more make-up. these firms happen to eat far better. they begin to physical activity. but, they are afraid to just let foreigners realize that they are on the market to a frolic, that a affair some thing significant.
history is actually the male chases after the female, Liem supposed. things the other way around inside of Vietnam. One Vietnamese u s of a''s mechanic professional since Silicon pit to whom didn aspire to provide his good reputation concern with producing his second work permit matters: try to get injured onto throughout the day,every day. quite possibly at your accommodation. You check in thus injured a person. I can do better 10 days around Vietnam at the moment. in addition, I are involved in fuss. who also your self a second work permit often have demanding bounds designed into them, says San Jose john p Nguyen, who just ultimately last spring has a significant other appearing in Ho chihuahua Mlondonh. until recently, someone towards his or her overstayed a two week second work permit issued by previous girlfriend. he returned, that she threw almost her stuff and also against the road, he said.
was basically using so thrilling, Nguyen included. temptations are generally so excellent. affiliate marketers 50 and over can acquire young ladies which are inside 20s and looks like pieces. keep in mind this excessively suitable to pass up. Friskiness permeates the civilization in Vietnam that lots of people men planning to far from extra international locations can come across amazing.
a specific ideas page that you just wear encounter in Singapor evene tibet, let alone u. s, suggested Chung Hoang Chuong, A college component in the asiatische united states revisions unit with destination school associated with san francisco bay area, and uses over half the time in Vietnam. you earn a pass at someone, the actual triumphed in flick you and your family off. your woman clear-cut conclusion with a grin. crystal clear part reversal is probably led partly by the recognition of gulf world in addition,yet sub standard economic considerations in Vietnam. surely, Nguyen, A 40 years old workers,who are your employees found in customer care even though has grown to be discharged, stated that their ex-girlfriend in Vietnam of late broke up with her dad as they failed to find a good job in Vietnam.
Vietnam actually a demographically fresh community all around 70 per of the nation 90 million folks actually are younger unlike what 35 and so teenagers circulation in to big towns and cities this countryside day-to-day want occasions. Viet Kieu, the concept of a with regard to racial Vietnamese home in another country, and also and also the emerged as [url=http://www.dailymotion.com/video/x6djmnz]Asiame scam[/url] suited attracts for a little lady since they can hold these products their loved ones.
suitable jobs are extremely hard to find, and possibly in Vietnamese by way of advanced schooling degress fahrenheit, Nhan said.
Nguyen le, A 29 yr old to whom manages a Ho chi Minh urban sidewalk coffee shop, tells how your wife as well as other adult females are generally interested in Viet Kieu and foreigners for assorted top reasons, the exact first being stock welfare.
Have more assets, a lot of earnings, shown Nguyen. They are definitely more careful, lots more irritated also patient their lady. inside a eyes of foreigner, completely love is critical as opposed to with Vietnamese gents.
romance lively, merely we are not absurd, known Khanh Tran, A corporation,even though the list coach which of you abides in about San Jose. are remains to be in good shape, and that''s not me gonna (Misbehave from Vietnam) At the trouble relating to my in laws, my cousin. were in tact in excess of 40 many. an individual''s spouse, going on certainly no percentages, will not send [url=https://www.pinterest.com/asiamescam/]Asiame Review[/url] the former expert around the the southern region of Vietnamese marine corps a second work permit. would die to go back, he said wistfully.
considerably, few Vietnamese announce, usually the selling point of foreigners will be waning caused by a new grade of loaded Vietnamese, this consists of lots multimillionaires. and others Viet Kieu guys utilize a bad fame basically because act like playboys the people that throw currency surrounding and tell most wives they might be determined throughout their ailments only to vanish immediately after return to in america.
but rather often the game the Viet Kieu what individuals locate regarding the dropping off end. numerous what persons wed in addition,yet can be used those upcoming spouse the place to find in america have found the women imagined a significantly more potent diet and lifestyle compared with what they can assist with, inducing strife and then divorce proceedings.
Viet Kieu men subscribe to little compassion through Viet Kieu mothers her or his dalliances, whether be responsible for love as heartbreak. blame the many men with regard to weak point, For not being responsible, told me options Hanh, A 31 yr old San Jose person.
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5/7/2018 12:00:00 AM | Contact Name : | ibrahim yiğit |
Email ID : | psk.yigit@gmail.com | Subject : | About the Journal "Klinik Psikoloji Dergisi" |
Message : | Dear Sir/Madam,
We would glad to inform us about the process of "Klinik Psikoloji Dergisi" (in English Journal of Clinical Psychology Research). I sent an e-mail a month ago but have not get any information from you. Thank you in advance for your interest.
Kind regards. |
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|
5/13/2018 12:00:00 AM | Contact Name : | Muhammad Nadim Akbar Khan |
Email ID : | prh.jiimc@riphah.edu.pk | Subject : | Indexation with DRJI |
Message : | Dear Sir
I am Managing Editor of a science research journal titled"Journal of Islamic International Medical College" (JIIMC). We want to index our journal in DRJI . You are requested to guide about the process through which we can apply. |
Reply : | Reply to this Complaint. |
|
5/25/2018 12:00:00 AM | Contact Name : | ali |
Email ID : | alikaygisiz@ksu.edu.tr | Subject : | correct |
Message : | Dear colleage,
what is correct format,
COUNTRY : +90
city : 344
lokal : 3002108 |
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6/1/2018 12:00:00 AM | Contact Name : | Dr. Raj Kumar Yadav |
Email ID : | drrajkumaryadav@rediffmail.com | Subject : | Regarding Indexing journal and certificate after indexing |
Message : | Yesterday I received a mail from DRJI. Mail was that : -
Respected Sir/Madam,
We are very happy to inform you that your journal Journal of Educaional is indexed successively in our indexing service
Now, You can submit your articles
The login details are :
User Name : 2230-9586
Password : 698355
Thanking you,
Techical Dept.
Now my questions is that How can i get my indexing certificate.
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6/5/2018 12:00:00 AM | Contact Name : | Dr. Madhu Gupta |
Email ID : | dr.madhu@jjtu.ac.in | Subject : | Indexing the journal |
Message : | Dear sir
We would like to index our journal please guide me for further process |
Reply : | Reply to this Complaint. |
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3/20/2018 12:00:00 AM | Contact Name : | FastFundingAdvisors |
Email ID : | noreply@fastfundingadvisors.com | Subject : | Additional Capital to Grow Your Business |
Message : | Hi, letting you know that http://FastFundingAdvisors.com can find your business a SBA or private loan for $2,000 - $350K Without high credit or collateral.
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3/24/2018 12:00:00 AM | Contact Name : | GetaBusinessLoan247 |
Email ID : | noreply@getabusinessloan247.com | Subject : | SBA Capital for Your Business |
Message : | Quicker and Simpler than the SBA, http://GetaBusinessLoan247.com can get your business a loan for $2K-350,000 With low-credit and without collateral.
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3/29/2018 12:00:00 AM | Contact Name : | Marcos R. da S. Vieira |
Email ID : | amazonianjournalofplant@gmail.com | Subject : | Indexing the articles in the system |
Message : | Dear Sir,
I m so happy for the news!
One question. How Do I do to add the manuscripts of Amazonian Journal of Plant Research on the Directory of Research Journal Indexing system.
Best regards
Marcos Ribeiro da Silva Vieira
Editor in chief
Amazonian Journal of Plant Research |
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4/1/2018 12:00:00 AM | Contact Name : | Scottdaype |
Email ID : | scottBedyfrindygrere@gmail.com | Subject : | Test, just a test |
Message : | Hello. http://jakshgy773733.us |
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5/21/2018 12:00:00 AM | Contact Name : | Sharon Hindley |
Email ID : | s.l.hindley@swansea.ac.uk | Subject : | New Journal Submission Query |
Message : | Good afternoon,
I am the marketing manager for IJPDS and would like to submit the journal to DRJI for consideration. We can satisfy all the stipulated criteria but I am unable to find information relating to one point - ''Journal must contain the maximum stipulated number of editorial board members''. Could you please tell me how many this is please?
Many thanks
Sharon |
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3/9/2019 12:00:00 AM | Contact Name : | KiethRob |
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3/28/2018 12:00:00 AM | Contact Name : | GetMyBusinessFunded |
Email ID : | noreply@get-my-business-funded.com | Subject : | Capital to Grow Your Business |
Message : | Hi, letting you know that http://Get-My-Business-Funded.com can find your business a SBA or private loan for $2,000 - $350K Without high credit or collateral.
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3/31/2018 12:00:00 AM | Contact Name : | GetBusinessFunded |
Email ID : | noreply@getbusinessfunded.com | Subject : | SBA Funds to Grow Your Business |
Message : | Quicker and Easier than the SBA, http://GetBusinessFunded.com can get your business a loan for $2K-350,000 With low-credit and without collateral.
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10685-B Hazelhurst Dr. # 21465 Houston, TX 77043 |
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4/1/2018 12:00:00 AM | Contact Name : | Cihad Özsöz |
Email ID : | cozsoz@gmail.com | Subject : | Entered Wrong Information in Submitting |
Message : | Hi, I just suggested our journal Sosyoloji Notları in your web-site. But I wrote our frequency "Quarterly" by mistake. It would be "Biannually". Sorry for it, can we fix it?
Here is our suggestion: http://olddrji.lbp.world/JournalProfile.aspx?jid=2618-5903 |
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4/7/2018 12:00:00 AM | Contact Name : | Saravanan |
Email ID : | saravanan.sivaji@scientificscholar.com | Subject : | want login details - American Journal of Interventional Radiology |
Message : | Dear sir/mam
We register "American Journal of Interventional Radiology" in you site. but we are not receive the login details. Please sent the login details to us.
Thank you |
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4/3/2018 12:00:00 AM | Contact Name : | BusinessCapitalAdvisor |
Email ID : | noreply@businesscapitaladvisor.com | Subject : | SBA Capital for Your Business |
Message : | Quicker and Easier than the SBA, http://BusinessCapitalAdvisor.com can get your business a loan for $2K-350,000 With low-credit and no collateral.
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10685-B Hazelhurst Dr. # 21465 Houston, TX 77043 |
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4/8/2018 12:00:00 AM | Contact Name : | Kennethtrobe |
Email ID : | be.st.@seobest.website | Subject : | таблетки для усиления мужской потенции
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Message : | ВСЕГО ОДНА КАПСУЛА В ДЕНЬ
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Продлевает половой акт до 2-х часов
В 3 раза увеличивает количество спермы при эякуляции
Продлевает твой оргазм на 15-20 секунд
Сайт: http://cpagetti2.com/ZvOV/ |
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4/10/2018 12:00:00 AM | Contact Name : | ibrahim yiğit |
Email ID : | psk.yigit@gmail.com | Subject : | Inclusion of Klinik Psikoloji Dergisi |
Message : | Dear Sir/Madam,
Our journal (Klinik Psikoloji Dergisi from Turkey) has been "under evaluation" section but I could not see it there. And also, when I search for our journal from "countries" section, we could see it but it seems to not have a "licence." However, it has a licence of Creative Commons (CC-BY-NC-ND). So, we would like to learn what stage our journal is in and whether there are any problems regarding the inclusion. Thank you in advance for your time.
Kind regards,
İbrahim. |
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|
4/18/2018 12:00:00 AM | Contact Name : | ERNESTO LORENZO RAVELO CONTRERAS |
Email ID : | revistaacta@ucatolica.edu.co | Subject : | Solicitud corrección de pais - revista Acta Colombiana de Psicología |
Message : | Me permito solicitar corrección del país de origen de nuestra revista, la cual es de Colombia y no de Afganistán, dado que cometí error al incluirla, saludos.
|
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|
4/20/2018 12:00:00 AM | Contact Name : | Khalid shah |
Email ID : | khalidaup.shah@gmail.com | Subject : | Indexing journal |
Message : | it is to request you that abasyn university Peshawar Pakistan starting a journal entitle, Abasyn educational research journal. This journal provide new trend information to students as well as faculty members in the field of education.,so please index this journal with your esteem organization.The journal urls is below.
http://www.aerj.info/intro.php |
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|
6/11/2018 12:00:00 AM | Contact Name : | Diego Felipe Arbeláez Campillo |
Email ID : | amazoniainvestiga@uniamazonia.edu.co | Subject : | Indexación Revista Amazonia investiga issn 2322-6307 |
Message : | Estmados
Directorio de indexación de revistas de investigación.
Cordial saludo:
Al buscar el listado de revistas indexadas no evidenciamos la revista Amazonia investiga con ISSN 2322-6307 y la cual se encontraba alojada en sitio web en la siguiente dirección. http://www.drji.org/JournalProfile.aspx?jid=2322-6307
Por tal motivo, nos permitimos solicitarles nos permitan activar nuevamente su revista en su prestigioso directorio.
Sinceramente,
Diego Felipe Arbeláez C.
Editor revista Amazonia investiga |
Reply : | Reply to this Complaint. |
|
4/8/2018 12:00:00 AM | Contact Name : | ProFund247 |
Email ID : | noreply@profund247.com | Subject : | SBA Funds to Grow Your Business |
Message : | Quicker and Easier than the SBA, http://ProFund247.com can get your business a loan for $2K-350,000 With low-credit and without collateral.
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10685-B Hazelhurst Dr. # 21465 Houston, TX 77043 |
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4/10/2018 12:00:00 AM | Contact Name : | ProFunding247 |
Email ID : | noreply@profunding247.co | Subject : | SBA Funds to Grow Your Business |
Message : | Hi, letting you know that http://ProFunding247.co can find your business a SBA or private loan for $2,000 - $350K Without high credit or collateral.
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|
4/12/2018 12:00:00 AM | Contact Name : | Cristhian David Ramos Sosa |
Email ID : | sumadnegocios@konradlorenz.edu.co | Subject : | Indexing issues |
Message : | Dear all, good afternoon
Receive a warm greeting from the Suma de Negocios Journal team.
We are registered as a journal and we have the Print ISSN: 2215-910X - Electronic ISSN: 2027-5692 numbers, and we used to have an account with you all, Username: 2027-5692 and Password: %sumadenegocios% and we are unable to access since a very long time. So that, we want to ask you how we can access and upload all the manuscripts/papers we have since 2010.
Thank you very much for the information you may provide.
Best regards,
Cristhian David Ramos Sosa
Editor Assistant at the Suma de Negocios Journal
Konrad Lorenz University Foundation
|
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|
5/14/2018 12:00:00 AM | Contact Name : | Roderick Tilly |
Email ID : | tilly.roderick15@yahoo.com | Subject : | Crypto Affiliate 50% Lifetime Revenue share |
Message : | I can see you have cryptocurrency traffic
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3/7/2019 12:00:00 AM | Contact Name : | Robertdam |
Email ID : | leha.strochek@mail.ru | Subject : | Отзывы Иванчук Максим |
Message : | Плохие отзывы об Иванчуке максиме сергеевиче
https://plastic-surgeon.ru/forum/showthread.php?t=13277 |
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4/9/2019 12:00:00 AM | Contact Name : | BrianDob |
Email ID : | nare@course-fitness.com | Subject : | 台北市 萬華 禾云室內設計 |
Message : | 台北市 萬華 禾云室內設計
http://interior-plan.com/ |
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10/19/2015 12:00:00 AM | Contact Name : | kjfhgjkhfkg |
Email ID : | kjhk@gmail.com | Subject : | ljklj |
Message : | kg;kfg lkhllkdf lfhjghlk |
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10/21/2015 12:00:00 AM | Contact Name : | Ezequiel Redin |
Email ID : | ezequielredin@gmail.com | Subject : | Revista Extensão Rural - Indexação |
Message : | Prezados,
Gostaríamos de indexar a Revista Extensão Rural (Santa Maria) no portal. Existe essa possibilidade?
Na qualidade de Editor do periódico Extensão Rural (ISSN Impresso 1415-7802; ISSN Online 2318-1796) gostaria de saber qual é o processo de tramitação para indexar nosso periódico a seu portal. Estabeleço esse contato com o objetivo de ficarmos ao dispor de V.Sa. para qualquer consulta a respeito das atuais condições de nosso periódico. http://cascavel.ufsm.br/revistas/ojs-2.2.2/index.php/extensaorural/index
De antemão, comunicamos que assumimos como editores há quase três anos e estamos em processo de modernização e atendimento das orientações da CAPES, visando aumentar nossa qualificação neste importante indicador.
Gostaria de rememorá-los que o Periódico Extensão Rural vinculado ao Departamento de Educação Agrícola e Extensão Rural da Universidade Federal de Santa Maria (DEAER/UFSM) é uma publicação que completa 22 anos de história em relação aos estudos rurais. Temos em nossa base impressa (e agora toda ela digital, desde 1993) uma série de artigos escritos por renomados pesquisadores na área de extensão rural, desenvolvimento rural, sociologia rural, antropologia rural, administração rural, ciências sociais, educação rural, psicologia rural, economia rural, geografia agrária, ciências agrárias (em geral), saúde rural, etc. Pode-se observar que a revista contempla um tema contemporâneo nos estudos rurais, congregando uma multidisciplinariedade requerida nos últimos anos.
Atualmente, revigoramos nossas áreas, e a revista está destinada à publicação de trabalhos inéditos, na forma de artigos científicos e revisões bibliográficas, relacionados às seguintes áreas: i) Desenvolvimento Rural, ii) Economia e Administração Rural, iii) Sociologia e Antropologia Rural, iv) Extensão e Comunicação Rural, v) Sustentabilidade no Espaço Rural, vi) Saúde e Trabalho no Meio Rural. São publicados textos em inglês, português ou espanhol. Acesso público e gratuito, mediante cadastro no portal.
Somos uma das poucas revistas que adotou em massa as redes sociais. Desde, então, aumentamos nosso índice de submissão de artigos em 600%, dessa maneira, nossa qualidade aumentou, atingindo diferentes regiões do país e fora dele. Consequentemente, aumentou-se a demanda intensa de e-mails respondidos a autores, aprovação e qualidade do periódico. Para conferir, nossa rede social acesse:
Perfil no Facebook –
http://www.facebook.com/extensao.rural?ref=tn_tnmn
Página no Facebook –
http://www.facebook.com/pages/Extens%C3%A3o-Rural/397710390280860?ref=hl
Aumentamos nossos indexadores, a saber:
Indexadores - Indexers
INTERNACIONAL: 1- AGRIS 2- Latindex-Directorio 3- Diretório Luso-Brasileiro 4- e-revist@s 5- Journals for Free 6- EZB Nutzeranfragen 7- OAJI
NACIONAL: 8- AGROBASE 9- Portal livRe! 10- Sumários.org 11- Diadorim 12- Portal SEER 13- Portal de Periódicos CAPES 14- Rede Cariniana 15 – Google Scholar
Em 2015 serão publicadas quatro edições para comemorar 22 anos de publicações científicas. Temos certeza que o reconhecimento atribuído à revista se deve ao crescente número de autores, de instituições de diferentes regiões brasileiras, que escolhem a revista para a submissão dos seus trabalhos, bem como a qualificação e seriedade dos assessores científicos que têm colaborado sistematicamente com seu trabalho voluntário, mas sempre rigoroso, para a avaliação dos trabalhos que lhe são designados.
Enfim, estamos muito motivados a qualificar esta importante revista que faz marcas históricas na extensão rural brasileira. Com isso, contamos com o apoio de vocês para auxilio e qualificação de nosso periódico.
A saber, temos mais algumas ações já realizadas:
- Maior pressão de seleção de artigos, mediante maior divulgação do periódico;
- Digitalização de todo o acervo da revista, desde 1993.
- Uso de ferramentas online para divulgação das edições em âmbitos nacional e internacional;
- Sistema de editoração online para dar agilidade à tramitação dos artigos;
- Nomeação dos Editores de Áreas;
- Formação e consolidação do Comitê Editorial;
- Criação, discussão e aprovação do Estatuto da Revista Extensão Rural
- Avanço de duas para quatro edições anuais;
- Reestruturação das instalações e equipamentos da revista;
- Aumento de três para 14 indexadores nacionais e internacionais;
- Inclusão da Revista Extensão Rural (Santa Maria) no International Standard Serial Number (ISSN) online;
- Em 2015, o processo de tramitação envolveu mais de 250 artigos.
- Em 2015 foram mais de 200 pareceristas em atuação na Revista, o que forneceu agilidade, renovação e qualidade nos pareceres.
- Ingresso na Rede Cariniana de preservação digital dos artigos deste periódico.
- Inclusão do Digital Object Identifier (DOI): http://dx.doi.org/10.5902/23181796
-Ampliamos a divulgação em rede social, universidades, Secretarias de desenvolvimento, sites institucionais públicos e privados, o que proporcionou um amplo acesso da Revista, batendo o recorde de acessos nos 22 anos de publicações.
- Aumento do Qualis/Capes B5 para o Qualis/Capes B2, em 2015.
Nosso portal:
http://cascavel.ufsm.br/revistas/ojs-2.2.2/index.php/extensaorural/index
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10/24/2015 12:00:00 AM | Contact Name : | Ashish Vilas Mane |
Email ID : | editor@environmentaljournal.org | Subject : | About problme in indexing of journal |
Message : | Sir
Please include our journal ''Universal Journal of Environmental Research and Technology'' as there was server error at the time of filling online journal suggestion form.
http://www.environmentaljournal.org
It is online and open access journal.
Thank you. |
Reply : | Reply to this Complaint. |
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10/25/2015 12:00:00 AM | Contact Name : | Ioan-Cosmin MIHAI |
Email ID : | cosmin.mihai@raisa.org | Subject : | Indexing a journal in DRJI |
Message : | Hi,
I tried to index a scientific journal to DRJI database, but I had an error on submission. Can you help me with another method?
Best regards,
Cosmin |
Reply : | Reply to this Complaint. |
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10/27/2015 12:00:00 AM | Contact Name : | Mukhtiar Ali Unar |
Email ID : | mikhtiar.unar@faculty.muet.edu.pk | Subject : | Submission of journal |
Message : | Dear Sir/Madam, Mehran University Research Journal of Engineering and Technology is a peer reviewed quarterly journal publishing since 1982. The journal web site is http://publications.muet.edu.pk The journal publishes original contributions in engineering and technology. Further details are available on the web site. Could you kindly index this journal in your darabase.
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10/27/2015 12:00:00 AM | Contact Name : | Mukhtiar Ali Unar |
Email ID : | mikhtiar.unar@faculty.muet.edu.pk | Subject : | Submission of journal |
Message : | Dear Sir/Madam, Mehran University Research Journal of Engineering and Technology is a peer reviewed quarterly journal publishing since 1982. The journal web site is http://publications.muet.edu.pk The journal publishes original contributions in engineering and technology. Further details are available on the web site. Could you kindly index this journal in your darabase.
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11/2/2015 12:00:00 AM | Contact Name : | Angela Anderson |
Email ID : | angela.anderson@usmcu.edu | Subject : | Submitting Journal |
Message : | I just tried to submit the marine Corps University Journal for consideration but there was some type of error with the site. Please contact me for the required information to complete this process. |
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11/5/2015 12:00:00 AM | Contact Name : | avijit das avi |
Email ID : | avijit1987@yahoo.com | Subject : | Publishing journal |
Message : | Hi there,
I want to publish my journal in your website. I am publishing for the first time, so I dont know how to do it in your website. Is there any fee for the publication? |
Reply : | Reply to this Complaint. |
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11/6/2015 12:00:00 AM | Contact Name : | Dr anuj singhal |
Email ID : | editmarinemed@gmail.com | Subject : | indexing of a journal |
Message : | I am editor of a medical journal which is a registered journal with Indian Registrar. please provide me application format and procedure fro getting my journal indexed with you. regards |
Reply : | Reply to this Complaint. |
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11/6/2015 12:00:00 AM | Contact Name : | Liudmyla Zagoruiko |
Email ID : | hvat203@gmail.com | Subject : | Ukraine journal |
Message : | Hello, I have submmited our journal "Studies in Comparative Education" ISSN 2306-5532 (print), ISSN 2312-1904 (on-line). Can you inform about the current state of the journal in your database. will be thankful for the answer.
with best regards, Liudmyla Zagoruiko |
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11/7/2015 12:00:00 AM | Contact Name : | ghjgj |
Email ID : | apiguide2014@gmail.com | Subject : | vghvgh |
Message : | vgh |
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3/7/2019 12:00:00 AM | Contact Name : | RichardTet |
Email ID : | ityuplyayev@mail.ru | Subject : | максим иванчук пластический хирург отзывы |
Message : | Отзывы Иванчук Максим
https://forum.plastic-surgeon.com.ua/showthread.php?t=234 |
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4/8/2019 12:00:00 AM | Contact Name : | Jamesjet |
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Message : | 第一借錢網
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10/3/2017 12:00:00 AM | Contact Name : | Mrs Berezutskaya Marina |
Email ID : | bermarser@ua.fm | Subject : | submit journal |
Message : | Collection "Musicology of Dnipropetrovsk" is a scientific publication devoted to the actual problems of contemporary academic music art.
The thematic sections of the collection comprise research on the most problematic areas of the musical art of Dnipropetrovsk, Ukrainian musical culture, artistic and aesthetic education of youth, academic music performance and pedagogy, chronicles of artistic events (reviewing), as well as many theoretical, historical and cultural issues of musical art.
The publication is intended for graduate students, doctoral students, scientists, teachers of higher education institutions of culture and arts, as well as a wide range of professional musicians-performers and connoisseurs of academic music. |
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10/6/2017 12:00:00 AM | Contact Name : | Mahdi Mohammad Beygi |
Email ID : | journalhz@gmail.com | Subject : | unable to enter my account |
Message : | Hello Dear Sir or Madam,
Our journal was indexed in your site last year but after your site''s problem now it is in under evaluation part
by the way I''m not able to enter my account and submit our articles
please send me an available account ID and Password which I can do it
Faithfully
Mohammad beigi |
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10/13/2017 12:00:00 AM | Contact Name : | BusinessFunds365 |
Email ID : | fundingteam+olddrji.lbp.world@businessfunds365.com | Subject : | RE: SBA Capital for Your Business |
Message : | Quicker and Simpler than the SBA, http://BusinessFunds365.com can get your business a loan for $2K-350,000 With low-credit and no collateral.
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10/14/2017 12:00:00 AM | Contact Name : | Dra. Mireya Valdez |
Email ID : | mireyavaldez@ucla.edu.ve | Subject : | Indixación de revista |
Message : | Buenas tardes
Solicito por este medio la planilla para optar a la indixación de la Revista Científica Agroindustria, Sociedad y Ambiente.
Agradecida espero su respuesta |
Reply : | Reply to this Complaint. |
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10/18/2017 12:00:00 AM | Contact Name : | justin hoffman |
Email ID : | justin@cluteinstitute.com | Subject : | clute Institute journals not listed in DRJI |
Message : | It has come to our attention that our journals are no longer listed in DJRI. We would like to know if this information is correct.
Regards,
Justin Hoffman
Clute Institute |
Reply : | Reply to this Complaint. |
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6/25/2019 12:00:00 AM | Contact Name : | mayanGus |
Email ID : | mayanbodelson@gmail.com | Subject : | Dear Sir/Madam, |
Message : | Did you hear about the revolutionary product that changed the rules of learning? |
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10/7/2017 12:00:00 AM | Contact Name : | carlos ventura |
Email ID : | polymerchemistry@eurosciconconferences.com | Subject : | Media partner invitation |
Message : | Dear Sir,
Greetings from EuroScicon!
Discover the ease of exploring by becoming a Media Partner!
Since we target similar market segments, we wish to share with you the excitement and ideas for creation and exchange of knowledge and information in the scientific field through our Journals, Conferences and other means. You will find it interesting that through partnering with us you can easily reach world class university academicians, eminent scientists from research institutions; and experts from medical and health science industries / companies.
Share with us the following benefits by supporting EuroScicon Conferences as a Media Partners:
Benefits of association
1. Logo and Link of your corporate website in the Conference website and/or related Journal website.
2. Recognition listing in the final conference program / proceedings.
Verbal recognition at the Inaugural Ceremony.
3. One free standard booth to exhibit your company’s profile and services.
4. A 15-minute podium presentation (Audio-Visual presentation) within one of the main conference programs, ensuring your target audience will be ready to listen to your presentation.
5. One full page advertisement in the conference hand book.
6. Opportunity to take over and organize interested scientific track (s) / session (s) if any.
7. Opportunity to organize a symposium / workshop at the conference venue.
One-to-One Business Meetings.
We are open to any kind of Media Partnering proposals and are ready to extend the privileges. If you are looking forward to explore partnership opportunities and know more about the price policies or exchange of benefits, please send us an email to polymerchemistry@eurosciconconferences.com
For more details about the conference: http://polymerchemistry.euroscicon.com/
We look forward to a fruitful working relationship.
Yours sincerely,
Carlos ventura
Program Manager
Polymer Chemistry 2018
Tel : (+44) 2033182512
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10/25/2017 12:00:00 AM | Contact Name : | Sharon Hindley |
Email ID : | s.l.hindley@swansea.ac.uk | Subject : | DRJI Selection Criteria Query |
Message : | Dear Sirs,
Can I enquire about one of the selection criteria points please.
It states that ''Journal must contain the maximum stipulated number of editorial board members''. What exactly is the maximum number of Editors?
Many thanks
Sharon |
Reply : | Reply to this Complaint. |
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10/28/2017 12:00:00 AM | Contact Name : | Lukas Varhol |
Email ID : | info@probiznis.eu | Subject : | Hyperlink is incorrect |
Message : | Hello,
please repair our hyperlink, because is bad. Check that.
http://olddrji.lbp.world/Search.aspx?J=european%20science&S=0
BR,
Lukas Varhol |
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10/15/2017 12:00:00 AM | Contact Name : | Elahe Javadi |
Email ID : | elahejavadi@ut.ac.ir | Subject : | request to be index |
Message : | To whom it may concern
we are delighted to announce that International Journal of Horticultural Science and Technology (IJHST) (p-ISSN: 2322-1461, e-ISSN: 2588-3143) is a free open access journal that provides publication of manuscripts in all aspects of horticultural science with DOI. The aims of the International Journal of horticultural science and Technology are to stimulate scientific research among horticulture scholars and scientists all around the world and to provide a forum for the exchange of information and dissemination of knowledge in the horticultural science.(https://ijhst.ut.ac.ir/)
Hereby, we would like to index IJHST on DRJI, please let me know what kind of information you need in this regards, moreover, we express our willingness to contribute metadata.
Kind regards- IJHST office |
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3/6/2019 12:00:00 AM | Contact Name : | MauricePaxia |
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10/18/2017 12:00:00 AM | Contact Name : | Kristin Bruk |
Email ID : | emergingdiseases@alliedconferences.org | Subject : | Collaboration with ICEDCS 2018,Orlando,USA As a Media Partner |
Message : | Dear Sir/Madam,
Greetings…!
Hope this email finds you better.
We would like to invite as a media partner at "International Conference on Emerging Diseases, Outbreaks and Case Studies" which is going to be held during March 28-29, 2018 at Orlando, USA wherein we place your Logo on our conference webpage and conversely our logo on your website without any payment. We would be delighted to have you with us for free.
We wish to share with your Company/ Organization the excitement and ideas for creation and exchange of knowledge and information in the scientific field through our journal, conferences and other means. You will find it interesting that through partnering with us you can reach world class university academicians, eminent scientists from research institutions, and experts from Clinical and Medical Background.
Benefits of association as Media Partners:
• Logo Recognition on website with your website link
• Logo on conference printing material & half page add in conference book.
• An opportunity to conduct a workshop/symposia of your Organization at the conference
• Special privileges on group registrations
• Special discount on registration fee for Emerging Diseases & Case Reports 2018
To know more about the conference please click here: http://casereports.alliedacademies.com/
Looking forward for a fruitful working relationship.
Best Regards,
Kristin Bruk
Organizing Committee Assistant
Emerging Diseases & Case Reports 2018| Orlando, USA
Allied Academies
Tel: +1 201-877-9344
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10/19/2017 12:00:00 AM | Contact Name : | Julius Martina |
Email ID : | spine@neuroconferences.com | Subject : | 3rd International Conference on Spine and Spinal Disorders scheduled on June 11-13, 2018 London, UK |
Message : | Conference series Ltd proudly welcomes you to the 3rd International Conference on Spine and Spinal Disorders which is held during June 11-13, 2018 London, UK around the theme Divulging Advances for Better Comprehension of Spine Health. This Conference provides a unique platform which aims to generate new ideas for treatment and management of Spine and Spinal Disorders that will be beneficial across the globe. Spine 2018 will witness an assembly of experts from all over the world who will present their current cutting-edge research for the betterment of Spine health. |
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10/21/2017 12:00:00 AM | Contact Name : | hari kiran |
Email ID : | hari@aquilusinternational.com | Subject : | Partner Request for Neurorehabmeet - 2018 Conference, Singapore |
Message : | Dear Sir/Madam,
Our organization, Aquilus International is organizing a conference in Neurorehabilitation and Brain Disorders. The conference will be held in Singapore. The conference title is “International Conference on Neurorehabilitation and It’s Associated Disorders” and it will take place from 6-7, June, 2018 with a theme: "An Intuition to Development Strategies of New Neurorehabilitation Technologies, Research and Clinical Evaluation".
Our conference website: http://www.neurorehabconferences.com/
We have gone through you website and your organization Directory of Research Journals Indexing is doing a great service for Women empowerment, healthcare organizations, hospitals and healthcare professionals. Nursing is a field, where most the employment would be created for women and it would be a great platform to support each other. We receive a great number of participants from all over the world. As a partner organization, we would like to have you in our website, as well as in our brochure. If it is possible, you can have our conference logo and link in your website. This is absolutely a mutual support and understanding.
If you are willing, please provide your logo.
With Regards,
Sreekanth
Conference Manager
Singapore |
Reply : | Reply to this Complaint. |
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10/24/2017 12:00:00 AM | Contact Name : | mounika |
Email ID : | mounika@aquilusinternational.com | Subject : | Partner Request for Alzheimersmeet - 2018 Conference, Dubai, UAE |
Message : | Dear Sir/Madam,
Our organization, Aquilus International is organizing a conference in Alzheimer’s. The conference will be held in Hyatt Regency, Dubai, UAE. The conference title is “World Congress on Alzheimer''s Research and Therapeutic Advances” and it will take place from September 6 - 7, 2018 with a theme: "A Discussion For Developing Advanced Knowledge of Treatment Mechanisms, Processes and Recovery Methodologies Associated with Alzheimer’s Disease ".
Our conference website: http://www.alzheimersmeet.com/
We have gone through you website and your Alzheimer’s Association center is committed to accelerating the global effort to eliminate Alzheimer''s disease. You are the largest nonprofit funder of Alzheimer''s research. The Alzheimer''s Association has been involved in every major advancement in Alzheimer''s and related dementias research since the 1980''s and is a leader in the global fight for a world without Alzheimer''s. Believe in the value of collaboration and are willing to imagine a time when we will have disease-modifying treatments, preventive strategies and gold-standard care for all people affected by Alzheimer''s disease. As a partner organization, we would like to have you in our website, as well as in our brochure. If it is possible, you can have our conference logo and link in your website. This is absolutely a mutual support and understanding.
If you are willing, please provide your logo.
With Regards,
Sreekanth
Conference Manager
Dubai, UAE
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10/24/2017 12:00:00 AM | Contact Name : | Sergii Tarasenko |
Email ID : | lancet21century@gmail.com | Subject : | Journal Data Loss "Clinical endocrinology and endocrine surgery" |
Message : | Dear Sir or Madam,
I am writing in order to complain, that our User Name and Password have not worked yet.
Periodically we have not had the access to the site.
However, some numbers or issues have been uploaded.
Today we could not find the our journal Clinical endocrinology and endocrine surgery (e-ISSN 2519-2582, Print ISSN 1818-1384, DOI: 10.24026/1818-1384) on your resourse.
Would you be so kind to renewal our access or give us piecies of advice.
The login details are :
User Name : 2519-2582
Password : 54055 |
Reply : | Reply to this Complaint. |
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11/8/2015 12:00:00 AM | Contact Name : | A. DINESH KUMAR |
Email ID : | editor.ijmrme@gmail.com | Subject : | TO GET INDEXING REGARDING |
Message : | Dear Sir/Madam, Good Evening, I (My Journal) would like to join with you. Kindly take the necessary actions in favor of me.
Website: www.rdmodernresearch.org
Email: editor.ijmrme@gmail.com
R&D MODERN RESEARCH PUBLICATION
INTERNATIONAL JOURNAL OF MULTIDISCIPLINARY RESEARCH AND MODERN EDUCATION
ISSN: 2454 – 6119 (APPROVED BY NATIONAL SCIENCE LIBRARY, NEW DELHI)
IMPACT FACTOR: 2.432 & IBI FACTOR: 2.1
Contact Us
A. Dinesh Kumar,
Publisher & Manager,
R&D Modern Research Publication,
321, North Mathavi Road,
Sengoal Nagar Opposite,
Perambalur (TK) & (DT),
Tamilnadu, India. Pin: 621212
Contact Number: 9486345452
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11/16/2015 12:00:00 AM | Contact Name : | jaikaran singh |
Email ID : | jksingh81@gmail.com | Subject : | regarding indexed journals |
Message : | please provide loging and password for the website www.ijset.com and www.ijer.in |
Reply : | Reply to this Complaint. |
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4/8/2019 12:00:00 AM | Contact Name : | MatthewAdjus |
Email ID : | uhup@course-fitness.com | Subject : | XYZ|國中基測|基測|基測歷屆試題|命題光碟|基測中心|基測試題滿1000送200 |
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|
11/17/2015 12:00:00 AM | Contact Name : | Dr.Srikanth Shastry |
Email ID : | editor@pimr.org.in | Subject : | Medicine |
Message : | Dear Sir, Perspectives In Medical Research” is a multidisciplinary, peer reviewed journal published three times a year by Prathima Institute of Medical Sciences, Karimnagar, Telangana. The journal is published concurrently in print and electronic version
The journal seeks high-quality research from all medical specialties encompassing basic, applied and translational research in order to support and disseminate the knowledge and evidence based practices in the field of medical sciences.
The journal publishes various studies like basic studies including animal experiments, clinical and epidemiological studies in the form of original research articles, review articles (narrative as well as systematic), short communications, case studies and letter to the editor that meet the criteria of scientific and technical merits.
print ISSN 2348- 1447 and print ISSN is 2348-229X
We want to get our journal indexed in DRJI.
Please accept
|
Reply : | Reply to this Complaint. |
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11/24/2015 12:00:00 AM | Contact Name : | Murat Topçu |
Email ID : | jocasdergisi@gmail.com | Subject : | Problem in submitting journal |
Message : | Dear Sir/Madam,
There is a problem in submitting journal to your index. I have tried a couple of times but everytime I tried the web site has failed. Is there another way to submit it?
Thanks in advance..
Murat Topçu
Editor of JOCAS (Journal of Caucasian Studies)
http://www.jocas.net |
Reply : | Reply to this Complaint. |
|
11/27/2015 12:00:00 AM | Contact Name : | Prof.Tushar Sangole |
Email ID : | ceditor@iejrd.in | Subject : | Indexing issue |
Message : | Dear Sir,
Last year we have included our journal in DRJI but now its not showing anywhere ....kindly solve the issue to index the articles ............www.iejrd.in |
Reply : | Reply to this Complaint. |
|
11/27/2015 12:00:00 AM | Contact Name : | venkatesan |
Email ID : | selvamvenkatesan@gmail.com | Subject : | journal to be indexed |
Message : | through the institution we are publishing two journals biannual, i want the journals to be indexed, let me know the procedure. |
Reply : | Reply to this Complaint. |
|
11/28/2015 12:00:00 AM | Contact Name : | Arta Moro Sundjaja |
Email ID : | asundjaja@binus.edu | Subject : | Status of Bina Nusantara University Journal Inclusion |
Message : | Dear DRJI Team,
Several weeks ago, i request an inclusion of our journal into DRJI. I want to ask about the status of my application. Thank you.
Regards,
AMS |
Reply : | Reply to this Complaint. |
|
11/28/2015 12:00:00 AM | Contact Name : | Arta Moro Sundjaja |
Email ID : | asundjaja@binus.edu | Subject : | Status of Bina Nusantara University Journal Inclusion |
Message : | Dear DRJI Team,
Several weeks ago, i request an inclusion of our journal into DRJI. I want to ask about the status of my application. Thank you.
Regards,
AMS |
Reply : | Reply to this Complaint. |
|
11/29/2015 12:00:00 AM | Contact Name : | Romina Karimzadeh |
Email ID : | info@focsci.com | Subject : | submitting a journal |
Message : | Dear
it seems that your submitting form has problem. when i fill the form and push the submit bottom it shows error.
regards |
Reply : | Reply to this Complaint. |
|
12/2/2015 12:00:00 AM | Contact Name : | Gina Perry |
Email ID : | ijfr@sciedupress.com | Subject : | Request on failure to submit our journal |
Message : | Dear DRJI Team,
We have encountered a problem is submitting our journal suggestion to your system. It always says "Server Error in ''/'' Application". Could you please give us any help regarding this. Thanks.
Gina Perry
Editorial Assistant, International Journal of Financial Research
Sciedu Press
*************************************
1120 Finch Avenue West, Suite 701-309, Toronto, ON., M3J 3H7, Canada
E-mail: ijfr@sciedupress.com
Website: http://ijfr.sciedupress.com |
Reply : | Reply to this Complaint. |
|
12/3/2015 12:00:00 AM | Contact Name : | Tran Van Kham |
Email ID : | tckhxhnv@vnu.edu.vn | Subject : | Unable to submit journal for indexing |
Message : | Dear DRJI
I am Kham Tran, Executive Editor of Vietnam Journal of Social Sciences and Humanities. I try to submit my journal for indexing in the "submit form", however it is unable. Please help me to index our journal in your database
Journal title: Vietnam Journal of Social Sciences and Humanities, ISSN 2354-1172
Journal url: http://journal.ussh.vnu.edu.vn
Organisation: VNU-University of Social Sciences and Humanities, Hanoi, Vietnam
Chief editor: Prof.Nguyen Van Khanh
email: tckhxhnv@vnu.edu.vn
contact: +84435581984
Frequency: bi-monthly
Regards
Kham Tran
|
Reply : | Reply to this Complaint. |
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12/4/2015 12:00:00 AM | Contact Name : | DR DHEERAJ VASU |
Email ID : | biobulletin2015@gmail.com | Subject : | SUMMIT A JOURNAL DETAIL BUT IT FAILS |
Message : | SUMMIT A JOURNAL DETAIL BUT IT FAILS |
Reply : | Reply to this Complaint. |
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12/5/2015 12:00:00 AM | Contact Name : | Dr. George Kolanchery |
Email ID : | profkolanchery@gmail.com | Subject : | Unable to submit journal through your website |
Message : | Whenever I try to submit our journal, it comes with some server error. Could you please rectify it? Our website : www. researchenglish.com |
Reply : | Reply to this Complaint. |
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12/7/2015 12:00:00 AM | Contact Name : | Mohammad Nikookar |
Email ID : | editor@crpase.com | Subject : | Request for indexing: CRPASE |
Message : | Dear DRJI manager,
My name is Mohammad Nikookar and I am managing Director of Computational Research Progress in Applied Science and Engineering (CRPASE). My request for indexing CRPASE was sent about 3 month ago but I didn''t receive any email yet.
Please let me say that CRPASE is really a new founded journal and we are trying to promote that by publishing high quality papers. We will be grateful if you could consider our journal as a new founded one for possible indexing in DRJI. We will appreciate if you could guide us in this regard. Here are the details on our journal:
Title: Computational Research Progress in Applied Science and Engineering (CRPASE) Publication format: electronic
Electronic-ISSN: 2423-4591
Frequency of publication: Quarterly in 2015 but will be bimonthly in the future.
Journal''s website: www.crpase.com
Electronic availability: open access
Article languages: English article
Abstract languages: English
Journal description:
Computational Research Progress in Applied Science & Engineering: An international Journal (CRPASE), ISSN : 2423-4591 is a multidisciplinary peer-reviewed journal devoted to the prompt publication of peer-reviewed articles related to all aspects of computational methods in applied science and engineering. The journal articles will be accessed freely online.
Publisher: Pearl publication, pearlpub.com location of the publisher: No. 17-lorong ilmu 10 - Taman ilmu - Nibong tebal - 14300 - Pulau Pinang Malaysia
If any other details are needed, please do not hesitate to contact me.
Regards
Mohammad Nikookar
|
Reply : | Reply to this Complaint. |
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12/7/2015 12:00:00 AM | Contact Name : | José João |
Email ID : | josebvicente@bol.com.br | Subject : | Indexação de Revista |
Message : | Gostaria de saber como proceder para indexar a minha revista neste site, pois não consigo fazê-la via o site
José João (Editor) |
Reply : | Reply to this Complaint. |
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12/9/2015 12:00:00 AM | Contact Name : | Eleonora Biletska |
Email ID : | medpers@dma.dp.ua | Subject : | Journal «Medical perspectives» |
Message : | Hello!
Our journal "Medical perspectives" (ISSN 2370-0404) has been included in your base, we loaded our issues of the journal. After updating your site, we do not find the journal "Medical perspectives" in the list of "All journals"?
How to fix it?
Thank you.
http://medpers.dsma.dp.ua/home
E-mail: medpers@dma.dp.ua
Regards,
Eleonora Biletska
editor "Medical perspectives" |
Reply : | Reply to this Complaint. |
|
12/9/2015 12:00:00 AM | Contact Name : | Pablo Dias |
Email ID : | pablo.dias@ufrgs.br | Subject : | IRES Conference - 2016 Mexico City |
Message : | Hello,
I''d like inforation on a conference. It''s the 21st International conferences on Engineering and Natural Science (ICENS) promoted by The IRES. The website shows DRJIr supports this conference (http://theires.org/Conference/2016/Mexico/ICENS/). Is this statement true?
Thank you for your time |
Reply : | Reply to this Complaint. |
|
12/12/2015 12:00:00 AM | Contact Name : | Dr. Binod Kumar |
Email ID : | binod.kumar.1970@ieee.org | Subject : | Indexing for Journal |
Message : | sir, I have tried to upload my Journal details for indexing but this is not working.
Journal Website: www.jspmjtc.com/International Journal/international Journal.html |
Reply : | Reply to this Complaint. |
|
12/16/2015 12:00:00 AM | Contact Name : | saeed asefzadeh |
Email ID : | jresearchdpt@qums.ac.ir | Subject : | submit journal |
Message : | Dear manager
we submitted our journal(The Journal of Qazvin University Of Medical Sciences) in your data base on Jul 2015, but we didnt receive any message of you. We will waiting for your reply, and ready to have your commants.
Best Regards
Editor_in_chief |
Reply : | Reply to this Complaint. |
|
12/20/2015 12:00:00 AM | Contact Name : | M. R. Kavianpour |
Email ID : | office@civilejournal.org | Subject : | Civil Engineering Journal |
Message : | Dear Mr/Mrs
I was wondering if you improved the Civil Engineering Journal''s country in your Agreement Pending list,
the country is "Iran", and in your list registered "Afghanistan".
Regards,
M. R. Kavianpour |
Reply : | Reply to this Complaint. |
|
12/21/2015 12:00:00 AM | Contact Name : | SPMohanty |
Email ID : | spmcuttack@gmail.com | Subject : | Correction of Country Name |
Message : | Dear Sir,
Pedagogy of Learning (POL) journal of education has been submitted in your on-line data base. The name of country mistakenly chosen Afghanistan instead of India. Please guide us to make it INDIA. |
Reply : | Reply to this Complaint. |
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12/22/2015 12:00:00 AM | Contact Name : | Marcela Tamagnini |
Email ID : | marcela.tamagnini@gmail.com | Subject : | consulta indexación |
Message : | Les escribo en calidad de directora de la Revista TEFROS, alojada
actualmente en
http://www.hum.unrc.edu.ar/ojs/index.php/tefros/index
Está dedicada al estudio de la Frontera Sur indígena de Argentina,
Chile y Uruguay (América Latina). Queríamos saber cuáles son los requisitos para poder
indizar la revista en vuestro portal. LA suscripción tiene un costo?
|
Reply : | Reply to this Complaint. |
|
12/27/2015 12:00:00 AM | Contact Name : | Vincentas Lamanauskas |
Email ID : | bjcem.journal@gmail.com | Subject : | Question related to the journal BJCEM |
Message : | Dear Sir/Madam,
We wrote to you before, however, the solution was not done. We have our journal BJCEM registered in DRJI but we can not access it. Also, on this page journal is not indicated: http://drji.org/BrowseByCountry.aspx?q=LITHUANIA. Our journal ID was ID: 2345-0193. Can you inform us?
Thank you in advance
Editor |
Reply : | Reply to this Complaint. |
|
12/29/2015 12:00:00 AM | Contact Name : | Tanya Malik |
Email ID : | tanu.mlk@gmai.com | Subject : | Indexing of our journal |
Message : | Pl send us the process and cost of indexing our management journal with you |
Reply : | Reply to this Complaint. |
|
12/29/2015 12:00:00 AM | Contact Name : | RAJESH RAMCHANDRA SHELAR |
Email ID : | svimslib@gmail.com | Subject : | Required Journal Indexing |
Message : | Dear Sir,
Please Send me Journal Indexing Procedure & Your Indexing Cost. |
Reply : | Reply to this Complaint. |
|
1/6/2016 12:00:00 AM | Contact Name : | mahmood seyyed |
Email ID : | mahmood.seyyed@yahoo.com | Subject : | History |
Message : | Hi Dear
I would like inform to you , I want submit article to your journal , but I have some question ,
1- do you have any journal about History and that journal indexing with ISI ?
2- should I pay for publishing article ?
thank you
seyyed |
Reply : | Reply to this Complaint. |
|
1/7/2016 12:00:00 AM | Contact Name : | Jermaine S. McDougald |
Email ID : | laclil@unisabana.edu.co | Subject : | Journal was accepted but not found in database |
Message : | I am inquiring about the status of the Latin American Journa of Content and Language Integrated Learning eISSN: 2322-9721 - ISSN: 2011-6721
Acceptance email was received on : Sat 21/09/2013 11:20 p.m.
From: ashok.drji@gmail.com
Content of email:
Respected Sir/Madam,
We are very happy to inform you that your journal LACLIL is indexed successively in our indexing service
Now, You can submit your articles
The login details are :
User Name : 2322-9721
Password : 770540
Thanking you,
Techical Dept.
Furthermore, the username and password that were provided do not work.
Do I need to reapply again?
Kind regards
Jermaine McDoougald
Managing Editor
LACLIL
jermaine.mdougald@unisabana.edu.co
laclil@unisabana.edu.co |
Reply : | Reply to this Complaint. |
|
1/8/2016 12:00:00 AM | Contact Name : | Taras Kotyk |
Email ID : | taras1390@gmail.com | Subject : | Publisher Login |
Message : | Dear Managers of DRJI!
Publisher team of Galician Medical journal (which is already indexed by DRJI) ask You how we can get access to publisher page at DRJI?
Thank You!
Regards, publisher team of Galician Medical journal |
Reply : | Reply to this Complaint. |
|
1/8/2016 12:00:00 AM | Contact Name : | Yuri Velikodsky |
Email ID : | yuri.velikodsky@gmail.com | Subject : | Indexing |
Message : | What is the status of our journal "Astronomical School’s Report" (ISSN No. : 2411-6602)? It is added to your site (http://drji.org/JournalProfile.aspx?jid=2411-6602), however it is not listed in "Indexed Journals". Thank you! |
Reply : | Reply to this Complaint. |
|
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1/9/2016 12:00:00 AM | Contact Name : | Mukhtiar Ali Unar |
Email ID : | mukhtiar.unar@faculty.muet.edu.pk | Subject : | Indexing of the Journal |
Message : | I am thankful that you have accepted Mehran University Research Journal of Engineering and Technology to be included in your directory. However, I am waiting for user name and password to upload papers of this journal |
Reply : | Reply to this Complaint. |
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1/10/2016 12:00:00 AM | Contact Name : | Journal of Law and Political Science |
Email ID : | suhelftlaui@yahoo.com | Subject : | journal Evalution |
Message : | We would like to include our(Journal of Law and Political Science) in your index (DRJI)
2222-7288
Issued by the Faculty of Law - University Alburk - Denmark
ISSN 2222-7288
Established in 2011
Best regards
Prof. Suhail Hussein Fatlawi
Editor in chief of the journal |
Reply : | Reply to this Complaint. |
|
1/12/2016 12:00:00 AM | Contact Name : | Journal Wychowanie w Rodzinie [Family Upbringing] |
Email ID : | wwr@uwr.edu.pl | Subject : | application to drji |
Message : | Dear Madame/ Sir,
I represent Journal "Family Upbringing" which is affiliated by University of Wrocław. Journal is published twice a year. Article focused on education, family and pedagogy. Journal was created in 2011 and we published 11 issues.
I would like to start the cooperation with drji. Could you send me information that the cooperation is possible and what we should do to start coopetition.
I will be grateful for details,
Regards,
Aleksandra Marcinkiewicz-Wilk
http://wychowaniewrodzinie.uni.wroc.pl/
|
Reply : | Reply to this Complaint. |
|
1/14/2016 12:00:00 AM | Contact Name : | BHARATI JANI |
Email ID : | jani.bharati10@gmail.com | Subject : | EDUCATION |
Message : | GOOD MORNING SIR.
I HAVE SUBMITTED MY ARTICLE PLEASE PROVIDE SOME INFORMATION HOW I AM SUBMTTING MY RESEARCH ARTICLE |
Reply : | Reply to this Complaint. |
|
1/17/2016 12:00:00 AM | Contact Name : | DR. Vijai Kumar Umrao |
Email ID : | editorhortflora.vku@gmail.com | Subject : | Need of Login ID & Password |
Message : | Dear Sir
Pl send publisher''s panel login ID & password for uploading and updating issues of HortFlora Research Spectrum (ISSN: 2250-2823) |
Reply : | Reply to this Complaint. |
|
1/18/2016 12:00:00 AM | Contact Name : | Hakan Kibar |
Email ID : | hakan.kibar@ibu.edu.tr | Subject : | Change country |
Message : | Dear,
Our country on the link below should be changed to Turkey. Mistakenly it is written as Afghanistan.
http://www.drji.org/JournalProfile.aspx?jid=2149-8245 |
Reply : | Reply to this Complaint. |
|
1/22/2016 12:00:00 AM | Contact Name : | Trianokta Akbar Wardana |
Email ID : | trianokta.akbar@yarsi.ac.id | Subject : | Edit Publisher Name |
Message : | Dear Team DRJI,
Please help to change the name of the publisher name of our journal, the name Publisher should be YARSI University ( same as Organization ) not Rika Yuliwulandari, we incorrectly enter the name publisher.
Our Journal Name is Jurnal Kedokteran YARSI
Thank you for your attention
(sorry for my bad english) |
Reply : | Reply to this Complaint. |
|
1/23/2016 12:00:00 AM | Contact Name : | DINESH KUMAR A |
Email ID : | editor.ijmrme@gmail.com | Subject : | Get Indexing Reg............. |
Message : | Dear Sir, I try to apply indexing. But "Server Error in ''/'' Application". So kindly guide me to join with you. |
Reply : | Reply to this Complaint. |
|
1/23/2016 12:00:00 AM | Contact Name : | Mykhailo Khmelniuk |
Email ID : | reftech@ukr.net | Subject : | REFRIGERATION ENGINEERING AND TECHNOLOGY |
Message : | Few months ago we received from You letter, about indexed our journal REFRIGERATION ENGINEERING AND TECHNOLOGY in Your indexing service.
There was login and password in that letter, when we try open our page we have "server error".
Can You help with this problem? |
Reply : | Reply to this Complaint. |
|
1/25/2016 12:00:00 AM | Contact Name : | Dr. Mritunjay Sharma |
Email ID : | drmritunjaysharma@gmail.com | Subject : | society/publisher registration |
Message : | Dear sir,
we have a registered society from himachal. we are running 3 journals. we want to reg. with you. Shall we reg. as publisher or other?
Thanks |
Reply : | Reply to this Complaint. |
|
1/27/2016 12:00:00 AM | Contact Name : | Dilip Bangthai |
Email ID : | dimorianreview@gmail.com | Subject : | Status of Submit Journal for Indexing |
Message : | Sir/Madam,
Kindly refer our Submit Journal dated 18.12.2015 for indexing our e-journal DIMORIAN REVIEW, URL http://www.dimorianreview.com, a peer reviewed multidisciplinary indexed e-journal, ISSN (Online): 2394-9163, published bi-monthly in English from Assam, India. This is an academic, non-profit making and voluntary initiative and we publish free of cost.
We have seen the status that our e-journal is Under Evaluation with the link http://www.drji.org/JournalProfile.aspx?jid=2394-9163
We believe that indexing under your esteemed Directory will help us enormously providing value to the work of the authors and research scholars.
Therefore, in view of the above, we would like to request you to kindly look forward with regard to indexing our e-journal.
Thanking you,
Dilip Bangthai
Editor (Hon.)
|
Reply : | Reply to this Complaint. |
|
1/28/2016 12:00:00 AM | Contact Name : | ashwanii sengar |
Email ID : | ashwaniisrl1987@gmail.com | Subject : | Regarding Submission |
Message : | Hello Sir,
I had submitted 3 journal on the behalf of my Editorial team. I got the mail of indexed my journals in DRJI.
But i am unable to search the journals in your indexed list. Could you please help to find out these journal in your indexed list journals.
Journal of Applied Chemistry ISSN 2348-0408
Journal of Applied Science And Research ISSN 2348-0416
Journal of Engineering And Technology Research ISSN 2348-0424 |
Reply : | Reply to this Complaint. |
|
2/6/2016 12:00:00 AM | Contact Name : | Mukhtiar A. Unar |
Email ID : | mukhtiar.unar@faculty.muet.edu.pk | Subject : | Pending Agreement |
Message : | Dear Sir, our Journal "Mehran University Research Journal of Engineering and Technology" has been included in your database. We are waiting for the agreement. Could you kindly send us the same whenever possible for you. |
Reply : | Reply to this Complaint. |
|
2/8/2016 12:00:00 AM | Contact Name : | abdul hadi al khalili |
Email ID : | info@tofiq.org | Subject : | About Our Journal Application |
Message : | Greetings,
I tried submitting our journal (TOFIQ Journal Of Medical Sciences, TJMS) on the application form but was not successful. I got the following message:
Line 328: int status = mread.GetInt16(19);
Line 329: if (status == 0)
Line 330: {
Please advise
Hadi |
Reply : | Reply to this Complaint. |
|
2/9/2016 12:00:00 AM | Contact Name : | sdmvms polytechnic college hatta |
Email ID : | 1006principal@msbte.com | Subject : | regardingrequirment of national and international journals |
Message : | please send details
waybase s g
principal |
Reply : | Reply to this Complaint. |
|
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|
2/10/2016 12:00:00 AM | Contact Name : | Prof. Suhail Al-Fatalwi |
Email ID : | suhelftlaui@yahoo.com | Subject : | Suggest our Journal |
Message : | we are issued the journal Law and political sciences. we tried to enter to your site, but the site does not work |
Reply : | Reply to this Complaint. |
|
2/11/2016 12:00:00 AM | Contact Name : | Borys Vitaliy |
Email ID : | vitala_boris@mail.ru | Subject : | Username and password |
Message : | Hello, I am an official representative of the journal Food Science and Technology. Not if you could send me your login information in my profile (username and password), I would like to unload the article.
http://www.drji.org/JournalProfile.aspx?jid=2409-7004 |
Reply : | Reply to this Complaint. |
|
2/13/2016 12:00:00 AM | Contact Name : | Dr. Sushil Nagar |
Email ID : | jibresearch1@gmail.com | Subject : | Login not working |
Message : | my login is not working, please tell me how to log-in to update my journal''s volume and issues in DRJI
Thank you
Dr. Sushil Nagar
Journal of Innovative Biology
User login: 2348-6473 |
Reply : | Reply to this Complaint. |
|
2/15/2016 12:00:00 AM | Contact Name : | Mohammad Nikookar |
Email ID : | editor@crpase.com | Subject : | Request for indexing: CRPASE |
Message : | On behalf of the editorial board members of CRPASE (2423-4591), I should say that this journal is really a new founded journal and we are trying to promote that by publishing high quality papers. Since DRJI is really a prestigious index for our scientific community, we will be grateful if you could consider our journal as a new founded one for possible indexing in DRJI. We will appreciate if you could guide us in this regard. |
Reply : | Reply to this Complaint. |
|
2/16/2016 12:00:00 AM | Contact Name : | Ali KORKUT |
Email ID : | ali.korkut@dicle.edu.tr | Subject : | IJOKS published issues |
Message : | Dear Editors,
Thank you for including our journal ''INTERNATIONAL JOURNAL OF KURDISH STUDIES'' in your index.
Now, we would lke to update our journal info and published issues online. We want to add our issues here.
However, I forgot my login name and password.
Will you please help me reset them, or you may send them to my e-mail address?
Thanks again
Yours sincerely,
Ali KORKUT
Asst. Editor
INTERNATIONAL JOURNAL OF KURDISH STUDIES
http://ijoks.com/index.html
|
Reply : | Reply to this Complaint. |
|
2/18/2016 12:00:00 AM | Contact Name : | Ömer Tuğrul KARA |
Email ID : | gevheri76@hotmail.com | Subject : | Çukurova Araştırmaları Journal |
Message : | First of all, we thank a lot for your inclusion our journal (Çukurova Researches) in your index. However, in the application period of your index, we changed the name of our journal website to make online access much easier. The former website is out of use. Our new website address is www.cukar.org Could you please make the required change by correcting the name of our website?
Journal Editor
Dr. Ömer Tuğrul KARA
www.cukar.org
|
Reply : | Reply to this Complaint. |
|
2/26/2016 12:00:00 AM | Contact Name : | Dr. Varun Jindal |
Email ID : | editordjas@gmail.com | Subject : | indexing in Worldcat |
Message : | Thank you for Including Dental Journal of Advance studies in DRJI. this is a query regarding indexing. does indexing in DRJI means that this is indexed in worldcat also. if no then what is the procedure of getting this journal indexed in Worldcat? |
Reply : | Reply to this Complaint. |
|
2/29/2016 12:00:00 AM | Contact Name : | Dr. Yuval Rottenstreich |
Email ID : | editor@ijbassnet.com | Subject : | Publication |
Message : | http://ijbassnet.com/current |
Reply : | Reply to this Complaint. |
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Message : | I do not know |
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3/1/2016 12:00:00 AM | Contact Name : | Gabriela Popoviciu |
Email ID : | gpopoviciu@uoradea.ro | Subject : | indexed journal |
Message : | Dear All,
in February 2013 you confirm me that our journal - Journal of Applied Engineering Sciences, with ISSN 2247-3769 and e-ISSN: 2284-7197, which is belong to Faculty of Civil Engineering, Cadastre and Architecture, from University of Oradea, Romania.
Now, because I must take some printscrens after our indexing and proof to have its..., I found that our journal is not in DRJI! What happened? Could you please answered me to that as soon as posible you can, because I cannot keep your logo on our journal''s website if we are not in there. Hope you understand and also help me in this direction.
Thank you! |
Reply : | Reply to this Complaint. |
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3/2/2016 12:00:00 AM | Contact Name : | Adela Ruiz |
Email ID : | adelaruiz@perio.unlp.edu.ar | Subject : | Enquiry - Journal Oficios Terrestres (Argentina) |
Message : | Estimated,
I write you in order tu consult if you received the postulation of the cientific journal OFICIOS TERRESTRES (Argentina) to integrate your Directory of Research Journals Indexing.
Thank you for your replay.
My compliments.
Lic. Adela Ruiz
---
Dirección de Publicaciones Científicas
Secretaría de Investigaciones Científicas
Facultad de Periodismo y Comunicación Social
Universidad Nacional de La Plata
Buenos Aires | Argentina |
Reply : | Reply to this Complaint. |
|
3/5/2016 12:00:00 AM | Contact Name : | Seval Kayabolen |
Email ID : | secretary@eng-scoop.org | Subject : | your site has some problem |
Message : | We cannot open our journal info which is published on
http://www.drji.org/JournalProfile.aspx?jid=2458-8822
Your site has some problems. Please fix it. |
Reply : | Reply to this Complaint. |
|
3/7/2016 12:00:00 AM | Contact Name : | Dr. Sandeep Singhal |
Email ID : | editor.ijoetr@gmail.com | Subject : | Not able to loging |
Message : | Dear Sir,
we are not able to login in account
The login details are :
User Name : applied
Password : 250062 |
Reply : | Reply to this Complaint. |
|
3/9/2016 12:00:00 AM | Contact Name : | Zunilka Limonta Favier |
Email ID : | zunilka@ciget.gtmo.inf.cu | Subject : | About a journal submission |
Message : | Hello, I work in the edition of Hombre, Ciencia y Tecnología journal, we appear in your site as a journal recently added, but I need to know if we could put your logo in our home page. Thenaks for your attention, and I will wait your answer, We are included in your database oficially or we are under evaluation? |
Reply : | Reply to this Complaint. |
|
3/14/2016 12:00:00 AM | Contact Name : | Mina Raeisi |
Email ID : | ijrelt.journal@gmail.com | Subject : | indexing process |
Message : | Dear Dr.
Hello
Would you please inform me that how can I follow the indexing process of my journal?
and how long does it take time?
Regards
Mina Raeisi
|
Reply : | Reply to this Complaint. |
|
9/10/2017 12:00:00 AM | Contact Name : | Chemistry of Advanced Materials |
Email ID : | editor.cam@issrpublishing.com | Subject : | I could not find my Indexied journal information |
Message : | Dear Directory of Research Journals Indexing
I have submitted my journal for indexing by DRJI and it has approved and a certificate. But now i couldn''t find my journal information of your website.
Chemistry of Advanced Materials (CAM) ISSN 2415-6213
Regards
--
Editor-in-Chief
Chemistry of Advanced Materials
ISSR Publisher |
Reply : | Reply to this Complaint. |
|
11/6/2017 12:00:00 AM | Contact Name : | Dr. A. Noor |
Email ID : | greenfarming@gmail.com | Subject : | Guidance for Indexing and Impact Factor analysis. |
Message : | We shall be highly thankful if you will kindly guide us in getting impact factor for our research Journal entitled “GREEN FARMING“ (International Journal of Applied Agricultural and Horticultural Sciences) published Bi-monthly at Jodhpur (Rajasthan) India. If any performa or guidelines are available, the same may kindly be sent along with the charges etc. on our e-mail id greenfarming@gmail.com
The Journal is already rated by NAAS, New Delhi from 2017. If impact factor is also given from 2015, 2016, 2017 and onwards. Plz. inform about the branch office in India. Plz. inform if any information and clarification is required by you at your earliest. |
Reply : | Reply to this Complaint. |
|
11/10/2017 12:00:00 AM | Contact Name : | Dr Mohammad Zibaei |
Email ID : | entericpathogens@gmail.com | Subject : | please change our journals publisher |
Message : | Dear Sir or Madam
I am Editor of chief of INTERNATIONAL JOURNAL OF ENTERIC PATHOGENS.would you please change our journals name of publisher that show in this link http://olddrji.lbp.world/JournalProfile.aspx?jid=2345-3362
safnek and change it to Alborz University of Medical Sciencs
thanks |
Reply : | Reply to this Complaint. |
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3/14/2016 12:00:00 AM | Contact Name : | Yuri Morales López |
Email ID : | revistauniciencia@una.cr | Subject : | Error Login |
Message : | Hello
I tried to submmit the news articles to your database but we got this (see below).
Please inform me when this system be able for upload the files. Our ID is EISSN: 2215-3470.
Regards,
Yuri Morales
Invalid attempt to read when no data is present.
Description: An unhandled exception occurred during the execution of the current web request. Please review the stack trace for more information about the error and where it originated in the code.
Exception Details: System.InvalidOperationException: Invalid attempt to read when no data is present.
Source Error:
Line 40: string ps = Server.HtmlEncode(txtPassword.Text);
Line 41:
Line 42: if (mread.GetString(1).Equals(us))
Line 43: {
Line 44:
Source File: d:\inetpub\vhosts\academicprofile.org\directory_of_research_journals_indexing\Publisher\Default.aspx.cs Line: 42
Stack Trace:
[InvalidOperationException: Invalid attempt to read when no data is present.]
System.Data.SqlClient.SqlDataReader.ReadColumn(Int32 i, Boolean setTimeout) +4897219
System.Data.SqlClient.SqlDataReader.GetString(Int32 i) +19
Administrator_Default.btnLogin_Click1(Object sender, EventArgs e) in d:\inetpub\vhosts\academicprofile.org\directory_of_research_journals_indexing\Publisher\Default.aspx.cs:42
System.Web.UI.WebControls.Button.OnClick(EventArgs e) +111
System.Web.UI.WebControls.Button.RaisePostBackEvent(String eventArgument) +110
System.Web.UI.WebControls.Button.System.Web.UI.IPostBackEventHandler.RaisePostBackEvent(String eventArgument) +10
System.Web.UI.Page.RaisePostBackEvent(IPostBackEventHandler sourceControl, String eventArgument) +13
System.Web.UI.Page.RaisePostBackEvent(NameValueCollection postData) +36
System.Web.UI.Page.ProcessRequestMain(Boolean includeStagesBeforeAsyncPoint, Boolean includeStagesAfterAsyncPoint) +1565
|
Reply : | Reply to this Complaint. |
|
6/19/2017 12:00:00 AM | Contact Name : | Vijay Muni |
Email ID : | editor.ijmtst@gmail.com | Subject : | Unable to Login in to Control Panel |
Message : | Respected Sir/Madam, We IJMTST team received the username and password. But we are unable to login into the panel please solve our problem |
Reply : | Reply to this Complaint. |
|
6/20/2017 12:00:00 AM | Contact Name : | Prof.(Dr.)Ramanjeet Singh |
Email ID : | drramanjeet@gmail.com | Subject : | Indexing of Journals |
Message : | We are interested in getting our Journals indexed in your database. Kindly guide. |
Reply : | Reply to this Complaint. |
|
6/21/2017 12:00:00 AM | Contact Name : | Mustafa CANBEK |
Email ID : | info@odad.org | Subject : | Indexing - Journal of Organizational Behavior Research |
Message : | Dear Madam/Sir,
I''m writing to you as Publication Co-ordinator of Journal of Organizational Behavior Research. Our journal was accepted by you to be indexed in DRJI. For couple mounts our journal was indexed but today I saw our journal is not in the list. Probably you''ve deleted our journal because of we didnt enter the meta data of our recent issue. I can explain. Maybe you are aware maybe not but when I try to enter your website I face with system error. And it occurs frequently. When I was ready to upgrade the meta data your system gave failure, when I saw your website is working I was not possible to do that. Now I see your system is working. I made a new application for the journal. I''ll upgrade the meta data with the latest issue.
Regards,
Mustafa CANBEK
info@odad.org |
Reply : | Reply to this Complaint. |
|
3/30/2018 12:00:00 AM | Contact Name : | Stephenfew |
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|
6/24/2017 12:00:00 AM | Contact Name : | Ravindra Sharma |
Email ID : | ravindra.8810@gmail.com | Subject : | Regarding Publisher Login problems |
Message : | Dear Sir,
We have registered our Journals in June 2013 and already indexed many papers in your website. But Now i am unable to login my panel.
As i am old user, so please help me and provide new user name and password.
I have received user name password email by ashok.drji@gmail.com on 10 June 2013.
My Journals:
INTERNATIONAL JOURNAL OF ENGINEERING AND TECHNICAL RESEARCH
The login details are :
User Name : 2321-0869
Password : 907633
INTERNATIONAL JOURNAL OF MODERN COMMUNICATION TECHNOLOGIES AND RESEARCH
The login details are :
User Name : 2321-0850
Password : 876551
Thanks. |
Reply : | Reply to this Complaint. |
|
6/26/2017 12:00:00 AM | Contact Name : | Amy Varera |
Email ID : | amy.varera@gmail.com | Subject : | Want more clients and customers? |
Message : | We can put your website on 1st page of Google to drive relevant traffic to your site. Let us know if you would be interested in getting detailed proposal. We can also schedule a call & will be pleased to explain our services in detail. We look forward to hearing from you soon. Thanks! |
Reply : | Reply to this Complaint. |
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6/26/2017 12:00:00 AM | Contact Name : | BusinessFunds365 |
Email ID : | fundingteam+olddrji.lbp.world@businessfunds365.com | Subject : | RE: Capital for Your Business |
Message : | Hi, letting you know that http://BusinessFunds365.com can find your business a SBA or private loan for $2,000 - $350K Without high credit or collateral.
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Reply : | Reply to this Complaint. |
|
6/29/2017 12:00:00 AM | Contact Name : | Maria Gonzalez Polo |
Email ID : | mgonzalez@cug.co.cu | Subject : | Solicitud de informacion sobre indexación de Revista EduSol |
Message : | Soy la editora de la Revista EduSol y estaba indexada en su base de datoas con la dirección
http://www.drji.org/JournalProfile.aspx?jid=1729-8091
Ahora la busco y no la encuentro. Podrian decirme que ha pasado y donde la puedo encontar, pues habia sido incorporada a DRIJ en diciembre de 2016.
Saludos
En espera de su respuesta |
Reply : | Reply to this Complaint. |
|
6/30/2017 12:00:00 AM | Contact Name : | Hasan BOZGEYİKLİ |
Email ID : | hbozgeyikli@gmail.com | Subject : | index problem |
Message : | I''am the editör of OPUS- International Journal of Society Researches which is indexed on Directory of Research Journal Indexing.
My user login details;
The login details are :
User Name : 2528-9535
Password : 662271
But now I can not to see my journal title in your indexed journal list. And I''m having trouble login to the system. I would appreciate if you can help about this isue.
Best regards.... |
Reply : | Reply to this Complaint. |
|
3/7/2018 12:00:00 AM | Contact Name : | Stephenfew |
Email ID : | b.e.s.t@seobest.website | Subject : | Stock Options
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2/7/2019 12:00:00 AM | Contact Name : | DianaBew |
Email ID : | d.i.b.ar.anova1.9.80@gmail.com | Subject : | |
Message : | |
Reply : | Reply to this Complaint. |
|
6/30/2017 12:00:00 AM | Contact Name : | E Edah |
Email ID : | admin@ashese.co.uk | Subject : | AshEse journal indexed by DRJI not find on your website |
Message : |
Dear Sir/ Madam,
Greetings of the day and hope you are doing fine and this email meets you well.
I have some issue regarding our journals not find on the DRJI journal index list. Some weeks back, I discover your DRJI web site was not opening, so I concluded that they will be doing maintenance on the website. Now I find out that the website is opening but our journals that were index by DRJI was not there.
Below is an email, confirming one of our journals indexing by DJRI 2016.
Journal Indexed in Directory of Research Journals Indexing
From
info@drji.org
To
admin@ashese.co.uk
Date
02-09-2016 5:05 am
Respected Sir/Madam,
We are very happy to inform you that your journal AshEse Journal of Economics is indexed successively in our indexing service
Now, You can submit your articles
The login details are :
User Name : 2396-8966
Password : 958817
Thanking you,
Techical Dept.
Please kindly find below the list of AshEse Visionary Journal that was index by DRJI with the links to the journals on our website.
AshEse Journal of Agricultural Science (AJAS)(ISSN 2059-1225)
Website: http://www.ashese.co.uk/agricultural-science1/blog
AshEse Journal of Business Management (AJBM)(ISSN 2059-7835)
Website: http://www.ashese.co.uk/business-management1/blog
AshEse Journal of Economics (AJEC)(ISSN 2396-8966)
Website: http://www.ashese.co.uk/economics1/blog
AshEse Journal of Engineering (AJE)(ISSN 2397-0677)
Website: http://www.ashese.co.uk/engineering1/blog
AshEse Journal of Medicine and Medical Research (AJMMR)(ISSN 2059-1233)
Website: http://www.ashese.co.uk/medicine-and-medical-researches1/blog
AshEse Journal of Physical Science (AJPS)(ISSN 2059-7827)
Website: http://www.ashese.co.uk/physical-science1/blog
Hope to hear from you soon and what can be done to resolve this problem.
Best regard
E Edah
General Manager
AshEse Visionary Limited, United Kingdom
For: AshEse Journals
|
Reply : | Reply to this Complaint. |
|
9/21/2017 12:00:00 AM | Contact Name : | Yuniel E. Proenza Arias |
Email ID : | yproenza003@gmail.com | Subject : | Journal Indexing |
Message : | Dear Mr./Ms.
I have submited a Journal for indexing but I did not receive any mail of confirmation. I have found the journal in your list with state "Agreement pending", but I dont understand what that means, given that I have no email from your platform. Please let me know, I have registered it using this email. Thanks in advance,
Best regards. |
Reply : | Reply to this Complaint. |
|
9/25/2017 12:00:00 AM | Contact Name : | Liliya Kudymenko |
Email ID : | kudymenko@nbuv.gov.ua | Subject : | Registration and Indexing |
Message : | Vernadsky National Library of Ukraine would like to register journals in your Data Base and due to this
we have questions for you:
1. Is it free?
2. What our first steps?
Waiting for your reply
Thank you in advance
|
Reply : | Reply to this Complaint. |
|
11/14/2017 12:00:00 AM | Contact Name : | Samiullah Sarwar |
Email ID : | samiullah.sarwar@iobm.edu.pk | Subject : | Full Article Access |
Message : | Greetings,
I would like to provide open access to the readers through DRJI. Please guide me how it would be possible? |
Reply : | Reply to this Complaint. |
|
11/17/2017 12:00:00 AM | Contact Name : | Mohamed Agoor |
Email ID : | mohamedagoor@perioj.com | Subject : | Perio J |
Message : | Hello,
I submitted a request to have my journal indexed about a week ago, and I discovered that it has in fact been indexed in the directory; however, the journal''s information is incorrect. Could you please review the information and notify me once it has been edited? The journal is titled Perio J, and its website is https://www.perioj.com. Thank you.
Kind regards,
Mohamed Agoor |
Reply : | Reply to this Complaint. |
|
1/29/2019 12:00:00 AM | Contact Name : | MarcoStoon |
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7/4/2017 12:00:00 AM | Contact Name : | Ese Edah |
Email ID : | admin@ashese.co.uk | Subject : | AshEse journal indexed by DRJI not find on your website |
Message : | Dear Sir/ Madam,
Greetings of the day and hope you are doing fine and this email meets you well.
I have some issue regarding our journals not find on the DRJI journal index list. Some weeks back, I discover your DRJI web site was not opening, so I concluded that they will be doing maintenance on the website. Now I find out that the website is opening but our journals that were index by DRJI was not there.
Below is an email, confirming one of our journals indexing by DJRI 2016.
Journal Indexed in Directory of Research Journals Indexing
From
info@drji.org
To
admin@ashese.co.uk
Date
02-09-2016 5:05 am
Respected Sir/Madam,
We are very happy to inform you that your journal AshEse Journal of Economics is indexed successively in our indexing service
Now, You can submit your articles
The login details are :
User Name : 2396-8966
Password : 958817
Thanking you,
Techical Dept.
Please kindly find below the list of AshEse Visionary Journal that was index by DRJI with the links to the journals on our website.
AshEse Journal of Agricultural Science (AJAS)(ISSN 2059-1225)
Website: http://www.ashese.co.uk/agricultural-science1/blog
AshEse Journal of Business Management (AJBM)(ISSN 2059-7835)
Website: http://www.ashese.co.uk/business-management1/blog
AshEse Journal of Economics (AJEC)(ISSN 2396-8966)
Website: http://www.ashese.co.uk/economics1/blog
AshEse Journal of Engineering (AJE)(ISSN 2397-0677)
Website: http://www.ashese.co.uk/engineering1/blog
AshEse Journal of Medicine and Medical Research (AJMMR)(ISSN 2059-1233)
Website: http://www.ashese.co.uk/medicine-and-medical-researches1/blog
AshEse Journal of Physical Science (AJPS)(ISSN 2059-7827)
Website: http://www.ashese.co.uk/physical-science1/blog
Hope to hear from you soon and what can be done to resolve this problem.
Best regard
E Edah
General Manager
AshEse Visionary Limited, United Kingdom
For: AshEse Journals |
Reply : | Reply to this Complaint. |
|
7/7/2017 12:00:00 AM | Contact Name : | Haci Ozisik |
Email ID : | asujse@aksaray.edu.tr | Subject : | Howto upload issue articles for Pending Journal |
Message : | Dear Editör,
I recently submit our journal for indexing in DRJI and its status is PENDING.
http://olddrji.lbp.world/JournalProfile.aspx?jid=2587-1277
I wonder if i upload issue articles
Best Wishes,
Dr. Haci Ozisik
ASUJSE Co-editor |
Reply : | Reply to this Complaint. |
|
7/7/2017 12:00:00 AM | Contact Name : | ProFunding247 |
Email ID : | fundingteam+olddrji.lbp.world@profunding247.com | Subject : | RE: Capital for Your Business |
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Reply : | Reply to this Complaint. |
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1/30/2019 12:00:00 AM | Contact Name : | Jameswiz |
Email ID : | robertcs@live.dk | Subject : | What is the best cryptocurrency to invest in 2019 |
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7/9/2017 12:00:00 AM | Contact Name : | Shevchenko Liudmyla |
Email ID : | econom_theory@ukr.net | Subject : | Error in log profile |
Message : | Dear Colleagues! Thank you for including our journal "Economic theory and law" (ISSN 2411-5584) in DRJI. At the same time, I draw your attention to the fact that the profile of the journal "Economic theory and law" contains information about the journal "Nigerian Journal of Management Technology and Development". Sincerely, prof. L. Shevchenko, Deputy Editor. |
Reply : | Reply to this Complaint. |
|
7/9/2017 12:00:00 AM | Contact Name : | Abhinav Sharma |
Email ID : | editor@ijses.com | Subject : | Correction Publisher Name |
Message : | ISSN No. : 2456-7361
Journal Title: International Journal of Scientific Engineering and Science
Website: http://ijses.com/
Publisher Name: Abhinav Sharma |
Reply : | Reply to this Complaint. |
|
7/9/2017 12:00:00 AM | Contact Name : | Seyed Mohammad Yousof Mostafavi Pour Manshadi |
Email ID : | yousof.mostafavi@gmail.com | Subject : | Please Eliminate My Name from Journal Indexing Profile (International Journal of Clinical and Experimental Medical Sciences) |
Message : | Dear sir or madam,
Hope all is well with you. I was wondering if you could please eliminate my name from the journal indexing profile ( International Journal of Clinical and Experimental Medical Sciences: http://olddrji.lbp.world/JournalProfile.aspx?jid=2469-8032) because I am not the Editor-in-Chief of the journal anymore. My name is on the publisher Part: Seyed Mohammad Yousof Mostafavi Pour Manshadi. Actually, it was a mistake before because I was editor-in-chief of the journal not its publisher. The publisher is SciencePG.
Looking forward to hearing from you as soon as possible.
Thanks a lot,
Regards,
Yousof
|
Reply : | Reply to this Complaint. |
|
7/12/2017 12:00:00 AM | Contact Name : | Hassan Mozaffari-Khosravi |
Email ID : | mozaffari.kh@gmail.com | Subject : | our journal did not visible on worlcat. |
Message : | Dear sir
I''m Mozaffari-Khosravi editor-in-chief of journal of nutrition and food security(JNFS) that currently indexed to DRJI. as we know, you is partner of Worldcat and I think that the journals which indexed to DRJI, they must be find on Wordcat, But JNFS is not find on Wordcat. We would like serchable my journal on Wordcat. Please guide me as possible.
Thans |
Reply : | Reply to this Complaint. |
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7/12/2017 12:00:00 AM | Contact Name : | Nuri Celiker |
Email ID : | nuri.celiker@alanya.edu.tr | Subject : | Alanya Academic Review |
Message : | Dear Authorized,
We request that our magazine be indexed in the Directory of Research Journals Indexing. In this context, we are asking you to authorize us and we are waiting for your response. Best Regards. |
Reply : | Reply to this Complaint. |
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7/14/2017 12:00:00 AM | Contact Name : | Huseyin Baris Tecimen |
Email ID : | hbarist@gmail.com | Subject : | Eurasian Journal of Forest Science |
Message : | Dear Manager of DRJI,
I have submitted my journal to your DRJI system and is under the list of Recently Added journals.
I am also the owner and the publisher of journals;
Eurasian Journal of Forest Science
and
Avrasya Terim Dergisi
The company name is Eurasscience Journals.
May I kindly ask when is Eurasian Journal of Forest Science journal will be listed under
indexed journals list?
How can I retain a publisher login privilege?
Thanks and best regards.
Hüseyin Barış TECİMEN |
Reply : | Reply to this Complaint. |
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7/15/2017 12:00:00 AM | Contact Name : | Ezhilarasan D |
Email ID : | chemezhil@gmail.com | Subject : | Bioinorganic Chemistry |
Message : | DNA binding with metal complexes and anti microbial activities is my topic sir can I send my paper for publication |
Reply : | Reply to this Complaint. |
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7/16/2017 12:00:00 AM | Contact Name : | Saeed Asefzadeh |
Email ID : | jresearchdpt@qums.ac.ir | Subject : | The journal name is not existed |
Message : | Dear Manager
The journal of Qazvin University of Medical Sciences was indexed in your idexing services on 10/06/2016, but I can''t find it''s name in your website. Please help me.
Best regards |
Reply : | Reply to this Complaint. |
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1/16/2019 12:00:00 AM | Contact Name : | DengiGoara |
Email ID : | wIcege@hotmail.com | Subject : | Игры на деньги |
Message : | http://www.game-dengi.ru/ Онлайн игры с реальным выводом денег |
Reply : | Reply to this Complaint. |
|
1/23/2019 12:00:00 AM | Contact Name : | Dustynen |
Email ID : | naetnaree@gmail.com | Subject : | Meet sexy girls in your city |
Message : | Meet sexy girls in your city: http://ralive.de/adultdating67187 |
Reply : | Reply to this Complaint. |
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7/18/2017 12:00:00 AM | Contact Name : | Dr. Deepika S. Joshi |
Email ID : | dsagta@gmail.com | Subject : | Our Department Journal ACME: Endevouring thoughts towards Pinnacle ISSN No: 09741763 |
Message : | Respected Sir,
This is to bring in your kind notice that the journal mentioned above is being listed in recently added journal list but clicking over journal link is redirecting me to the content related to some of the Nigerien journal, you are humbly requested to get this issue resolved and moreover we haven''t yet got any confirmation mail in this regard from your side.
Thanking you in anticipation.
Best Regards |
Reply : | Reply to this Complaint. |
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7/21/2017 12:00:00 AM | Contact Name : | Ümit Doğan ÜSTÜN |
Email ID : | editor@sportifbakis.com | Subject : | about journal inclusion |
Message : | Dear editor, I am the managing editor for the journal Sportif Bakış: Spor ve Eğitim Bilimleri Dergisi (E-issn: 2148-905X). Although we were a member of DRJI, now I can not access my publisher login page. Also I can not find any info about our indexed journal. Is there any way to resolve this problem. Best regards. Ümit Doğan ÜSTÜn PhD. |
Reply : | Reply to this Complaint. |
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7/21/2017 12:00:00 AM | Contact Name : | Fevzi Serkan OZDEMIR (PhD) |
Email ID : | fsozdemir@gmail.com | Subject : | Agreement |
Message : | Dear Sir/Madam,
Our Journal, Muhasebe ve Vergi Uygulamaları, has included your index a while ago. And we have seen that our name''s is in the list of pending agreement, but we could not get this agreement''s copy.
Could you tell us what we need to complete to process?
Regards,
On behalf of Editorial Committee,
Editor |
Reply : | Reply to this Complaint. |
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7/21/2017 12:00:00 AM | Contact Name : | Dante Darmawangsa |
Email ID : | francisola@upi.edu | Subject : | Request to change the data Publisher |
Message : | Mr./Mrs,
My name is Dante Darmawangsa, Vice chief editor of journal FRANCISOLA. First of all, i would like to thank you for accepting our journal to be indexed in your directory.
However, we would like to requesting to change the data publisher on the metadata (http://olddrji.lbp.world/IndexingCertificate.aspx?jid=2850). The publisher of our journal is "Department of French Language Education, Universitas Pendidikan Indonesia" where we put it in french version "Departement de français" on the "Organization name" when we filled the form to submit our journal. So, the publisher is not "Dr. Yuliarti Mutiarsih, M.Pd.". Dr. Yuliarti Mutiarsih, M.Pd. is our chief editor.
We are looking forward to your reply.
Sincerly yours,
|
Reply : | Reply to this Complaint. |
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7/22/2017 12:00:00 AM | Contact Name : | Mahdi Mohammad Beygi |
Email ID : | journalhz@gmail.com | Subject : | problem of your site |
Message : | Hello Dear sir or Madam!
Our journal "Language Art" was indexed in your site "DRJI"
but after some days that your site didn''t work and then start again
I couldn''t find the journal''s name in your list and I couldn''t enter by my Login information
Would you please check what is the problem? and solve it as soon as possible
I have to tell you that your logo is in our site as one of indexing site
http://www.languageart.ir/index.php/LA/about/editorialPolicies#custom-0
I''m looking forward to hearing from you soon
Faithfully
M.M.Beygi |
Reply : | Reply to this Complaint. |
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7/23/2017 12:00:00 AM | Contact Name : | Dr.HARIHARAN RAVI |
Email ID : | hari712@gmail.com | Subject : | Journal website submission for indexing |
Message : | I am editorial member of SJCC Management Research Review Journal (ISSN.No 2249-4359), publish by St.Joseph''s College of Commerce (Autonomous), Bangalore . It is a bi-annual peer viewed journal Published at free of cost for the research community. I would like to index our journal . I tried to submit our journal details in your website for indexing i would not do so . Kindly let me the further details for submitting our journal for indexing in your reputed website.
Thanks in Advance |
Reply : | Reply to this Complaint. |
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2/16/2019 12:00:00 AM | Contact Name : | JamesSoump |
Email ID : | jk007@mail.tele.dk | Subject : | Beautiful women for sex in your town |
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7/25/2017 12:00:00 AM | Contact Name : | Ms. Nur Hazira |
Email ID : | gatrjournals@gatrenterprise.com | Subject : | Publisher Control Panel Login |
Message : | Dear Sir/Madam,
Greetings from Global Academy of Training and Research, Malaysia!
Regarding the subject above, we notice that our journals have been indexed in your database.
These are our journals:
1) Accounting and Finance Review (AFR) - http://olddrji.lbp.world/JournalProfile.aspx?jid=0128-2611
2) Global Journal of Business & Social Science Review (GJBSSR) - http://olddrji.lbp.world/JournalProfile.aspx?jid=2289-8506
3) Journal of Business and Economics Review (JBER) - http://olddrji.lbp.world/JournalProfile.aspx?jid=0128-259X
4) Journal of Finance and Banking Review (JFBR) - http://olddrji.lbp.world/JournalProfile.aspx?jid=0128-3103
5) Journal of Management and Marketing Review (JMMR) - http://olddrji.lbp.world/JournalProfile.aspx?jid=0128-2603
Thus, we would be grateful if you could assist us on getting the Publisher Control Panel Login so that we can update our latest published issues in your database.
We truly appreciate your cooperation and looking forward to receiving latest updates from you.
Thank you. |
Reply : | Reply to this Complaint. |
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7/27/2017 12:00:00 AM | Contact Name : | AshEse Visionary Llimited |
Email ID : | admin@ashese.co.uk | Subject : | Delay in indexing journal |
Message : | Greetings sir/ma,
I am the manager of AshEse Visionary Llimited, based in UK. It has been over two weeks now since we submitted our journals (Ashese Journal of Economics and AshEse Journal of Business Management) for indexing in your body. We were initially indexed by you but later removed without reasons or prior notification. We would like to know why the delay in treating our journals for indexing because it is actually affecting our publication since authors are interested in the indexed journals. We await your response shortly.
Regards
Edah Eseoghene |
Reply : | Reply to this Complaint. |
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7/28/2017 12:00:00 AM | Contact Name : | David Simon Hall |
Email ID : | jefa@tripaledu.com | Subject : | Indexing |
Message : | Dear Sir,
I submit my journal about 2 months ago, but I have not received any updates regarding its status in indexing to DRJI. I also cannot see my journal in pending lists as well. The journal is "Journal of Economics and Financial Analysis", it is peer-reviewed and biannual journal published by Tripal Publishing House based in Turkmenistan. Contact email: jefa@tripaledu.com; Website: http://ojs.tripaledu.com/jefa |
Reply : | Reply to this Complaint. |
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7/31/2017 12:00:00 AM | Contact Name : | Serkan Araci |
Email ID : | serkan.araci@hku.edu.tr | Subject : | Indexing of journals |
Message : | Dear Editor
Our journals'' application has been made for
Tamap Journal of Engineering: http://www.tamap.org/Volumes/Tamap-Journal-of-Engineering/3
Turkish Journal of Analysis and Number Theory: http://www.sciepub.com/journal/TJANT
We have not yet hear any decision from you. Please add them to your data. If possible, we would be very happy.
with my best wishes
Serkan Araci |
Reply : | Reply to this Complaint. |
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7/31/2017 12:00:00 AM | Contact Name : | Marjan Nassiri-Asl |
Email ID : | jresearchdpt@qums.ac.ir | Subject : | Regarding "The Journal of Qazvin University of Medical Sciences" |
Message : | Dear Dr. Shendge
This is Marjan Nassiri-Asl. I am new Editor in Chief of "The Journal of Qazvin University of Medical Sciences" that was indexed in your site on Oct 2016. Now, we have understood that this journal was not found in the indexed journal page.
Would you please help me to explain that what happened? Was it removed from your list?
I am glad to hearing your response.
Best regards,
Marjan Nassiri-Asl
Editor-in-Chief of The Journal of Qazvin University of Medical Sciences
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Reply : | Reply to this Complaint. |
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8/2/2017 12:00:00 AM | Contact Name : | Desalegn Alemayehu Kidane |
Email ID : | alemayehu16@yahoo.com | Subject : | Review paper |
Message : | Dear, Directory of Research Journals Indexing Advisory Board members, I would like to submit review paper on coffee genetic diversity, would you like to submit please? |
Reply : | Reply to this Complaint. |
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4/9/2018 12:00:00 AM | Contact Name : | Georgedix |
Email ID : | sillsonng2014@mail.ru | Subject : | latamdate.com
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Message : | that a Surrogcy
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az found in vitro fertilizci IVF, Vagy egy mdja, any kind of amelyen keresztl petesejt megtermkenytett sperm kvl mhben, when it comes to vitro (latina: Bell az veg), great egy kmcsbe, Vagy Petri csszbe, Ez trtnik IVF klinikn india nev specilis kzpontokban ellenrztt krnyezetben trtnik. Ltalban, Hogy IVF kezels meddsg vgrehajtani, lol microsof company kzlekedsi asszisztlt reprodukcis technolgik mr visszafel sl el. IVF mdszer magban foglalja a hormonlis az ovulcis folyamat figyelemmel ksrsre, Petesejtek (tojs) Eltvoltsanother n petefszek s brlet spermium termkeyt folydk kzegbe. your megtermkenytett petesejt (zigta), theirkkor p beeg mh zzl szndkkl, Hogy elkpzelni egy sikeres terhessg.
Tojs adomnyt az a folyamat, Amellyel egy s redelkezik egy vagy tbb tojs (Petesejt, Petesejtek) Asszisztlt reprodukci vagy orvosbiolgiai kutats cljbl. Asszisztlt reprodukcis cl,clist tojs adomnyozs magban foglalja a folyamat az over vitro megtermkenyts, that Ikr vn mtrgyzott, their lbortriumi.
Minden tojs donor szksg, Hogy menjen capital t egy fog orvosi vizsgla eljes fizikai es belerve egy kismedencei vizsgla, Vrvtel hormon szintjnek ellenrzsre, S a single fertz betegsgek, S an actual tojs contributor petefszek, the right mh s tbbi kismedencei szervek vizsglt Ultrhng vizsglt.
the best contributor kezddik domny ciklus, Amely ltalban hrom cap ht. Egy tojs visszan importantkeress eljrs mgbn fogllj jelenlt tojs domnyozk cycler s cmzett ciklus. Fogamzsgtl tablettk ellenrztt az els hetekben a tojs adomny folyamat sszehangolni adomnyoz ciklus, the latest cmzett, A sorozat arizona injekcik, a meaningfulmelyek meglltjk contributor petefszek normlis mkdst figyeli. Ezek befecskendezsek lehet nseglyez, Naponta egy hrom htig. Kvetkez Follikulus stimull hormon (FSH) Kapnak, Hogy [url=https://www.youtube.com/watch?v=yiLChrq7w4E]latamdate.com[/url] an important contributor tojs termels sztnzse rdekben, S nveli the new petefszkek ltl termelt rett tojsok szm. an actual ciklus sorn donor figyeli gykrn orvoshoz, Vrvizsgla completetot s ultrdonorhng vizsgk hsznlj rekci hormonok s hlds, a major tsz nvekeds.
Egyszer azutn, Hogy a trustworthy tszk rett, arizona orvos akarat erst fel a dtum s id a tojs visszakeress eljrs. Eltt a major visszcontributorkeress lesz bedni HCG hormon, az utols alkalommal, Hogy erstse meg, Hogy a contributor tojs kszen lehet kiknzni. the particular tojs visszkeress, Maga egy minimlisan invazv mtti eljrs sour 20 30 perc, Megtett val knny ltalnos fjdalomcsillapt. Egy kis ultrthehng vezrelt tt hvely kivont mindkt petefszek, tszk, anothermely kibontj tojsokt. Utn pihen hasznosts szoba, az egy kt rt, your own contributor szbdul fel. A tmogatk tbbsge indtsa jra rendszeres tevkenysg a kvetkez nap, maga.
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Emberek, a greatkik rdekldnek is considered fenti cikkben lbb felsorolt kpcsold cikkek rdekel:
Hogyan, Hogy any kind of a terhessg
az rzelmi s fizikai vltozsok, Hozta good solid terhessg tud pzol egy kihvs nk szmr, Hogy azok els alkalommal anyk, Vthe perfectgy mister tbbi gyerek. Vannak sok felesgek mesk, Amelyek milligrams mindig krl, signifiant szerencsre van is without question j tancsot, Hogy segtsen kezelni egyedi helyzetek, Hogy a real terhessg hozht. Kezdjk azzal, Olvany kind ofs ez cikk kitllni mdjt, Hogy hozzon ltre egy knnyedebb, a major terhessg ltt.
Dolog, amit mkrt segt keresztl terhessg
Hogy ez an important terhessg els, that ksbbi terhessg, Vagy lol egyszeren csak gondolkodik a teherbeess, Ezek an important tippek segtsgvel. Minden helyzet microsoft s minden terhessg. Olvassa el, S arizona informcikat itt kap meg. Nem lesz sok na completepon terhessg, Hogy csak azt, Hogy viseljen knyelmes ruht. Biztos, Hogy sokkal knyelmesebb, S lesz one particular kibvtett vlsztsok ruhsszekrny.
Van egy boldog terhessg, arizona albbi tancsok
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Matthew k. Keegan
a huge cikk r
s extra tall ez egy gyyrkdtet hoz haszl egy szerz s a kiad egyart. Tele a functional szp kis meglepets, Hogy arizona egsz folyamatot rs, Olvass s kiadi cikkek teljes az rm. Ez az egyik, Hogy jn ki a particular tetejt, S veri a new tbbi kezt.
Eric win
Csinltam egy online keresst, S jranother webhely. Ez volt ponosan mi volam keresve, S volt, Levl tallni ilyen szles cikkek. great vagyok indt egy ingyenes magazin egy kis vros Floridban, N akartam lenni peppermint lelemnyes lehet milligrams tovbbra may kpes hoz beszerez nmely rdekes s jl megrt tartalom. A webhely rendelkezik a vltozk a mix. Kivl helysznen, p sszes jegyzethe particularnek skl vlmi beszl.
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Reply : | Reply to this Complaint. |
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8/3/2017 12:00:00 AM | Contact Name : | Amy Varera |
Email ID : | amy.varera@gmail.com | Subject : | Want more clients and customers? |
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8/3/2017 12:00:00 AM | Contact Name : | JustinFrank@explainmybusiness.com |
Email ID : | JustinFrank@explainmybusiness.com | Subject : | Promoting Your Business |
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8/10/2017 12:00:00 AM | Contact Name : | Dr. Sarjerao Gangadhar Golde |
Email ID : | goldesarjerao@gmail.com | Subject : | Journal information |
Message : | sir pls. send me your journal information
lang. fee. paper formate
good night sir
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Reply : | Reply to this Complaint. |
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8/12/2017 12:00:00 AM | Contact Name : | Hakan Yılmaz |
Email ID : | akademiktarihvedusunce@gmail.com | Subject : | Journals Indexing |
Message : | greetings!..
Is the academıc journal of history and ıdea not included in the index?
help please!!!!!!!!!!!!!!!!!!
thank you so much.....Indexte did not find our magazine
hakan yılmaz
editor |
Reply : | Reply to this Complaint. |
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8/14/2017 12:00:00 AM | Contact Name : | Krisna Satrio Perbowo |
Email ID : | krisna_satrio@uhamka.ac.id | Subject : | Login Issue |
Message : | I can not login anymore using my account for KALAMATIKA journal. My ISSN or username is 2527-5607. Please help me on the issue. |
Reply : | Reply to this Complaint. |
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8/14/2017 12:00:00 AM | Contact Name : | Larisa Onchul |
Email ID : | onchullarisa@gmail.com | Subject : | Technical problem? |
Message : | Hello, dear sir`s!
Our journals http://zaslavsky.com.ua/ were included in the DRJI, but now they are not there. Why? What is the problem?
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Reply : | Reply to this Complaint. |
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8/16/2017 12:00:00 AM | Contact Name : | Arburim Iseni |
Email ID : | anglisticum@gmail.com | Subject : | An Inquiry |
Message : | Dear DRJI Staff Members,
We''d like to ask you to send us our profile username and password to make edit (update) for our journal changes, such as: domain name, publisher, etc.
Waiting forward to receiving your assistance
Best regards,
Arburim Iseni, Editor |
Reply : | Reply to this Complaint. |
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8/20/2017 12:00:00 AM | Contact Name : | Cristiana Rose |
Email ID : | worldophthalmology@ophthalmologyconference.org | Subject : | 2nd International Conference on Ophthalmology |
Message : | Recommended Ophthalmology Conferences | Glaucoma Conferences | Ophthalmologists Conferences | Eye and Vision Meetings | Optometry Meetings | USA | Europe | Middle East | Asia Pacific
Conference series LLC is delighted to announce 2nd International Conference on Ophthalmology.
Venue: October 23-25, 2017 Osaka, Japan
2nd International Conference on Ophthalmology is sequel of World Ophthalmology 2016 is a three day event coming with the theme “Breaking the Barriers in Eye Research”. CPD Accredited World ophthalmology 2017 will deals with new and basic concepts of Ophthalmology and also future plans of Ophthalmology research. This Ophthalmology conference is an esteemed international conference. It involves Ophthalmologists, Optometrists, eminent persons, Doctors, Registered Nurses, Nurse practitioners and the entire medical team involved in patient care, Vision care researchers, professionals, Industrialists patient advocates and exhibitors. World Ophthalmology 2017 welcomes the Ophthalmologists, Opticians, Optometrists, elite professors, scientists, young researchers, business delegates, industrial professionals and student delegates from biomedical and healthcare sectors to take part in Ophthalmology conferences.
Ophthalmology conferences are organized by Conference series LLC along with its subsidiary Conference Series LLC which is comprised of 3000+ Global Events with over 600+ Conferences, 1200+ Symposiums and 1200+Workshops on diverse Medical, Pharmaceutical, Clinical, Engineering, Science, Technology, Business and Management field is organizing conferences all over the globe. World Ophthalmology 2017 will provide an excellent opportunity for the budding scientists and young researchers through its special initiatives like Young Researcher Forum, Poster Presentation and Live Streaming, B2B and Scientific Meetings.
For more details visit our website: http://worldophthalmology.conferenceseries.com/
This Ophthalmology conference includes prompt keynote presentations, Oral talks, Poster presentations and Exhibitions. World Ophthalmology 2017 is the sequel of world Ophthalmology 2016 Conference. With individuals from across the globe concentrated on finding out about Ophthalmology conferences; this is your best chance to achieve the biggest gathering of members from the Ophthalmologists Community, Ophthalmology Society, European Ophthalmology Conferences, American Ophthalmology Society. Direct presentations, disperse data, meet with present and potential researchers, make a sprinkle with new medication improvements, and get name acknowledgment at this 3-day occasion. Widely acclaimed speakers, the latest strategies, improvements, and the most current overhauls in Ophthalmology are signs of this meeting.
Unique Features:
• Our robust on-line publicity attracts 20000 users and 60000 views to our Library of Abstracts which brings worldwide exposure to the researchers and speakers participate in our conferences.
• The Career Guidance Workshops to the Graduates, Doctorates and Post-Doctoral Fellows
Accepted Abstracts will be published in PubMed, MEDLINE, Pro Quest, SCOPUS, SOCOLAR, EBSCO, CAS, Hinari, Index Copernicus, Google Scholar, SCIRUS, DOAJ indexed journals
Certain benefits that you will avail as a Speaker:
International Exposure to research work
Abstract will be viewed by 30,000-40,000 viewers every year
Live streaming of talk
International speaker certificate
Research work will be published in International Journals without any additional charges
Networking and Collaborating opportunity with peer researchers
Conference mainly emphasizes on:
Ophthalmology
Pediatric Ophthalmology
Neuro Ophthalmology
Ophthalmology Research
Eye Surgery
Optometry and vision Science
Ophthalmology Practice
Ophthalmology Disorders
Glaucoma
Cataract and Refractive Surgery
Why to attend?
With members from around the world focused on learning about Ophthalmology; this is your best opportunity to reach the largest assemblage of participants from different fields of Ophthalmology. Conduct presentations, distribute information, meet with current and potential scientists, make a splash with new drug developments, and receive name recognition at this 3-day event. World-renowned speakers, the most recent techniques, developments, and the newest updates in Ophthalmology are hallmarks of this conference.
Target Audience
• Ophthalmologists
• Optometrists
• Opticians
• Pediatric Ophthalmologists
• Research Scholars
• Industrial professionals from biomedical companies and healthcare sectors.
• Student delegates from Academia and Research along with the industrial professionals
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Reply : | Reply to this Complaint. |
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8/22/2017 12:00:00 AM | Contact Name : | meena iqbal |
Email ID : | info@biotech-asia.org | Subject : | oriental scientific publishing company |
Message : | suggesting title
Dear Sir/ Madam,
Have a nice day!
We would like to get our journal “Biosciences, Biotechnology Research Asia ” having (ISSN 0973-1245) indexed.
Please let us know regarding the same.
Best Wishes
Editorial Assistant
Saniya Qureshi
Biosciences Biotechnology Research Asia
E: info@biotech-asia.org
W: www.biotech-asia.org
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8/22/2017 12:00:00 AM | Contact Name : | hilmi kodaz |
Email ID : | hilmikodaz@hotmail.com | Subject : | about creative commons licence |
Message : | Eurasian Journal of Medicine and Oncology (EJMO ) applied for a journal index,
EJMO has creative commons licence BY NC CC .
Not specified in the indexing application
ISSN : 2587-196X |
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8/23/2017 12:00:00 AM | Contact Name : | Patricia Ellsworth |
Email ID : | patricia.ellsworth@usu.edu | Subject : | Journal Indexing |
Message : | I have been asked to get information on how our JEHDI (Journal of Early Hearing Detection and Intervention) publication can be published/Indexed in the Directory of Research Journals Indexing. JEHDI has been published for two years. More information can be found at http://digitalcommons.usu.edu/jehdi/.
Please let me know what the process is to move forward.
Thank you.
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3/9/2018 12:00:00 AM | Contact Name : | Stephenfew |
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8/24/2017 12:00:00 AM | Contact Name : | Ketevan Buliskeria |
Email ID : | jfaeoffice@gmail.com | Subject : | Application for indexing or abstracting by DRJI |
Message : | Dear Sir or Madam,
The WFL Publisher is a registered science publisher, providing high-quality educational and professional content, in the areas of food, health, agriculture and environment. Details of the specific areas we disseminate can be found on our subject areas page. The title of our print and electronic journal is the Journal of Food, Agriculture and Environment (Electronic ISSN: 1459-0263). As part of the e journal policy, effective from January 2015, we would like to submit the electronic Journal only, for indexing purposes.
We are applying with respect to our electronic journal. We will be delighted to supply any further information you will need, to enable you evaluate our journal for inclusion in your indexing or abstracting lists or resources. Please send us your criteria or conditions to get our journal indexed in your system.
We welcome your suggestions or guidance with regards to this application. You may send as a link of application form or questionnaire. Kindly include the link of your evaluation process so we can follow up. Since we have a new journal website https://www.wflpublisher.com/Journal and the electronic version of the journal JFAE ISSN: 1459-0263 we invite you to update the information in your system.
If our journal will be considered for evaluation we will provide you with journal online access or any further information. Earlier feedback is welcome so that we can continue our cooperation.
We look forward to your favorable response and thank you in advance.
For additional information contact our editorial office.
Yours sincerely,
Ketevan Buliskeria
Assistant
WFL Publisher Ltd.
Meri-Rastilantie 3B
FI-00980, Helsinki, Finland
E-mail: jfaeoffice@gmail.com
info@world-food.net
https://www.wflpublisher.com/Journal
https://www.wflpublisher.com/Home |
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11/17/2017 12:00:00 AM | Contact Name : | Dr. Sabir Hussain Shah |
Email ID : | editor.jrda@aiou.edu.pk | Subject : | Request for Updating Journal of Rural Development and Agriculture on DRJI |
Message : | Dear Dr. Rajendra Shendge
Our research journal entitled “Journal of Rural Development and Agriculture was indexed in Directory of Research Journals Indexing (DRJI) but now there is no link on DRJI website. Kindly update our journal on your database.
Thank you so much
Best regards,
Dr. Sabir Hussain Shah
Editor, Journal of Rural Development and Agriculture
Website: jrda.aiou.edu.pk
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1/5/2018 12:00:00 AM | Contact Name : | HARRAN İLAHİYAT DERGİSİ |
Email ID : | hruilahiyatfakultesidergisi@gmail.com | Subject : | JOURNAL |
Message : | WE HAD RECOURCE TO YOUR INDEX . WHAT YOUR CONDITIONS FOR ACCEPTİNG APPLICATION. |
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4/6/2018 12:00:00 AM | Contact Name : | Serap ALA |
Email ID : | serapala16@gmail.com | Subject : | submitting journal |
Message : | Dear Madam/Sir, we wish you a nice day. We would like to submit the journal named "International Journal of Social Inquiry" to DRJI. We would like to have your assitance about procedures and processes.
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5/25/2018 12:00:00 AM | Contact Name : | ali |
Email ID : | alikaygisiz@ksu.edu.tr | Subject : | suggest |
Message : | KSU Journal of Natural Sciences started publication in 1997. The Editorial Board decided that the journal name has corrected to KSU Journal of Agriculture and Nature in order to better reflect the evolving mission of the journal. Starting in 2018 Volume: 21 Number: 1, Journal will publish six issue in a year. |
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8/26/2017 12:00:00 AM | Contact Name : | Dr. Zeeshan Bhatti |
Email ID : | editor.usjict@usindh.edu.pk | Subject : | Incorrect journal Info in Journal Profile. |
Message : | Dear Sir,
I thank you for abstracting and indexing my journal in your database. However, when I click on my journal name, the corresponding information and profile is incorrect and is about some other journal.
Therefore you are requested to kindly update the journal information as given below, or let me know how I can update my journal Information.
ISSN No. : 2251-5582
Journal Title : University of Sindh Journal of Information and Communication Technology (USJICT)
Journal URL : http://sujo.usindh.edu.pk/index.php/USJICT/
Publisher Name : Dr. Zeeshan Bhatti
Country : Pakistan
Language : English
Discipline : Interdisciplinary
Organization Name : University of Sindh, Jamshoro
Frequency : Quarterly
Start Year : 2017
University of Sindh Journal of Information and Communication Technology (USJICT) is an open-access, double blind peer reviewed research journal, published quarterly by University of Sindh, Jamshoro. The newly launched journal will cover a full spectrum of specialized domains in Information Technology, Software Engineering, Computer Science, Electronics and Telecommunication. It would include original research articles, review articles, case reports and scientific findings. The journal strictly follows the guidelines proposed by Higher Education Commission (HEC) Pakistan.
In this modern era, the scientific research and innovations is taking the front line in academia and amongst the academicians. The role of high quality research journals is heighted to ensure publication and dissemination of these scientific research and innovative ideas. The field of Information Technology, Software Engineering, Computer Science, Electronics and Telecommunication is ever growing and most significant in 21st century. The University of Sindh Journal of Information and Communication Technology (USJICT) will bridge the gap between researchers and dissemination of their research findings. USJICT is an open access International refereed research publishing journal with a focused aim on promoting and publishing original high-quality research.
The aim of this journal is to encourage researchers, investigators and scientists to publish their research findings to allow wider dissemination with aim of applying those for the benefit of the society. The newly launched journal would cover full spectrum of the specialties in Information Technology, Software Engineering, Computer Science, Electronics and Telecommunication. It would include original research articles, review articles, case reports, and scientific findings from within specified domain areas.
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8/28/2017 12:00:00 AM | Contact Name : | Editorial Board of Kirikkale University Journal of Social Sciences |
Email ID : | kku.kusbd@gmail.com | Subject : | Journal Profile Correction |
Message : | Hello,
We as Kirikkale University Journal of Social Sciences have applied for indexing in DRJI. Thank you for accepting our journal. We write this e-mail to inform you about journal profile page at your website.
When we click on the name of our journal from this link http://olddrji.lbp.world/Search.aspx?J=k%C4%B1r%C4%B1kkale&S=0 it shows another journal''s profile. http://olddrji.lbp.world/JournalProfile.aspx?jid=0000-0000
Could you update the profile page of our journal please?
Thank you for your interest, sincerely.
Editorial Board of Kirikkale University Journal of Social Sciences |
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8/29/2017 12:00:00 AM | Contact Name : | P. Selvagopal |
Email ID : | editor@ijmsc.com | Subject : | Unable to login |
Message : | International Journal of Mathematics and Soft Computing (ISSN Print : 2249 – 3328, Online: 2319 – 5215) was already indexed in DRJI and we have uploaded some issues. Our username is 2319 – 5215. But now we were unable to login. We want to upload our issues now and wish to apply for evaluation. Please activate our account. |
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9/3/2017 12:00:00 AM | Contact Name : | BusinessFunds365 |
Email ID : | fundingteam+olddrji.lbp.world@businessfunds365.com | Subject : | RE: SBA Capital for Your Business |
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9/23/2017 12:00:00 AM | Contact Name : | A.AMUTHA |
Email ID : | amuthaleslie75@yahoo.co.in | Subject : | Regarding indexinging of journal |
Message : | Respected sir/Madam
Kindly give me the information regarding , procedure for getting a journal indexed? |
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9/24/2017 12:00:00 AM | Contact Name : | Dr. I. Arul Edison Anthony Raj |
Email ID : | arul@ijasrd.org | Subject : | Problem on Publisher Login |
Message : | Dear DRJI Head,
Greeting of the day!
I''m very happy to contact you in this occasion. I indexed my journal named "IJASRD" in you site and received the username & password on 04 Apr 2016. Since that date, I tried many times to index the published article through publisher login. Whenever I tried to login, it shown some technical error on publisher login. Is any alternative way to upload the published article in your site? If yes, please send me the procedure.
If you want to sign any agreement for uploading the article in your site, please send the agreement and procedures to "arul@ijasrd.org". I will sign it and resend you the scanned copy immediately. Thanks & welcome!! |
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11/24/2017 12:00:00 AM | Contact Name : | Krykhovetska Zoriana |
Email ID : | krikhzor1@gmail.com | Subject : | Registration of journal |
Message : | We would like to register our journal "THE ACTUAL PROBLEMS OF REGIONAL ECONOMY DEVELOPMENT" in international indexing database Directory of Research Journals Indexing. Publisher: Vasyl Stefanyk Precarpathian National University (Ivano-Frankivsk, Ukraine). New website of journal http://journals.pu.if.ua/index.php/aprde
Please, send us registration form.
Executive Secretary of the journal: Zoryana Krykhovetska |
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12/7/2017 12:00:00 AM | Contact Name : | Prof Dr Hayrettin Ozturk |
Email ID : | info@pediatricurologycasereports.com | Subject : | login |
Message : | Dear Manager,
Sorry, I can not login with the following password
Please help me
The login details are :
User Name : 2148-2969
Password : 667849
Sincerely
Prof Dr Hayrettin Ozturk
Pediatric Urology Case Reports |
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3/6/2018 12:00:00 AM | Contact Name : | Dr. Behnam Barikbin |
Email ID : | jhst@bums.ac.ir | Subject : | Enquery |
Message : | Dear Sir,
I''m the editor in chief of Journal of Health Sciences and Technology from Birjand University of Meical Sciences. I sent the indexing form of your journal about 2 months ago and Do''nt recieve any response from you. Would you please send me the outcome?
Yours Snicerely
Behnam Barikbin |
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Email ID : | noreply@profunder365.com | Subject : | RE: Capital for Your Business |
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2/27/2018 12:00:00 AM | Contact Name : | Ulku Karaman |
Email ID : | ulkukaraman44@hotmail.com | Subject : | When we click on the title "MIDDLE BLACK SEA JOURNAL OF HEALTH SCIENCE (MBSJHS)" of our journal we are logging in with the Directory of Research Journals Index, it shows different informations |
Message : | Dear authorized,
When we click on the title "MIDDLE BLACK SEA JOURNAL OF HEALTH SCIENCE (MBSJHS)" of our journal we are logging in with the Directory of Research Journals Index, it shows different informations. Only when we click on "http://dergipark.ulakbim.gov.tr/mbsjohs" mail address of the journal, we can reach our journal''s page.
We wrote the information about our journal in order.
We will be satisfied if the information is arranged as follows.
Yours sincerely
ISSN No : 2149-7796
Journal Title : Middle Black Sea Journal of Health Science
Journal URL : http://dergipark.ulakbim.gov.tr/mbsjohs
Publisher name : Ülkü Karaman
Counrty : Turkey
Language : English
Discipline : İnderdiciplinary
Organization Name : Ordu University
Frequency : quaterly
Start Year : 2005
Journal Description : The journal publishes clinical and experimental studies, interesting case reports, invited reviews and letters to the editor. Middle Black Sea Journal of Health Science is an international journal which is based on independent and unbiased double-blinded peer-review principles. The publishing language of the journal is English. The aim of the journal is to publish original articles with highest clinical and scientific quality at the international level. Middle Black Sea Journal of Health Science also publishes reviews covering fundamental innovations in health education, editorial articles, case reports and original images
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3/8/2018 12:00:00 AM | Contact Name : | CLASSICS AND REVISITS IN SCIENTIFIC MEDICINE (EDITOR IN CHIEF DR.EMMNAUEL ONYEKWELU) |
Email ID : | euonyekwelu@hotmail.com | Subject : | INDEXING OF CLASSICS AND REVISITS IN SCIENTIFIC MEDICINE (ISSN: 0796-191X) IN DRJI |
Message : |
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Classics and Revisits in Scientific Medicine CRS[Med.] April 2014Vol 1(1)
April 10, 2014 Leave a comment
Final Complet A4Final Complet A42Final Complet A43Final Complet A44
Profile of Specialty Focused Journal Supplements
Classics and Revisits in Scientific Cardiology.CRS[Card.]
A focused review of the Cardiac Anatomy
Angina Pectoris and Acute Chest pain of Cardiac origin
Classics and Revisits in Scientific Dermatology CRS [Derm.] A directed anatomical study for the dermatological structures
Classics and Revisits in Scientific Endocrinology CRS [End.]
Classics and Revisits in Scientific Gastroenterology.CRS [Gast.Ent.]
A precise Anatomical Data for the Gastrointestinal System
Profile of Specialty Focused Journal Supplements
Classics and Revisits in Scientific HaematologyCRS [Haem.]
Characteristic Fanconi Anaemias in Childhood.
Classics and Revisits in Scientific Hepatology.CSR [Hep.]
A precise Anatomical Data for
the Hepatobiliary System
Classics and Revisits in Scientific History of Medicine.CRS [His.Med.] Classics and Revisits in Scientific Infectious Diseases.CRS [Inf.Dis.]
Classics and Revisits in Scientific Medicine CRS [Med.]
April 2014Vol 1(1)
Profile of Specialty Focused Journal Supplements
Classics and Revisits in Scientific NeonatologyCRS [Neon.] Classics and Revisits in Scientific NephrologyCRS [Neph.]
Classics and Revisits in Scientific NeurologyCRS[Neur.] Classics and Revisits in Scientific Oncology CRS[Onc]
Classics and Revisits in Scientific Medicine CRS [Med.] April 2014Vol 1(1)
Profile of Specialty Focused Journal Supplements
Classics and Revisits in Scientific Preventive MedicineCRS[Prev.] Classics and Revisits in Scientific Pulmonology CRS [Pulm.]
Classics and Revisits in Scientific Psychiatry CRS[Psych.] Classics and Revisits in Scientific Radiology CRS[Rad.]
Classics and Revisits in Scientific RheumatologyCRS [Rheum.]
Osteogenesis Imperfecta fragilis with blue sclerotics in childhood
Classics and Revisits in Interdisciplinary Medical Themes.CRS [Int.Disc.Med.Them.]
Proteus syndrome
Cervical haemangiomas with intracerebral extensions
Contents
Background and purpose of Journal and Supplements 2 .
List of Specialty Focused Journal Supplements Profiles 3. Journal and Supplements Editorial policy 4
Specialty Focused Journal Supplements Profiles. 7
Classics and Revisits in Scientific Cardiology Supplement (1) 14
Angina pectoris and Acute Chest Pain of Cardiac Origin.
Classics and Revisits in Scientific Rheumatology Supplement (1) 22
Osteogenesis Imperfecta Fragilis with Blue Sclerotics
in Childhood
Classics and Revisits in Scientific Interdisciplinary Medical Themes.
Proteus Syndrome in Childhood 29
Cervical Haemangiomas with Intracerebral extensions. 47
Classics and Revisits in Scientific Haematology. 49
Fanconi Anaemias in Childhood.
April 2014 1(1) Classics and Revisits in Scientific Medicine.
Back ground
Classics and Revisits in Scientific Medicine CRS [Med.] is new.
CRS [Med] is a balanced portal of continuing medical education information ,
Purpose
The intent is that CRSM [Med] could provide a fairly well effective reciprocal communicative pathway for continuing medical education development.
The text delivers the details of good basic and clinical medical practice clearly and concisely .It is a unique contribution to medical specialty practice development nationally, regionally and globally, in the context of ongoing health trends.Although the current directions in health care developments were highlighted, an unpretentious emphasis to quite often unavailable advanced technological innovations and models were reasonably. weighted.
Focus and Theme
Throughout the series efforts were made to balance the emphasis on the scientific fundamentals and the basic principles of management and prevention with research directions and recent advances in investigations and interventions.
In order to make it more reader friendly and handy, efforts were made to minimize the volume while at the same time not compromising the contents.
On this basis, suggestions were made that it should be a regular and frequent topical medical periodical in scientific basic and clinical medicine.
It is hoped that these compendia will be found most rewarding by medical practitioners, health professionals undergoing their undergraduate and postgraduate medical trainings and other health conscious professionals and individuals.
This is a legendary textbook of scientific medicine in a journal format.
Issues and Future Trends
In this edition of the CRS [Med], a preamble of the format of the publications is suggested.
However, it would be worthwhile to hasten to say that it is likely that there may be considerable alterations in these serial publications with regards to their academics structure, print layout, constituent parts, focus and certainly contents and periodicity.Therefore suggestions and opinions on contents inclusions and modifications are welcome.
In principle, publications would normally follow positive peer and editorial reviews, revisions and decisions.
Specialty Focused Journal Supplements
For clarity sake, the other associated serial medical scientific publications and channels of classics and revisits in scientific medicine, which will normally be published periodically as attached, merged or adhoc as isolated supplements to the parent journal CRS [Med] are alphabetically itemized according to the relevant medical specialties focus as follows:
April 2014 Vol 1(1) Classics and Revisits in Scientific Medicine
April 2014 1(1) Classics and Revisits in Scientific Medicine.
Specialty focusedSupplements to JournalNumber Specialty focusedSupplements to Journals Fulland abridged Title Anticipated IssuesPer year/PublicationCycle Specialty focusedJournalSupplementClassification
01 Classics and Revisits in Scientific Cardiology- CRS [Card.] 12Monthly Academic, clinical.professional&Scholarly
02 Classics and Revisits in Scientific Dermatology. CRS [Derm.]. 12Monthly Academic, clinical.professional&Scholarly
03 Classics and Revisits in Scientific Endocrinology. CRS [End.]. 12Monthly Academic, clinical.professional&Scholarly
04 Classics and Revisits in Scientific Hepatology . CRS [Hep.] 12Monthly Academic,clinical,professional&Scholarly
05 Classics and Revisits in Scientific History of Medicine. CRS [His.Med.] 12Monthly Academic, Informative.professional&Scholarly
06 Classics and Revisits in Scientific Infectious Diseases. CRS[Inf.Dis.] 12Monthly Academic, clinical.professional&Scholarly
07 Classics and Revisits in Scientific Interdisciplinary Medical Themes.CRS [Int.Disc.Med.Them.] 12Monthly Academic, Clinical, Informative.professional&Scholarly
08 Classics and Revisits in Scientific Neonatology. CRS [Neon.] 12Monthly Academic, clinical.professional&Scholarly
09 Classics and Revisits in Scientific Nephrology. CRS [Neph.] 12Monthly Academic, clinical.professional&Scholarly
10 Classics and Revisits in Scientific Neurology. CRS [Neur.] 12Monthly Academic, clinical.professional&Scholarly
11 Classics and Revisits in Scientific Oncology. CRS [Onc.] 12Monthly Academic, clinical.professional&Scholarly
12 Classics and Revisits in Scientific Preventive Medicine. CRS [Prev.Med,] 12Monthly Academic, clinical, Informativeprofessional&Scholarly
13 Classics and Revisits in Scientific Pulmonology. CRS [Pul.] 12Monthly Academic, clinical.professional&Scholarly
14 Classics and Revisits in Scientific Psychiatry. CRS [Psy.] 12Monthly Academic, clinical.professional&Scholarly
15 Classics and Revisits in Scientific Radiology. CRS [Rad.] 12Monthly Academic, clinical.professional&Scholarly
16 Classics and Revisits in Scientific Rheumatology. CRS[Rhem] 12Monthly Academic, clinical,professional&Scholarly
17 Grey Medical Literature. Ad-hoc Academic&Scholarly
18 News Letters,Brochures&Bulletins Ad-hoc Professional
19 Medical Health Updates Ad-hoc. Clinical &Professional
20 Other Miscellenous Publications Ad-hoc Clinical&Professional
Classics and Revisits in Scientific Medicine. April 2014 1(1)
Editorial Policy
We are pleased to consider papers in the following categories.
Original Articles from primary and secondary research:
The original articles could normally be up to 3000 words.
The documentations of the original papers should follow the traditional IMRAD pattern introduction, methods/ patients, results.discussion.conclusion, acknowledgements and references
The contents are expected to be precise, factual and as concise as possible.
Illustrative colour, black and white photographs and line drawings could be used if they could make the contents more lucid.
The abstract or the summary section should be structured and limited to 250 words or less.
Review Articles:
Review articles would generally be less than 3000 words.
It should be well researched and referenced.
Short Reports and Brief Communications:
Short reports and brief communications may contain up to 1000 words.
No summary or abstracts are required, but authors could decide to include them.
The contents should be logically ordered, but division into sections is optional and up to ten references may be used.
This may be suitable for the presentation of descriptive studies, some personal experience/observation and some field studies.
Case Reports and Case Series
This is the format most suitable for the presentation of individual case reports of particular interest or an illustrative series of cases.
Correspondence to the Editor
This could be on any topic related to the subject areas of the journal, which could be new topics or based on articles which have appeared in the series.
Current Topical Issues, opinions, view points, annotations and perspectives
This part of the series will provide a forum for expert opinion on topical issues in the area relevant to the series.
Appropriate submissions may include proposals for action such as:
[I]-Sound clinical judgment and good practice.
[II]-Appraisal or critiques of ongoing practice or principles.
[III-Responses to published statements.
The length of submissions in this section should normally be less than 1000 words. The presentations are expected to be precise, succinct and factual.
Usually about 20 references or less will suffice.
Occasional papers
Manuscripts of reasonable academic or clinical relevance belonging to this category could be published in the journal or in its supplements.
April 2014 1(1) Classics and Revisits in Scientific Medicine.
Research Letters:
This is a good medium for communicating research findings, which are too brief for short reports or brief communications.
Supplements:
Supplements to the series by Consulting Guest Editors on emerging topical issues will be published with a specialty based focus, periodically and on ad-hoc basis.
Normally these specialty focused supplements will be published as attachments, merged or isolated and detached supplements to the parent journal CRS [Med]
Technical Notes, Methods and Devises:
Papers describing procedures, techniques or equipment adapted by authors to their own conditions of work are welcome.
Medical Highlights and Forum Digest:
Summaries or Abstracts of ongoing and emerging topical medical issues will be published in the relevant sections of the journal or its specialty supplements.
Normally, these abstracts and summaries will be limited to 350 words or less and are best presented unstructured.
Editorials:
Normally, each series will be accompanied by an editorial perspective which as much as possible will aim to illuminate on the most significant contributions in the series.
Periodically, and on ad hoc basis editorials by consulting academic clinical guest editors will also be included, in the journal series or its supplement.
Book updates, Book Reviews and the Academic Clinicians Book shelf:
The series will consider book reviews, book updates relevant to the specialty series. A book shelf highlighting other updated books relevant to the specialty could be mentioned or displayed.
Report of the Highlights of Meetings and Conference Proceedings:
Proceedings of meetings and conferences relevant to the theme of the series could be published along the main journal series or as a supplement.
The specialty relevant highlights of appropriate medical scientific meetings will be published in the journal series or as supplements.
Academic Clinicians Web-Blog watch and alert:
Clinicians and medical scientists could be made aware of interesting Web sites and web-blogs in their relevant specialties.
Expert Opinions and View Points:
This involves a best evidence based expert advise and answer on a difficult or ambiguous academic or clinical encounter in any area relevant to the scope of the series. Normally the question or the scenario would be presented in a contextual format.with no more than 250 words.
The interventional answer or the solution to the difficulty will then be printed along with the question in the journal or a supplement following consultations with academic clinical experts in the topic and specialty.
April 2014 1(1) Classics and Revisits in Scientific Medicine.
Ongoing and forthcoming medical conferences, courses and pathways to medical career opportunities and progressions.
The series will from time to time and on adhoc basis publish a list of accreditated medical conferences, courses and achievable pathways to medical career progressions and opportunities.
Health News
The series will from time to time publish a balanced scientific perspective of the medical health literature on emerging clinical issue of public health importance.
Profile of Medical Journals and other related Publications
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April 2014 1(1) Classics and Revisits in Scientific Medicine.
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April 2014 Vol 1(1) Classics and Revisits in Scientific Medicine
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CRS [Med]Classics and Revisits in Scientific Medicine.
This novel edition of CRS[Med] is a composite compilation of continual medical educational resource consisting of a profile of four supplements to the journal with a medical specialty focus with a considerable weighting given to their editorial aspects and themes:Classics and Revisits in Scientific Cardiology.CRS[Card]Classics and Revisits in Scientific Rheumatology .CRS [Rhem.]Classics and Revisits in Scientific Interdisciplinary Medical Themes.CRS[Int.Disc.Med.Them]
Classics and Revisits in Scientific Haematology.CRS[Haem,]
The Classics and Revisits in Scientific Cardiology.CRS[Card] journal supplement Presents a Guest Editorial Perspective on Angina pectoris and Acute chest pain of Cardiac origin by the CRSM Academic Clinical Consulting Guest Editors.
Classics and Revisits in Scientific Rheumatology .CRS [Rhem.] Classics and Revisits in Scientific Rheumatology .CRS [Rhem.]With a paediatric slant dilates on the peculiarities of Osteogenesis Imperfecta fragilis with blue sclerotics in childhood as a Guest Editorial review article by the CRSM Academic Clinical Consulting Guest Editors.a paediatric slant dilates on the peculiarities of Osteogenesis Imperfecta in negroid Africans as an Editorial short communication.
Classics and Revisits in Scientific Interdisciplinary Medical Themes.CRS [Int.Disc.Med.Them] with a paediatric slant.This supplement includes a guest editorial review article on Proteus syndrome in childhood and cervical haemangiomas in children with an intracerebral extension as editorial review articles by the CRSM Academic Clinical Consulting Guest Editors.
In this Classics and Revisits in Scientific Haematology.CRS [Haem] supplement with a paediatric slant characteristic Fanconis anaemia in childhood was discussed as an Editorial review article by the CRSM Academic clinical consulting Guest Editors.
Specialty Focused Journal Supplements Profile April 2014Supplements to Classics and Revisits in Scientific Medicine.Volume No. 1 Issue No.1
Classics and Revisits in Scientific CardiologyCRS [Car]
Angina Pectoris
and Acute Chest
pain of Cardiac origin
Classics and Revisits in Scientific Haematology
CRS[Haem]
Fanconi-anaemias
Classics and Revisits in ScientificRheumatologyCRS[Rheum]
Osteogenesis Imperfecta
Classics and Revisits in ScientificInterdisciplinary Medical ThemesCRS [Int.Disc.Med.Them.]
Cervical haemangiomas with intracranial extensions
and complications
Proteus Syndrome
Contents:
. Acute Chest pain of cardiac origin and Angina Pectoris.
. Osteogenesis Imperfecta fragilis with blue sclerotics in childhood.
. Proteus syndrome in childhood.
. Cervicalhaemangiomas with intracerebral extensions in childhood
. Characteristic Fanconi anaemias in childhood.
April 2014 Supplement 1 Classic and Revisits in Scientific Cardiology.
Angina Pectoris and Acute Chest pain of cardiac origin
Guest Editors: CRS [Med] Consulting Academic clinical Experts.
Definition:
Angina pectoris is an acute chest pain of cardiac origin due to a poor reception of oxygen supply by the heart muscles:
Aetio-Patho-physiology:
Angina pectoris usually ensues when the oxygen demands and requirements of the heart exceeds the capacity of the coronary arteries to supply an adequate amount of blood to the heart for the coronary cardiac perfusion.
In typical exertional angina pectoris, ischaemia results when myocardial oxygen demand is increased but supply is relatively compromised because of occlusion of more than fifty percent of the coronary arteries either directly through the obstruction by the eccentrically located arteriosclerotic plaque or following dynamic coronary artery vasoconstriction of the subjacent areteriosclerotically uninvolved vessel muscle wall.In the setting of atherosclerotic blood vessel pathology.
Usual Causes:
Artheriosclerosis, Coronary Artery Spasms, Anaemia and Erythrocytopaenia:
Coronary-Cardiac perfusion could be compromised or made ischaemic by a diminution in the caliber of the coronary arteries, erythrocytopaenia or anaemia.
The diminution in the caliber of the coronary arteries may be arteriosclerosis related or sympathetic dysautonomic resulting in coronary artery spasms.
The usual causes of erythrocytopaenia or anemia were reviewed elsewhere.
In intense anaemia or erythrocytopaenia, angina pectoris is likely because the number of red blood cells [which contains haemoglobin –the oxygen carrying molecule in the red blood cells or the amount of haemoglobin in themselves is below the normal range for the age and gender of the case leading to a diminution in the oxygen supply to the heart muscles and acute cardiac pain-angina. Other pathologies which cause an increase in the work of the heart and thus an augmentation of the oxygen requirements of the heart include:
Hypertension:
Intense to profound high blood pressure causes coronary artery vascular flow resistance following fibromuscular hyperplasia of the coronary artery wall and coronary artery fibromuscular spasms from reflex sympathetic dysautonomia.
Valvular Heart Disease:
Cardiac valvular stenosis or narrowing and valvular regurgitations or incompetencies especially of the aortic valves given its close anatomical relationship to the coronary arteries associated with rheumatic valvular heart disease or of other aetiological factors will lead to a net ineffective or insufficient aortic outflow and consequently coronary artery perfusion difficulties.
April 2014 Supplement 1 Classic and Revisits in Scientific Cardiology
Angina Pectoris and Acute chest pain of cardiac origin.
Cardiomyopathies:
Idiopathic heart muscles pathologies especially the hypertrophic obstructive cardiomyopathic forms leads to a thickening of the cardiac muscles -increasing the surface areas to be perfused, in addition to causing a compressive effect on the coronary arteries and a relative myocardial perfusion insufficiencies.
Arrythmogenic Right Ventricular Dysplasia or Type Four Cardiomyopathy could also cause arrhythmia related angina pectoris.
Hypercoagulable states:
The angina pectoris associated with hypercougulable states are usually a consequence of compromised ischaemic oxygenation of the heart muscles secondary to poor myocardial perfusion following sludging of poor blood flow due to its hyperviscocity.
Cardiac Dysarythmias:
Atrial and ventricular fibrillations are usually associated with angina pectoris because of the associated poor ventricular filling and hence cardiac output.
Aetiopathogenic Classification Scheme for Angina Pectoris:
Effort Angina Pectoris:
This is a chest pain or discomfort that typically occurs with activity or stress.
This pattern of angina is usually transient, but appears to be induced repeatedly by these activities or stress profile in a predictable and regular manner.
Postural Angina Pectoris
This is the form of angina that occurs on sudden recumbency due to the redistribution of the body fluid with an increase in left ventricular end diastolic pressure initiated increase in the cardiac work load and subsequent myocardial ischaemia.
Cardiac Syndrome X:
This form of angina pectoris usually affects middle aged menopausal women following hysterectomy and it is usually associated with ST segment depression on exercise thread mill test, but angiographically normal coronary arteries.
The prognosis for progression to coronary artery disease is the same as those for the normal population.
The cause of this pattern of angina is thought to be due to narrowing of the smaller coronary arteries or arterioles due to neurotransmitter or myocytes related chemical imbalance causing ventricular dysfunction or other forms of coronary arteriolar dysfunctions.
Hypersomniac Angina Pectoris:
This is angina related to sleep, especially the rapid eye movements [REM] sleep, it is probable that this form of angina is related to the relative hypoxia in this stage of sleep or the obstructive sleep apnea syndrome or associated nightmares.
April 2014 Supplement 1 Classics and Revisits in Scientific Cardiology
Variant or Prinzmetal Angina Pectoris:
This is an effortless Angina Pectoris and refers to episodic unpredictable angina which occurs during rest rather than during activities and it is usually accompanied by specific electrocardiographic changes such as transient ST segment elevation during the anginal attacks.
This form of angina is thought to be consequential to spasms of the large coronary arteries on the surface of the heart with or without atherosclerotic lesions.
Unstable Angina Pectoris:
This form of angina pectoris is metarmorphic.
It refers to angina pectoris with variable symptomatologies,given the constant symptomatology patterns of angina in any one individual, any augmentative change in its symptomatologies such as its severity, attack frequencies, uninduced events or whilst in the restive state should be taken seriously, and investigated, because the risk of a heart attack is usually considerable, because this may indicate a sudden narrowing due to a new cloth formation or rupture of an atheroma.
This form of Angina Pectoris is considered a form of Acute Coronary Syndrome.
Symptomatologies:
More often than not anginal pains are usually felt as a pressure or an ache beneath the breastbone-sternum.
This is usually interpreted as a chest discomfort or heaviness rather than pain.
The affected individual has an exertional chest discomfort assuaged by rest.
This discomfort may also occur in either shoulder or down either sides of the arm, the back, the throat, jaw or the teeth or as a burning sensation especially in the middle aged female.Anginal pains are usually transient, worse following cigarette smoking, emotional, exercise, exposures to cold or physical exertions especially after meals, walking in cold windy environment, sleep or nightmares
Patterns of anginal pains may be predictable or unpredictable.
Diagnosis:
Accurate historical details of the nature of the pain following the Ryles ten point algorithm questionnaire with its associated symptomatologies is very crucial for the exact diagnosis of angina pectoris. A physical examination in between events although mandatory may not be all that very useful. If an opportunistic examination is achievable during an event, a mild tachycardia, hypertension or dysarrthymia or specific or non specific ECG changes may be observable.
Coronary reserve is mostly compromised most in the subendocardium, ischaemia occurs initially in that region. If an ECG is undertaken during an episode of an anginal pain, ST segment depression suggestive of a subendocardial ischaemia will be observable.
April 2014 Supplement 1 Classic and Revisits in Scientific Cardiology
Angina Pectoris and Acute Chest Pain of Cardiac Origin.
Adverse prognostic features in Angina pectoris
The prognosis for angina pectoris is usually affected adversely by advancing age, extensive coronary artery disease, diabetes mellitus, hypertension, previous heart attacks, tobacco smoking, hypercholesterolaemia, events with excruciating chest pains, ventricular pump failures, the extent and severity of the coronary artery occlusions, abnormal ECG results, the prognosis for people with stable angina and efficient ventricular pump function, the prognosis is reasonably good.
Although the mortality rate for people with isolated angina pectoris is about 1.5%, it is considerably higher for those with the risk factors outlined above.
A good historical description of the symptoms may be enough to achieve the diagnosis of angina pectoris in most instances of the classically typically presenting cases.
Electrocardiogram
Electrocardiogram (ECG) will show ST segment changes as outlined above.
Interval ECGs are not usually very satisfactory, most useful information are usually achieved with the baseline and pharmacological exercise stress testing ECGs using a vasodilator.
Continuous ECG monitoring with a Holter monitor may detect abnormalities indicating symptomatic or silent ischaemia or prinzmetal angina which typically occurs at rest.
Exercise Stress Test.
An exercise stress test incites myocardial ischaemia, in patients with coronary artery diseases and offers some useful prognostic information to the clinician.
The patient who manifests ischaemia either clinically or electrocardiographically following the first few minutes of exercise is likely to have myocardial insufficiency related to coronary artery dysfunction or disturbances of its structural integrity.
An exercise stress test should be undertaken to define objectively the degree of exertion required to induce myocardial ischaemia due to coronary artery related haemodynamic disturbances. Patients with a positive exercise stress testing should be presumed to have a relatively considerable morbidity and should be referred for further angiographically gated imaging procedures.
Echocardiography.
In this procedure, ultrasonic waves could produce images of the heart called echocardiograms thereby outlining the size of the heart, the movement of the heart muscles, blood flow through the heart valves and the valvular functions during rest or related to exercise ischaemic conditions, Left ventricular functions is usually compromised by ischaemia
April 2014 Supplement 1 Classics and Revisits in Scientific Cardiology.
Angina Pectoris and Acute Chest Pain of Cardiac Origin.
Coronary Angiography:
For this investigation to be achieved, x-rays of the coronary arteries are taken after a radiopaque dye is injected.
Coronary angiography is probably the most accurate procedure for diagnosing coronary artery disease.
It is usually indicated when the diagnosis of angina pectoris or coronary artery disease is uncertain.
Coronary angiography is commonly used to help evaluate whether Coronary by-pass Graft or percutaneous coronary intervention is appropriate.
It could also be employed in the diagnosis of a spontaneous or drug induced coronary artery spasm.
Multimodal Computer Assisted Tomographic Scans.
Electron beam computer assisted tomographic scans could detect the amount of calcium deposits in the coronary arteries.
The amount of calcium present could be computed as the calcium score is roughly proportional to the likelihood of the person having an angina or a heart attack. The limitation of this procedure is that occasionally, calcium deposits may be present in people whose coronary arteries are not terribly compromised so the scores derived may not very much predict the need for a percutaneous coronary intervention or coronary artery bypass grafting.
Given the significant radiation exposure with this procedure, EBCT will not usually be applied universally, but would be restricted to those cases with a relatively significant high risk of death or acute coronary syndrome especially those with risk factors such as metabolic syndromes, diabetes mellitus, hypertension, hypercholesterolaemia, hyperuraceamia, abnormal, unclear or ambiguous test results.
Multidetector series Computer Assisted Tomographic scan
This is a new technique that employs a high velocity CT scanner comprising of multiple small detectors that could accurately identify coronary artery narrowing.
April 2014 Supplement 1 Classics and Revisits in Scientific Cardiology.
Angina Pectoris and Acute Chest Pain of Cardiac Origin
However the amount of radiation exposure is considerable.
It could also be used to ascertain the patency of a stent or a bypass graft, or to ascertain if atheromas have undergone dystrophic calcifications.
In addition it is very useful for displaying the cardiac and the coronary venous anatomy.
This procedure is absolutely contraindicated in pregnancy, or in patients who are unable to hold their breath for at least a quarter of a second or tachycardiac or cannot tolerate bradycardia or the drugs used to induce bradycardia.
If the test must necessarily be employed in patients with tachycardia, they may need to be allowed a cardio-selective beta blocker to regulate the heart rate below 70 beats per minute.
The technique is noninvasive and highly accurate in excluding coronary artery narrowing as a source of a person’s symptoms especially in those instances where the exercise stress test was unachievable or its results inconclusive.
Cardiac Magnetic Resonance Imaging
In cases of coronary artery disease, MRI could be employed to evaluate the narrowing of the coronary arteries, measure the blood flow in the coronary arteries and test how well the heart is being supplied with oxygen.
It could also be used to assess the abnormalities of the heart wall motion during exercise stress test which may signify a poor blood supply to that area.
MRI may also be used as a test of viability to assess whether areas of the heart muscles damaged following an acute coronary syndrome could recover.
It could be used to evaluate the heart and the large blood vessels arising from the heart such as the aorta and the pulmonary arteries.MRI is radiation free.
Management:
Treatment of angina pectoris depends partly on the stability and severity of the symptoms of the angina.
Management of Angina pectoris implies attempts to slow or reverse the progression of coronary artery disease by the rapid treatment and modifications of risk factors, such as hypertension, high cholesterol levels, cessation of cigarette smoking, obesity, recommendation of a low varied polyunsaturated fatty diet, which is low in highly refined sugar carbohydrates and exercise.
The patient will need to comprehend the pathological process to drive the motivation to modify coronary artery disease risk factors and to be able to know
where and when to seek medical assistance during aggravation of symptomatologies.
In stable angina with symptomatologies of mild to moderate severity, modifications of the above risk factors with occasional drug use may suffice.
If this approach is not enough to ameliorate the symptomatologies of angina considerably, a procedure to restore the myocardial perfusion by improving the coronary blood flow through a revascularization procedure should be recommended. When symptomatologies worsen rapidly, immediate hospitalization is usually required and the case should be evaluated for a acute coronary syndrome.
April 2014 Supplement 1 Classics and Revisits in Scientific Cardiology.
Angina pectoris and Acute chest pain of cardiac origin.
Pharmacotherapy:
Pharmacotherapy of stable angina pectoris is aimed to ameliorate myocardial perfusion and decrease the work load on the heart.
The pharmacotherapy of angina pectoris revolves around six groups of drugs.
A-The organic nitrates
B-The beta-blockers
C-The calcium channel blockers
D-The angiotensin-converting enzyme inhibitors.
E- The antiplatelet drugs.
F-Non-opoid and opoid analgesics.
The Beta Blockers
These groups of drugs antagonize the effects of the catecholamine hormones epinephrine and norepinephrine on the heart and other organs.
These hormones have chronotropic (increases the heart rate) and inotropic effects (increases the strength of the heart contractions, and causes the arterioles to constrict (there by causing the blood vessels to constrict)
The resting and the activity related augmentation of the heart rates and blood pressures are usually ameliorated by these groups of drugs, thereby the myocardial oxygen demand is reduced. This group of drugs reduces the risk of heart attacks and sudden deaths.The long term outcome for people with coronary artery disease is therefore improved.
[2]-Organic Nitrates
[A]-Short Acting Organic Nitrates
Nitroglycerin is a very short acting organic nitrate drug that dilates the blood vessels.Following the administration of this drug .the anginal events are usually ameliorated in about two to four minutes. It is usually quite handy and could be taken shortly in anticipation of an anginal event, however the effects of nitroglycerin usually lasts about 30 minutes. The dose could be administered sublingually (i.e. as a tablet placed under the tongue) or bucally (as a tablet placed next to the gum) or intranasally (as a spray inhaled through the nostrils)
[B]-Long Acting Organic Nitrates
Isorsorbide dinitrate is an example of a long acting organic nitrate
This drug could be taken as tablets orally about one to four times daily.
It could also be administered as nitrate skin patches and paste allowing cutaneous absorption over several hours.
However if they are taken regularly they may loose their ability to provide relief.
Usually a period of nocturnal drug holiday is usually appropriate for its long term anginal relieving effect to be maintained. The use of their effect is mainly aimed towards the relieve of anginal symptomatologies rather than prophylaxis against acute coronary syndromes or sudden deaths.
April 2014 Supplement 1 Classics and Revisits in Scientific Cardiology.
Angina Pectoris and Acute Chest pain of Cardiac origin.
[3]-Calcium Channel blockers
This group of drugs prevents the blood vessels from narrowing (constricting) and could attenuate coronary artery spasm and as a group have a hypotensive effect.
Calcium channel blockers are equally effective in Prinzemetal angina pectoris.
Verapamil and daltiezem has negative chronotropic and antiarrythmic effects.
This group of drugs on the average is used as third line drugs, if the above two groups of drugs are inadequate for symptomatic relief or are contraindicated.
[4]-Angiotensin Converting Enzymes Inhibitors
ACE inhibitors such as ramipril were often given to angina pectoris cases with the aim of attenuating the risk factors of acute coronary syndromes and sudden deaths rather than symptomatic relief of angina pectoris.
[5]-Antiplatelet Agents
Aspirin, disopyradimole, ticlopidine, and clopidegrel have antiplatelet effects and influence the function of platelets, so that they do not clump together and stick to the blood vessels walls.
Platelets which circulate in the blood, promote clot formation (thrombosis) when a blood vessel is injured.
If platelets collect on atheromas in an artery’s walls, the resulting clot could narrow or block the artery and result in an acute coronary syndrome.
Aspirin modifies the platelet function by reducing the tendency for its aggregation and thrombus formation thereby ameliorating the vascular occlusion associated with artheromatous thrombus formation.
Antiplatelet drugs are usually given to individuals with angina pectoris, unless there is a good reason not to, such as in individuals with bleeding diathesis or haematological dyscrasias of unclear significance or consequence.
The traditional teaching is for individuals with coronary artery syndrome to take cardio-aspirin 75mg daily in addition to clopidogrel daily to reduce the risk of an acute coronary syndrome. Another beneficial antiplatelet drug Ticlopidine, although with more frequent side effects could be employed in individuals who are allergic or intolerant to aspirin-clopidogrel combination.
[6]-Non-Opoid and Opoid Analgesics
Analgesia of varying strengths will invariably be a component of the anti-anginal pectorisarmamentarium.
Paracetamol, Aspirin, other non-steroidal anti-inflammatory agents and codeine or morphine could be employed in combination on individual cases as bridge therapy until appropriate beta blocker or calcium channel blocker is defined.
April 2014 Supplement 1 Classic and Revisits in Scientific Cardiology.
Angina Pectoris and Acute Chest pain of cardiac origin
In summary the objectives of the pharmacotherapy of angina pectoris is aimed at the reduction of myocardial oxygen demand during exercise or stress by the use of organic nitrates, beta blockers or calcium channel blockers
Promotion of maximal dilatation of the coronary arteries by the use of nitrates, calcium channel blockers.
Revascularization Procedures:
Percutaneous Coronary Intervention (PCI) angioplasty and Coronary Artery By Pass Grafting [CABG]
These procedures are the two main revascularization procedures employed in the management of angina pectoris where pharmacotherapy was unsuccessful.
However, it is worthwhile to note that these procedures are invasive anatomical techniques which aim to alleviate the immediate pathology, but they are not usually effective for the cessation of the progression of the underlying disease without influencing the risk factors favourably.
[A]-PCI is non-invasive and hence it is often preferred to CABG in a selected group of patients. It is particularly indicated in cases in one or two coronary artery vessel disease, especially when the blocked coronary vascular segments are not lengthy or extensive. However, increasing experience and more sophisticated techniques is allowing this procedure to be used confidently and more frequently.
[B]-CABG, is particularly beneficial to individuals with severe angina and coronary artery disease which were not amenable to relevant pharmacotherapy, good cardiac reserve and a normally functioning heart, no previous acute coronary syndromes, no chronic obstructive pulmonary disease, thrombophilias, carcinomatosis or other conditions that could preclude surgery or make it hazardous About nine of ten of such people will have a rapid and absolute relief of symptoms after surgery.
CABG could improve symptoms, exercise tolerance, and could decrease the number and dose of drugs needed
An electively planned CABG has an infinitesimal mortality at about less than 1 in 100 and a risk of asymptomatic or symptomatic intraoperative acute coronary event of about less than one in twenty.
Guest Editors: CRS [Med] Consulting Academic clinical Editorial Experts.
April 2014 Supplement 1 Classics and Revisits in Scientific Rheumatology.
Osteogenesis Inperfecta fragilis with blue sclerotics in childhood.
Guest Editors: CRS [Med] Consulting Academic clinical Editorial Experts.
An unusual preponderance of observational reports on children with phenotypic features consistent with Osteogenesis Imperfecta fragilis presenting covertly with a history of multiple long bone fractures and /or easy bruisability of the skin espoused an expert guest editorial consultations and review article on this theme.
Introduction/Historical Perspective
Osteogenesis Imperfecta or Vroliks syndrome is a heterogeneous group of inherited conditions arising from a variety of biochemical and morphological collagen defects.Willam Vrolik professor of Anatomy ,Pathological Anatomy and zoology at the Athenaeum Illustre(University of Amsterdam) discussed in his handbook of Pathological Anatomy(1842-1844) and Tabulae and Illustradam embryogenesis hominis et mamalium,naturalem tam abnorman (1844-49) a new born infant with numerous fractures and hydrocephalus.The Tabulae, had both Latin and Dutch texts, in the Latin text ,Vrolik used , the term Osteogenesis Imperefecta (in Dutch, gebrekkige beenwording) Vrolik also mentioned that the infant lived for three days and that both parents were suffering from Lues Universalis at the time of the baby’s birth .
On re-examination of the Index case.
The whole skeleton of the baby appeared poorly mineralized.
The family had large skulls, exhibited a broad and high forehead, large fontanels, frontal and temporal bossing, shallow orbits, and a protruding occiput.
At autopsy, the calvaria of the baby consisted of many wormian bones.
The tubular bones, although of normal length and only slightly curved, were very thin as were the ribs. All the skeletal structures showed one or more fractures and many fractures showed callus formation. Following further investigations on this topic, this case was retrospectively diagnosed as a case of Osteogenesis Imperfecta type II..
Willan Vrolik was one of the first scientists to realize that many skeletal dysplasias were not the result of a post natally acquired disease such as rickets or osteomalacia as many of his contemporaries believed. He thought that it might be due to insufficient intrinsic generative energy. He substantiated this by stating that in his study specimen a primary impairment of ossification is present and not a secondary degeneration.
The Index Case Description.
The descriptions given by Willem Vroliks in some of the specimens ,generated the term Osteogenesis Imperfecta and the eponym Vroliks syndrome for this genetic disorder characterized by increased fragility.[1]Fractures are frequent and its onset depends on the type of Osteogenesis Imperfecta.[2]Osteogenesis Imperfecta is a heritable disease that may result in bone fragility, increased joint laxity, decreased muscle tone, thinning of the skin, a bluish appearance of the sclera, scoliosis, deafness, dentigenosis imperfecta and other multiple abnormalities.
April 2014 Supplement 1 Classics and Revisits in Scientific Rheumatology
Osteogenesis Imperfecta Type 1
Osteogenesis Imperfecta type 1 is a disorder characterized by frequent bone fractures and deformities associated with fracture malunion and poor healing, blue sclera, normal or near normal stature and autosomal dominant inheritance pattern.
The incidence is thought to be one in about twenty thousand live births.
Males and females are affected equally Osteopaenia is associated with an increased rate of long bone fractures upon ambulation.
For some reasons not well understood, fracture frequency decreased dramatically at puberty and during young adult life but increases once again in late middle age.
Most observed patients presented with clinically overt symptomatologies prepubertally.
Progressive hearing loss, often beginning in the second and third decade, is a feature of this disease in about half the families.The proportion of Osteogenesis Imperfecta Type 1 patients with significant hearing loss rises steadily into middle age ,despite the general decline in fracture frequency .Conductive or mixed conductive and sensorineural hearing loss are more common in dominant pattern oseogenesis imperfecta than sensorineural hearing loss alone. Dentigenosis Imperfecta is observed in some subset of the patient population.
Mortality Figures.
Mortality could be significant even in hospitalized patients at about three percent..
Mortality figures are 3.7 times more in the negroid race.
OI related demises are more likely in women. Admissions at younger age connote more severe disease.
Children at a younger age are more severely affected and more difficult to manage.
They tend to fair better when referred to a dedicated paediatric unit [3]
A classification scheme and the inheritance pattern for OI
Inheritance pattern depends on the type. The classification of OI has been debatable.
The detailed aspects of its nomenclature have been reviewed.
Anecdotally there are four types of Osteogenesis Imperfecta, although with recent advances in molecular biological techniques, other sub-types have been deciphered with considerable over-lap between the subtypes. The mode of inheritance is autosomal dominant for Type1and VI and recessive for Type II and III. It has been reported in one of two twins. [4]Osteogenesis Imperfecta is a defect of Type 1 collagen which is present in many tissues including blood vessels.
The differentiation of child abuse and Osteogenesis Imperfecta is critical, because of medical and legal implications for the latter. [5, 6, 7]
Considerations for the Inheritance pattern in OI
The mode of inheritance of OI is variable and the clinical manifestations are heterogeneous. In addition to its bone involvement, osteogenesis imperfecta, may involve tendons, ligaments, skin scleras, dentin, fascia and blood vessels.
April 2014 Supplement 1 Classics and Revisits in Scientific Rheumatology
A modified classification scheme for OI
The most commonly used classification schemes are those developed by Sillence and Colleagues. [8]Type 1 Osteogenesis Imperfecta, which was previously called Osteogenesis Imperfecta tarda, is the most common form and is a relatively mild to moderately severe disorder , inherited as an autosomal dominant trait with varying penetrance and expressivity.
It is associated with blue scleras and hearing loss in about fifty percent of the cases [9]
Type 2 Osteogenesis Imperfecta is lethal in the perinatal period and was previously known as Osteogenesis Imperfecta congenita, it is the most severe form and it is inherited as an autosomal recessive trait.
Types 3 and 4 Osteogenesis Imperfecta have clinical manifestations of intermediate severity between that of types 1 and 2 and are often associated with bone deformities and varying degrees of short stature and hearing loss.
Hearing loss commonly occurs in patients with Type 3 OI, it also occurs in some patients with Type 4 Osteogenesis Imperfecta. [8, 9]Loss of hearing is one of the least constant major features of Osteogenesis Imperfecta, with occurrence that varies between twenty six and sixty percent[10]The association of Osteogenesis Imperfecta, hearing loss and blue sclera’s is often referred to as de Van der Hoene Kleyn syndrome, which was originally well described in 1918 by two authors. [11]This syndrome usually occurs in the late second and early third decades of life, younger patients have a mixed or sensorineural hearing impairment.[12]
Histopathological Features and Molecular genetic pathological aspects of OI.
Findings at histopathologic examination of specimens extracted from patients with Osteogenesis Imperfecta provide some clues and values to the Type of hearing Impairment that could be expected in these patients.Osteogenesis Imperfecta.
OI is one of the inherited connective tissue disorders that result in brittle bones.
The molecular defects reside in various mutations that lead to defective or decreased Type 1 collagen synthesis [9,13, 14]In greater than ninety percent of cases , Osteogenesis Imperfecta results from mutations in one of two genes,COL1A1 gene on chromosome 17 and COL1A2 gene on chromosome 7 which encodes the chains of Type I collagen.[15,16]
Clinico-pathological correlations.
Findings at the histopathological examination of specimens from patients with Osteogenesis Imperfecta provide clues to the types of hearing impairments that could be expected in these patients.Temporal bone specimens extracted from patients with osteogenesis Imperfecta congenita(Type II) demonstrated markedly delayed and deficient ossification in the three layers of the otic capsule.[17]Conductive hearing loss is expected in these patients and is mainly due to fractures that commonly involve the crus of the stapes or the handle of the malleus.These fractures lead to discontinuity of the ossicular chain or to fixation by ankylosis of the head of the malleus to the medial attic wall, Therefore the hearing loss in Osteogenesis Imperfecta could be of a conductive type and may not always be progressive[18] Adult onset hearing loss with Osteogenesis Imperfecta therefore could be of a conductive type and is not always progressive.
April 2014 Supplement 1 Classics and Revisits in Scientific Rheumatology.
Reports of Osteogenesis Imperfecta tarda (Type 1) are somewhat scanty since the availability of specimens depends on surgical procedures or autopsies.
The whole question on the f temporal bones morphologies related to deafness in OI was answered elsewhere. [19, 20, 21, 22]
Radiological Features of OI
Conventional radiographs of the tibia and fibular in IO could demonstrate multiple healed or healing fractures of the tibia , as well as non-united fracture deformities of the tibia or the fibular shaft .The bones are usually gracile and osteoporotic in appearance, which are some characteristics radiological features of osteogenesis imperfecta.
Further discussions on the collagenous defects in OI
The evaluation of children with multiple unexplained fractures is a clinically and radiological challenging one, because of issues concerning non-accidental injuries.
An inconsistent, non-correlating historical, clinical and radiological profile and cessation of further events will point more to a non-accidental injury as the cause of the pathological fracture. Abnormalities in collagen in other tissues other than the bones will account for clinical findings in addition to fractures, such as dentinogenesis imperfecta (fragile discoloured teeth), blue or grey colour of the sclera, increased joints laxities and a tendency to bruising(thought to be due to defective collagen of small blood vessels)
The distinctive features of OI
Reports on most of the observed cases, suggest that they probably belong to the Type I Osteogenesis Imperfecta or Type III.OI subsets.
The exact classification scheme could be accomplished, clarified and distinguished with exact certainty at molecular biological studies to determine if the collagen structure is qualitatively normal, but quantitatively reduced (Type I) or defective in (TypeIII) where it is abnormal. On epidemiological basis however, Type 1 OI would likely to be the diagnosis in the most of the observed reports because it is the most common and complicating fractures may not necessarily be present at birth.
Fracture patterns in OI
All types of fractures could be seen in osteogenesis imperfecta such as transverse, oblique and spiral fractures. Greenstick fractures and torus also occur commonly in children with mild to moderately severe defects..
Localizations to the lower limbs are particularly more common in osteogenesis imperfecta in comparison to normal children. Fractures in osteogenesis imperfecta could be metaphyseal.[23, 24] Rib fractures are also relatively common.
Of all these fracture patterns, metaphyseal fractures are those with the most implication for a multidisciplinary intervention, because of its strong connotations for
non-accidental injuries. [25]
The associated osteopathies in OI with their diagnostic significance.
Given that the primary defect in Osteogenesis Imperfecta is principally with the collagen.
The anecdotal evidence that osteopaenia, osteoporosis and diminished bone density are features in Osteogenesis Imperfecta could be unfounded. This is buttressed by the fact that precise densitometry and native densitometry in unfractured bones in Osteogenesis Imperfecta is generally within the normal reference range.[26]
April 2014 Supplement Classics and Revisits in Scientific Rheumatology
Osteopaenia and other abnormalities occasionally inferred, were usually multiple previous fractures and prolonged immobilization related. This diagnostic enigma is worsened by the fact that the assessment of bone density from X–ray radiological features is unduly unreliable [27] and as such it is usually not very useful overall in achieving the diagnosis of osteogenesis imperfecta especially in older children.
The diagnostic accuracy, reontenographic-densitometric correlations and precision is more distinct in infants and children with temporary brittle bone disease [28, 29] where they could assist to distinguish these conditions from Osteogenesis Imperfecta.
However lack of a standard reference range has compromised the applicability of densitometry with robust precision.
Wormian bones in OI
Other features that could espouse the diagnosis of Osteogenesis Imperfecta includes the presence of Wormian bones which are additional small bones within the sutures of the skull as was originally described in 1643 by Olaus Worm, a Danish anatomist.
In osteogenesis Imperfecta the number of Wormian bones are usually excessive and their distribution is in a crazy-paving pattern rather than a linear profile.
They are particularly most pronounced in the majority of cases with Type III Osteogenesis Imperfecta.
However Wormian bones could also be seen in excess in the milder types I, IV and other pathological states. The x-ray radiological techniques for the demonstration of Wormian bones and its implication for the diagnosis of Osteogenesis Imperfecta and other simulating conditions is an interesting topic.[30]The lateral and especially the Townes views are the most informative views in the assessment and demonstration of wormian bones and are particularly recommended as most appropriate for the exact assessment of Wormian bones ,especially in children with fractures, where the exclusion of a non-accidental injury will be expedient. The absence of Wormian bones will not be adequate to achieve an exclusion of Osteogenesis Imperfecta, especially in early infancy when it is notoriously absent as was confirmed by interval projections latter on.
Wormian bones are not notable features in temporary brittle bone disease.
Isolated periosteal reaction as a phenomenon is not all that a common feature of classical Osteogenesis Imperfecta except in relationship to fractures.
Occasionally, symmetrical metaphyseal sclerosis could follow fractures in Osteogenesis Imperfecta.
Other collagenous defects in OI with haemodynamic and functional consequence.
Being a collagen based defect, other features such as excessive joint laxity and cerebral aneurysms[31] and cardiovascular abnormalities especially pulmonary and aortic dilatation and regurgitation, mitral valve prolapse and subtle microscopic changes in the pulmonary artery and aorta could be associated features .These associations might not necessarily be present at birth, but usually they appear with the passage of time and plays a dominant role in the natural history of the disease process, especially with regards to deleterious consequences such as aortic dissections.[32, 33, 34] OI could be associated with bleeding diathesis which could be spontaneous or follow mild trauma. [35]OI could be associated with spondylolisthesis caused by extreme pedicle elongation.
April 2014 Supplement 1 Classics and Revisits in Scientific Rheumatology
The clinico-radiological distinction between OI and non-accidental injuries.
The Type 1 collagen is thought to make an important contribution to the structure and function of long bones.[36]From a Biomechanics perspective, bone is considered to be a non homogenous composite structure consisting at the very least of mineral and organic phases. In test of whole bone properties e.g. four point bending, the sum of the various phases of a composite structure usually contributes to the mechanical properties measured in other mechanical tests, one phase may predominate over the other.
Increased number of fractures with few or no bruises will point more to classical Osteogenesis Imperfecta rather than a non-accidental injury as the aetiological factor in cases of fractures, where as in non-accidental injury, bruising is much more common than fractures.[37, 38]
Although imaging is relevant to the diagnosis of Classical Osteogenesis Imperfecta it cannot be considered in isolation from the clinical findings, the historical events, the family history, the social assessment and the laboratory features.
Plausible pharmacological and physical therapeutic Interventions for OI
The beneficial therapeutic aspects of Osteogenesis imperfecta were contentiously discussed in the medical literature .Although no pharmacotherapeutic intervention has revolutionized the medical management of OI as such,however,the evidence for the beneficial effect of several pharmacotherapeutic interventions appears to be most convincing for the biophosphonates ,especially ,palmidronate or alendronate.
Also other reports have suggested the beneficial effect of the growth hormone in some selected group of children with OI, especially in aiding the affected children to attain an aesthetically acceptable linear growths, in other wise a subset that would have been disfiguringly stunted if the natural history of the pathology is uninterrupted with these pharmacological interventions.Orthopaedic and physical therapy in addition to modifications of functional, social professional and occupational ergonomics using assistive devices are other plausible interventional strategies.
Conclusion and Importance:
This editorial review article buttresses the need for clinicians to apply an in-depth evaluation, and scrutiny during patient’s encounter, to be able to decipher clinically covert pathological conditions and circumstances. Normally these evaluations would be most rewarding if a holistic multidisciplinary approach is introduced.
Guest Editors: CRS [Med] Consulting Academic Clinical Editorial Experts.
References:
[1.] Baijet B Aspects of the history of Osteogenesis Imperfecta (Vroliks Syndrome) An Anat 2002, 184(1).1-7.
[2.] Darmanis S, Bircher) Fractures of the acetabulum in Osteogenesis Imperfecta (Journal of Bone and Joint Surgery May 1.2006
[3.] Vitality,MG,Matsumoto ,Hiroko MA,Kessler ,Weinstein M,Hoffmann ,William BA,Roye,David P.Osteogenesis Imperfecta.Determining the demographics and the predictors of death from an inpatient population .Journal of Paediatrics orthopaedics.March 2007,27(2).228-232.
April2014 Supplement 1 Classics and Revisits in Scientific Rheumatology
[4.] O.Seriki.Osteogenesis Imperfecta in one of twins.Acta Paediatrica 21Jan, 59(3).340-342. and as a familial aggregation (Braga S, Passage E.Congenital Osteogenesis Imperfecta in three sibs (Human genetics) 1Oct1981, 58(4).441-443.
[5.] Klieman PK.Differentiation of Child abuse and Osteogenesis Imperfecta has medical and legal implications AJR (American Journal of Radiology May1990, 154. (1047-1048.
[6.] Bishop .N, Sprigg A, Dalton A, Unexplained fractures in infancy. Looking for fragile bones.Arch.Dis, Child, March 1, 2007, 92(3).251-256.
[7.] Cabral.W.A, Milgrom S, Leticha AD, Moriarty E, Marini JC.Biochemical screening of Type 1 collagen in Osteogenesis Imperfecta.detection of glycine substitutions in the amino end of the alpha chains requires supplementation by molecular analysis J.Med.Genet.August 1, 2006, 43.685-690.
[8.] Sillence D.O, Seen A, Danks DM, 1979, Genetic heterogeneity in Osteogenesis Imperfecta.J Med Genetm16.101-116.
[9.] Ablin DS, 1998, Osteogenesis Imperfecta, a review Can Assoc Radiol J.49.110-123.
[10.] Nager GT 1988, Osteogenesis Imperfecta of the temporal bone and its relationship to Otosclerosis.Ann Otol Rhinol Laryngol.97.585-593.
[11.] Van der Hoene J de Kleyn A.1918.Blaue Skleren, Knochen bruchigkeit Und Schwerhohrigkeit. Arch Ophthalmol 95.81-93. (German).
[12]Reidner ED, Levin LS, Holliday MJ.1980, Hearing patterns in dominant Osteogenesis Imperfecta.Arch Otolarngol.106.737-740
[13.] Byers PH, Steiner RD, 1992, Osteogenesis Imperfecta .Annu Rev Med 1992, 43.269-282
[14.]Kuivaniem H,Tromp G,Prockop DJ 1997,Mutations in fibrillar collagens(Type I,II,II and XI) ,fibril associated collagen Type IX and network forming collagen Type X cause a spectrum of diseases of the bone, cartilage ,and blood vessels. Hum Mutat 9.300-315
[15.] Byers PH, Steiner RD 1992MOsteogenesis Imperfecta Annu.Rev.Med.43.269-282
[16.]Skyes B,Ogilvie D,Wordsworth P,et al 1990,Consistent linkage of dominantly inherited Osteogenesis Imperfecta to the Type I collagen Loci.COL 1A1 and COL1A2,Am J Hum Genet 1990,46.293-307.
[17.] Berger G, Hawke M, Johnson A, Proops D.Histopathology of the temporal bone in Osteogenesis Imperfecta Congenita. a report of 5 cases. Laryngoscope 1985, 95.193-199)
[18.] Zajtchuk JT, Lindsay JR.Osteogenesis Imperfecta Congenita and tarda. A temporal bone report .Ann Otol.1975, 84.350-358
[19.] Sando I, Myers D, Havada T, Hinojosa R, Myers E Osteogenesis Imperfecta tarda and Otosclerosis, a temporal bone histopathology report. Ann otol 1981, 90.199-203
[20]Marion MS, Hinojosa R. Osteogenesis Imperfecta Am J Otolaryngol 1993, 14.137-138.
[21.]Tabor EK ,Curtin HD,Hirsh BE, May M .Osteogenesis imperfecta tarda.appearance of the temporal bones at Computed Tomography .Radiology 1990,175.1810183
[22.]Mafee MF,Valvasson GE,Ditch RL,et al .Use of the CT scan in the evaluation of Cochlear Otosclerosis.Radiology 1985,156.703-708
[23.] Hoddsmorth CE, Endahl GL, Soifer N, et al Comparative biochemical study of Otosclerosis and Osteogenesis Imperfecta .Arch oto-laryngol 1973, 98.336-339
April 2014 Supplement 1 Classics and Revisits in Scientific Rheumatology
[24.] Dent JA, Paterson CR.Fractures in early childhood.Osteogenesis imperfecta or child abuse? J Paediatr Orthop 1991; 11:184-186.
[25.] Ablin DS, Greenspan A, Reinhart M, Grix A, Differentiation of child abuse from Osteogenesis Imperfecta.Am J Roentgenol 1990.154:1035-1046
[26.] Paterson CR, Mole PA.Bone density in Osteogenesis Imperfecta may well be normal Postgrad Med J 1994:70:104-107.
[27.] Williamson MR, Boyd CM, Williamson SL Osteoporosis: Diagnosis by Plain chest film versus dual photon absorptiometry.Skeletal Radiol 1999; 19:27-30.
[28.] Miller ME. Temporary brittle bone disease: a true entity? Semin Perinatal 1992; 23:174-182.
[29.] Koo WWK.Sherman R, Succop P et al.Fractures and rickets in very low birth weight infants: Conservative management and outcome.J Paediatr Orthop 1989; 9:326-330.
[30.]Cremin B, Goodman H, Spranger J, Brighton P, Wormian bones in Osteogenesis Imperfecta and other disorders. Skeletal Radiol 1982; 8:35-38
[31.]Hawlik DM ,Nashesky MB,Ruptured cerebral artery aneurysm and Bacterial meningitis in a man with Osteogenesis Imperfecta.American Journal of Forensic Medicine and Pathology June 2006,27(2).117-120
[32.]Modestino G,Criscitiello M ,James A ,et al Cardiovascular abnormalities in Osteogenesis Imperfecta .Circulation 1965,31.255.
[33.]Wong R.S Follis.F, M Shively.B.K, Wernly .J.A.Osteogenesis Imperfecta and Cardiovascular diseases Ann.Thorac.Surg.November 1.1995, 60(5).1395-1397.
[34.] Moore.J.B, E.G.Wook and Kinkead.L.R.Ulnar artery aneurysm in Osteogenesis Imperfecta.J.Hand Surg Eur vol, February 1983.Original series, volume 15(1).91-95.
[35.]Ganesh.A, Jenny.C,Geyer.J.Shouldice M,Leven A, Retinal haemorrhages in type 1 ostegenesis imperfecta after minor trauma.Opthalmology.Volume 111,Issue 7.Pages 1428-1431.
[36.] Martin, R.B, Burr .D.B. (1989) The structure, function and adaptation of compact bone (Raven, New York), pp.57-84.
[37] McMahon P,Grossman W,Gaffney M.Stanitski C.Soft tissue injury as an indication of child abuse.J Bone Joint Surg 1995;77A:1179-1183.
[38.]Paterson CR, Monk EA.Long term follow up of children thought to have temporary brittle bone disease.Osteoporos Int 2000;11(Suppl 4):S47-S48
April 2014 Supplement 1 Classics and Revisits in Scientific Interdisciplinary Medical Themes CRS [Int.Disc.Med.Them.]
Proteus syndrome in childhood with a selective literature review.
Guest Editors: CRS [Med] Consulting Academic Clinical Editorial Experts.
The need for an Editorial review article on Proteus syndrome PS.
An editorial review article following academic clinical experts consultations on PS is undertaken on the basis of concerns evoked following observations on few compatible cases encountered over a considerable time. Given the progressive compressive, the vascular and malignant neoplastic implications of PS,a high index of suspicion should be expedient for an early and timely exclusion or diagnosis for PS,especially those cases presently covertly, or with minimal phenotypic expressivities or attenuations.
Case Definition
Proteus syndrome (PS) is a rare sporadically occurring hamartomatous disorder
associated with mosaic multifocal irregular asymmetric overgrowth of multiple body
tissues and cell lineages. The aetiology of Proteus syndrome is still idiopathic.
However, the predominant hypothesis is that PS is caused by a postzygotic mosaic
alteration in a gene that is lethal in its nonmosaic state.
Most malformations in Proteus syndrome have a predominantly ectodermal or
mesodermal origin.
Characteristic multifocal, multicentric, metachronous or synchronous overgrowths
(partial or regional gigantism, or hemi hypertrophy) in Proteus syndrome could involve
any body structure, especially the skin, bone, connective tissues, and fat.
Globally, published or unpublished reports of PS , are still very few.
A cluster of phenotypic features evoking the possibility of PS ..
On closer scrutiny,observations and follow ups, a couple of children presented with some of the spectral phenotypic dysmorphological features of PS such as lower limb hemi-hypertrophies, cerebriform cutaneous hyperplastic unilateral sole overgrowths, widespread hyperpigmented capillary haemangiomatosis on the limbs and extremities, saccrococcygeal, gluteal, perineal protuberant lipomatous masses,congenital absence of the gluteal cleft and progressive abdominal tumefactions.
Establishing PS as the most plausible diagnosis.
Following the observations of these features consistent with the recognized clinical diagnostic criteria for Proteus syndrome, the possibility of .this diagnosis was evoked and suggested, on follow ups ,with progressive enlargement of these structures the probability of Proteus syndrome was considered and discussed.
Rare embryonic neoplasms in PS
Following focused radiological imaging procedures the diagnosis of Proteus syndrome complicated by nephroblastoma, multifocal neuroblastomas and embryonic tumours was established in these cases.Although a range of malignancies have been previously reported in PS, embryomas were relatively uncommon especially Wilms tumour and Nephroblastom as they were demonstrated in the editorial observations
April 2014 Supplement 1 Classics and Revisits in Scientific Interdisciplinary Medical Themes.
The diagnostic and therapeutic difficulties in PS
The diagnosis and management of Proteus syndrome is challenging, enigmatic and
difficult and were controversially discussed in the medical literature.
Proteus syndrome could be associated with synchronous, metachronous,
multicentric, multifocal, phenotypic expressivities and intrabdominal embryomas
Literature search to aid the diagnosis. PS.
In establishing this diagnosis,the extant,and current medical literature were briefly reviewed to examine the rarity and strengths of these associated structural features in achieving the diagnosis of this syndrome against its other differentials. Other methodological issues concerning its epidemiology, presentations, diagnosis and management were also proffered and discussed.
Case definition.
Proteus syndrome (PS) is an extremely rare disorder of patchy or mosaic postnatal
growth dysregulation, primarily involving overgrowth. The disorder is thought to be
related to a somatic genetic alteration ,but its exact etiology is still largely unknown.
Proteus syndrome was named after the Greek god Proteus who could change his shape at will.This rare disorder is characterized by multiple lesions of the mesodermal tissues, fat cells,lymphatic and blood vessels (lipolymphohaemangiomas), overgrowth of one side of the body(hemihypertrophy), an abnormally large head (macrocephaly), partial gigantism of the feet, darkened spots or moles (nevi on the skin).
Historical Perspective and Nomenclature of interest.
Studies on Proteus syndrome commenced in earnest in 1978, when Bennett proposed a putative experimental model for the mode of inheritance in PS and other related syndromes associated by a dominantly determined genomically patterned inheritance with mosaicism sustaining the survival of a naturally lethal gene.[1]
Although some evidence of this syndrome was originally published in the medical
literature as early as in 1907 or before, the more modern medical description of the
disease was attributed to Cohen and Hayden, who identified the syndrome in 1979.[2]
To stress the polymorphic nature of the clinical manifestations of this new
syndrome, in 1983,Wiedermann coined the term Proteus syndrome after the Greek mythological god Proteus (meaning polymorphous), the Old man of the Sea who could change his shape at will to avoid capture. [3]
The Elephant Man had PS on retrospective analysis.
Anecdotally, Joseph Merrick, the Elephant Man described by Sir Fredrick Treves in 1884
celebrated by stage plays and movies with the same name, was initially thought to have
suffered from elephantiasis and latter on a diagnosis of neurofibromatosis was suggested
, but now believed retrospectively to have had a Proteus syndrome after a study of the preserved castings of his soles revealed a cerebriform cutaneous hyperplasia, a characteristic finding in Proteus syndrome. [4][5]It was in 1996 that the answer to what affected Merrick was determined with exact certainty . A radiologist, Amita Sharma, of the National Institutes of Health (U.S.A), examined very meticulously the x-rays and CT scans of Merrick’s skeleton preserved at the Royal London Hospital after his death.
April 2014 Supplement 1 Classics and Revisits in Scientific Interdisciplinary Medical Themes
Dr.Sharma in 1979. determined that Merrick had Proteus syndrome, an extremely rare disorder, she asserts that Merrick’s appearance, and especially his skeleton, portrays all the identified stigmata of the defect in their extreme.
Folk-lores on the Elephant man
Historical legends had it that the head of the elephant man was so large that his hat measured about three feet in diameter.
Undoubtedly, Joseph Carey Merrick the elephant man (1862-1890) wanted to be like other people. He often wished he could lie down and sleep, but because of the size and weight of his head he had to sleep sitting up. One morning in 1890 he was found lying down in bed on his back, dead. The weight of his head had compressed his airway, and he suffocated. He was aged 28 years old at the time of his death.
Retrospectively, in 1983, Weidemann suggested that the patient reported by Graetz in
1928 could have had Proteus syndrome. [6]and that another case reported by Temtamy and Rogers in 1976 had features consistent with PS [7]
An earnest Search for the phenotypic features compatible with PS
The same year, Weidemann et al described a new syndrome in four unrelated boys with a
constellation of clinical features of partial gigantism of the hands and or the feet, nevi,
hemihypertrophy, subcutaneous tumors, macrocephaly or other skull anomalies, possibly
accelerated growth and visceral affectations which were in keeping with Proteus syndrome. [3]Additional advancements in the comprehension of the
pathomorphology in Proteus syndrome led to relevant updates in 1984.[8]
That same year, Wiedemann and Burgio studied the extent and the nature of the dermal
changes in Proteus syndrome and asserts that they were papillomatous epidermal nevi piquantly aligned along the lines of Baloshka.[9]
Time lines of the Investigations on PS
Several Investigations were undertaken on PS from 1984 to 1990
Further investigations into the pathogenesis and phenotypic expressivity in
Proteus syndrome was undertaken by Lezama and Buyse et al still in 1984[10]
In the same year, Gorlin described another case with features consistent with Proteus
syndrome. [11]
Lipomatosis as an association of PS was highlighted In 1985
Mucke et al et al highlighted the dynamism of the clinical features of the Proteus syndrome by illuminating on the natural history of lipomatosis in I985 [12]The same year, further reports on the multi-focality of Proteus syndrome were made by Costa et al.Two cases were reported, with both having abdomino-pelvic lipomatosis.This was noted in one of the cases aged seven who retrospectively had a conjuctival dermoid at the age of thirty six months.Laparatomy at the age of seventy two months for an acute abdominal pain revealed a right iliac fossa lipomatosis and a twisted necrotic mesenteric fat as the implicated aetiopathogenic factor for the pain.Some morphological similarities of PS to the Bannayan-Zonana syndrome and linear sebaceous nevus syndromes were demonstrated. [13]With further case enrollments into these series in the same year by Costa et al, they asserted the paternal age independence of these cases in a study of ten cases, with a mean paternal age of 30(range 23 to 40) which were not unusually extreme.
April 2014 Supplement 1 Classics and Revisits in Interdisciplinay, Medical Themes.
The alignment of dermopathy of PS along the lines of Baloscka was noted in 1986
Happle pointed out that the lesions follow the dermal lesions along the lines
Baloschka.Happle had proposed that the likely putative aetiopathogenic pathway in PS is
an autosomal dominantly determined genomically transmitted lethal gene persisting by
mosaicism akin to hypothetically proven aetiopathogenic mechanisms propagating the
MCCune-Albright syndrome and probably the Schimmelpenning-Feuerstein-Mims
syndrome [14,15]
Association of PS with adherent lipomatosis and haemangiomas reiterated in 1987
Clark et al reported eleven cases of PS syndrome all with the remarkable
association of lipomatosis. [16] The same year in 1987, a case series was reported in Southern Africa by Viljoen et al [17], in the same year they mentioned that the surgical removal of lymphatic, fatty or hemangiomatous elements could be difficult ,usually resulting in unsightly scars and keloids,at about the same period a further case of a child aged fifty four months who was severely affected by Proteus syndrome was reported by Malamitsi-Puchner et al. [18] Between 1987 and 1988, further works were undertaken retrospectively by Cohen with regards to the Elephant man. [4, 19, 20]
Further aspects of the dermopathies of PS were highlighted in 1988,
Viljoen et al described the skin manifestations of the Proteus syndrome in all of their six patients, with marked hypertrophy of the skin of the soles, being a consistent and universal feature of this syndrome that the authors inferred that it was almost pathognomonic for the syndrome. In three cases, additional features such as large epidermal nevi and linear macular lesions with areas of depigmentations were also demonstrated.
On light microscopy, the affected skins from the soles were noted to have an elongation
of the cytoplasm of the basal cells.
Bannayan-Zonana syndromes suggested as a possible differentials of PS
The differential diagnosis of the Proteus and Bannayan-Zonana syndromes were discussed by Bailer et al in 1988In their examination of the relevant literature they noted a history of consanguinity in 5.56% of their cases with compatible Proteus syndrome.
The overlap within syndromes that include hamartoma as eloquent features intimates that
these syndromes may be aetio-pathologically related, probably with regards to
perturbations in the secretion of a hypothetical humoural growth factor or an irregular tissue or receptor responsiveness to this growth factor.[21]
Malignant transformation of PS lesions exemplified by progressions to testicular malignancy.
Samlaska et al. in 1989 reported an archetypical case and reviewed thirty four others reported sporadically. [22], Malamitsi-Puchner et al in 1990 gave an account of the
follow up of the cases that they studied and reported earlier in 1987.
A striking tissue overgrowth occurred after surgical interventions in this child who latter
developed testicular malignancy. [23]Still in 1990, Beluffi et al reported a case of Proteus syndrome with pelvic lipomatosis and illustrated the use of a computer assisted tomographic scan for demonstrating pelvic lipomatosis.
April 2014 Supplement 1 Classics and Revisits in Scientific InterdisciplinaryThemes The musculoskeletal associations of PS highlighted.
Further investigations in 1990 by Hotamisligil and Ertogan described the case in
a nine month old girl who in addition to other features, had soft tissue masses in the
paravertebral and gluteal areas with aggressive involvement of the spinal canal and a
hyperpigmented epidermal nevus with hyperkeratosis on one side of his body.
He had a bipedal and unilateral left palmar and dorsal macrodactyly with syndactyly of
the third and fourth left digits. In this instance, nephrogenic diabetes insipidus was
described in Proteus syndrome for the first time.[24 ]
Familial transmission of PS demonstrated.
Although traditionally considered a sporadic congenital disorder, some anecdotal reports
have proposed the possibility of a familial transmission in Proteus syndrome.
Goodship, et al in 1991 presented a possible case of father to son transmission of Proteus
syndrome. The son had a cranial hemihypertrophy, a lymphangioma, a lipomata, and
epidermal nevi, where as the father had a large lymphangioma resected from the right
side of his face as a child, given this association, the possibility that his father was a mosaic was proposed. [25]
The possibility of PS presenting with minimal phenotypic expressivity or attenuation highlighted.
Kruger et al in 1993, observed mild Proteus syndrome in a boy whose mother had very
attenuated manifestations of PS. She had facial asymmetry with hypertrophy of her right lower cheek and impaired facial expression in that region. He was not cognitively impaired, but he a had minimally expressed left unilateral superior labial hypertrophy and cheek, causing a perilesionally lateralized impaired focal facial expressivity, hypertrophy of the left arm, partial gigantism of the left middle finger, and a large subcutaneous swelling in the upper left abdomen thought clinically and sonographically to be a lipomata.Both this mother and her son had distinctive superiorly localized venous markings. [26]
The probability of spinal cord compression from related structural defects suggested in PS.
Also Skovby et al in 1993 reported two illustrative cases of the two plausible
pathways by which spinal cord compromise may develop in Proteus syndrome:
In the first instance, spinal stenosis resulted from an anomalous vertebral architectonics
such as angular kyphoscoliosis.In the second case, cord compression resulted from tumoural infiltration most likely froma paraspinal, intrathoracic angiolipomata.[27]
Somatic Mosaism and further associated organic structural defects in PS remarked.
Two unusual cases of PS were further reported by Cohen which supported the concept of
somatic mosiacism.In one case, a huge connective tissue nevus covered the anterior
thoraco-abdominal walls, in addition to multifocal calvaria hyperostoses.
In the other subject, linear verrucous epidermal nevi, epibulbar dermoids, and
hyperostoses were demonstrated. However no appendageal, digital or superficial plantar
enlargements were demonstrated in these instances.In the same year further review of selective aspects of Proteus syndrome, including uncommon neoplasms, pulmonary and renal abnormalities, brain malformations,and patterns of abnormal growth in the craniofacial skeleton were undertaken by Cohen.
April 2014 Supplement 1Classics and Revisits in Scientific Interdisciplinary Medical Themes,
At this point, the presence of external auditory meatal hyperostosis was taken as a very specific feature of Proteus syndrome. [28]
The multifocality of PS lesions demonstrated.
Smeets et al in 1994 reported another patient with a cranio-facial manifestation of
Proteus syndrome. In this case, multi-focal calvarial, facial bony, external auditory
meatal and mandibular hyperostoses were the principal regional features demonstrated.
Also a scleral tumor was demonstrated in this patient. These observations were interpreted as lending a further support to the hypothetical concept of somatic mosaicism. [29]
Further aspects of malignant transformations in PS were reiterated.further
Gordon et al in 1995, observed two patients with Proteus syndrome who progressed to
malignant transformations. One case had a probable mesothelioma, in addition to features
suggestive of thyroid papillary carcinoma, which was not ruled out with
exact certainty pre-mortem, this case latter died at the age of five whilst asleep, at
autopsy, a papillary neoplasm, most likely of mesothelial origin, involving the inferior
surface of the diaphragmatic musculature and infiltrating into the omentum, the pelvic
area, the scrotum and some of the mesenteric lymph nodes were demonstrated.
The other case had bilateral ovarian serous cystadenomas with nuclear atypia identified at six years three months of age, warranting.a right ovarian oophorectomy, invasion of the right fallopian tube was also noted. During this period a catalogue of uncommon neoplasms in Proteus syndrome was compiled and proffered. [30]
Multiple organomegalies and Visceromegalies as an association of PS.
Lacombe and Battin, in 1996 described two unrelated children diagnosed at birth with
isolated macrodactyly .At follow ups they were noted to have developed hemihypertrophy. Three dorsally located angiomas were seen in one of these cases,
a female child, when she was aged four years. The clinical features encountered in both cases were consistent with the specific diagnostic criteria for Proteus syndrome. [31]
Ceelen et al in 1997described a man with Proteus syndrome who sustained an enlarged
splenic rupture while horse riding. [32]
Splenomegaly as a common feature of PS
Biesecker et al in 1998 described their experience with eighteen cases with a
presumptive diagnosis of Proteus syndrome.Splenic hyperplasia was noted to be a recognized feature of PS. The spleen was enlarged in two of the eighteen cases, and another case with asymptomatic splenomegaly and thymic hyperplasia was known to them earlier on. However, they annotated that it was likely that hemi-hyperplasia commonly over simulates Proteus syndrome [33]
In the same year Biesecker et al described a distinctive subset characterized by static or
mildly progressive hemi-hyperplasia and multiple lipomata.
April 2014 Supplement 1 Classics and Revisits in Scientific Interdisciplinary Medical Themes.
A review of the diagnostic criteria for PS
The recommendations for the diagnostic criteria, differential diagnosis, and
guidelines for evaluation of patients with Proteus syndromes which were developed at a
workshop held at the National Institutes of Health in 1998 were reviewed by
Biesecker et al.The general criteria suggested as mandatory for the diagnosis of PS were mosaic distribution of lesions, a progressive course, and a sporadic occurrence.
Pathognomonic Features for the diagnosis of PS suggested.
Specific clinical manifestations also were suggested as necessary to meet the diagnostic
Criteria for PS. Connective tissue nevi, which were common manifestations in Proteus
syndrome, were considered almost pathognomonic for the syndrome, although they were
not always present in all cases, where the diagnosis of PS was made.
Other combinations of manifestations (e.g., epidermal nevus, disproportionate overgrowth, specific tumours were suggested to meet the diagnostic criteria. [34]
Describing other mutational variants of PS
Happle in 1999 suggested the designation elattoproteus syndrome for a disorder that he considered to be an inverse form of Proteus syndrome.
He described a seven year old boy with partial lipohypoplasia and patchy dermal hypoplasia involving large body surface areas. These areas of attenuated growth were similar to those described in many cases of Proteus syndrome. Paradoxically; however, he had a minimally expressed inordinate overgrowth defects.Happle proposed to explain this unusual phenotype by suggesting that there may be various allelic mutations in the putative gene locus responsible for Proteus syndrome, which gives rise to overgrowth of somatic tissues.
Pleioproteus and Ellattoproteus as mutational variants extremes of PS.
Such mutations could be called Pleioproteus alleles, a term derived from the Greek word pleion meaning plus. Conversely, the same gene locus may harbor alleles responsible for deficient growth of somatic tissues. Mutations in these points were referred to as Ellattoproteus alleles after the Greek word elatton, meaning minus.
Patients affected with Proteus syndrome may show classic overgrowth or a composite picture with Pleioproteus and Elattoproteus lesions or even an isolated putative previously uncharacterized elattoproteus phenotype [35]
Ophthalmopathy of PS highlighted.
De Becker in 2000 described the ocular manifestations in a case of Proteus
syndrome and reviewed the ocular features demonstrated in published cases. [36]
Also the same year ,Hodge et al described a ten year old boy with Proteus syndrome
who presented with an effusive pericardial disease, a panhypogammaglobinaemia, with a specific deficiency in IgG and IgA subsets accompanied by low levels of specific antibodies to pneumococcal and hemophilus type B polysaccarides, and global lymphopenia.Given that no other cause was found for this immune deficiency in this case of PS, the authors suggested that it may represent a previously unrecognized feature of Proteus syndrome. [37]
April 2014 Supplement 1 Classics and Revisits in Scientific Interdisciplinary Medical Themes
Cognitive impairments associated with neuronal migration disorders and ophthalmopathies demonstrated in PS
Further work on PS was undertaken by Gilbert-Barnes et al, in 2000, who reported an exceptional patient with Proteus syndrome in whom manifestations included multifocal meningiomas, polymicrogyria, and periventricular heterotopias, with both eyes having epibulbar cystic lesions. The retina showed diffuse disorganization with nodular gliosis, retinal pigmentary abnormalities, chronic papilledema, and optic atrophy. Other abnormalities noted included progressive cranial, mandibular, maxillary, and auditory canal hyperostosis, epidermal nevi, and mental sub-normality. His limbs, hands and feet were noted to be proportionate and otherwise normal. [38]
Deleterious Pulmonary embolism observed in PS
The same year, Slavotinek et al reported three patients with Proteus syndrome who died suddenly from pulmonary embolism related to different clinical circumstances.
The first patient, who was diagnosed with Proteus syndrome at the age of twelve years, had varicose veins, portal vein thrombosis, right iliac fossa vein occlusion and recurrent pulmonary embolism. At the age of twenty five years old he died from pulmonary embolism. The second patient was a nine year old male who collapsed and died at home. Autopsy asserted the cause of death to be pulmonary embolus associated with deep vein thrombosis. The third patient was a seventeen year-old female undergoing inpatient treatment for sinusitis when she suddenly died. Autopsy showed a large pulmonary embolus with no identified deep vein thrombosis.
The need for relevant historical enquiry for PS and evaluation for thrombogenicity to direct anti-thrombogenic prophylactic measures in PS reiterated.
Given the fatality related to these complications, Slavotinek et al promptly suggested that patients undergoing surgical procedures should be evaluated for coagulopathic potential to determine whether antithrombotic prophylaxis will be appropriate for them [39]Cohen in 2001 reviewed at least seventeen reported cases of premature deaths in Proteus syndrome and suggested that patients with this disorder and/or their families should make their health care providers aware of the risk of deep venous thrombosis and pulmonary embolism. [40 ]
Biesecker in the same year reported Proteus syndrome in a five year old case and made a selective literature review.[41 ]
Controversies on the role of PET Gene mutations in PS.
Further work on the molecular genetic aspect of PS was undertaken by Barker et
al in 2001, however they did not identify mutations in the PTEN gene in eight unrelated patients with classic Proteus syndrome, hence suggesting that the mutation exclusion hypothesis may not be unfounded.[42]
April 2014 Supplement 1 Classics and Revisits in Scientific Interdisciplinary Medical Themes
Obstipation demonstrated in PS, a consequence of infiltrative colonic lipomata.
Mackay et al in 2002 reported a twelve year old boy with Proteus syndrome who had presented with gross abdominal distension and severe intractable obstipation.
At an Axial T1- weighted MRI, a diffuse hyperintense tissue, signal typical of fat surrounding and separating bowel loops, with a lesional aspect extending posteriorly on the left into the paraspinal musculature, displacing the psoas muscle anteriorly was
demonstrated. At laparatomy a huge infiltrating lipomatous mass was identified encasing the left colon, including the rectum. [43]
Induced abortion with Misoprostol by a mother related to birth of a baby with PS
Mohameddbhai et al. also in 2002 reported the case of a newborn male Proteus
syndrome case whose mother had ingested misoprostol, an orally active prostaglandins,
at six weeks of gestation in an attempt to abort that conception. [44 ]
Further definitions of diagnostic criteria for PS
Turner et al in 2004 reviewed two hundred and five reported cases of Proteus syndrome.
Interestingly, of all these lot, only ninety seven were thought to have strictly met the diagnostic criteria of PS, eighty cases clearly did not meet the criteria; and although twenty eight cases had features suggestive of Proteus syndrome, there were insufficient clinical data to substantiate this diagnosis.
Follow up adverse events on putative PS cases using a stringent criterion.
On follow up, the reported cases that met the strict criteria for Proteus syndrome had a higher incidence of premature deaths and other complications (Scoliosis, megaspondyly, central nervous system abnormalities, tumors, otolaryngologic complications, pulmonary cystic malformations, dental and ophthalmic complications) compared to those in the non-Proteus group. Cases that met the criteria were more often males, which had implications regarding the hypothesis for the etiology and pathophysiology of Proteus syndrome.Cerebriform connective tissue nevi (skin lesions characterized by deep grooves and gyrations as seen on the surface of the brain, which are striking features when seen on the hands and feet) were considered characteristic.
Specific later onset tumours in PS
Specific tumors occurring before the second decade include ovarian cystadenomas
and parotid monomorphic adenomas. Lung cysts were also added as a criterion.[45]
The Controversies surrounding PTEN in PS clarified.
Thiffault et al in 2004 reiterated that the most plausible suggestion for the genetic
basis of Proteus syndrome is the Happle somatic mosaic hypothesis, although no somatic
mutations in candidate genes had been reported, because germ line mutations in the
PTEN gene had been identified in patients diagnosed with Proteus syndrome.
In the same year, Thiffault et al screened affected and unaffected tissue from six patients
with Proteus syndrome by direct sequencing of genomic DNA for germ line or somatic
mutations in the PTEN or GPC3genes. No intraexonic mutations were identified,
indicating that neither PTEN nor GPC3 was likely to have a major role in the etiology of
Proteus syndrome in this series of cases. [46 ]
April 2014 Classics and Revisits in Scientific Interdisplinary Medical Themes
Post zygotic mutations associated Mosaicism suggested as a causality factor in PS
Brockmann et al in 2008 reported a pair of monozygotic nine year old male twins
discordant for Proteus syndrome. The affected boy showed progressive postnatal
overgrowth of his right lower limb and foot with asymmetric progressive overgrowth of
single toes. There was a small cerebriform connective tissue nevus on his right fourth toe.
The phenotype was mild but still fulfilled the diagnostic criteria for Proteus syndrome.
The findings supported the hypothesis that this condition is caused by a post zygotic
mutation event resulting in mosaicism. [47]
Further discussions on establishing the diagnosis of PS:
Although the exact aetiology of Proteus syndrome is still idiopathic; however the
predominant hypothesis is that Proteus syndrome is a consequence of a post zygotic
mosaic alteration in a gene that is lethal in its non mosaic form, however, Proteus like syndromes were associated with PTEN mutations.
Somatic mutations and defective production or regulation of tissue growth factors or receptors as a causality factor in PS
The concept of a somatic mutation that affects the production or regulation of tissue
growth factors or their receptors could explain the sporadic occurrence, the random
distribution of overgrowth, and the wide range of findings within the phenotype.
Many features of Proteus syndrome overlap with other overgrowth syndromes.
A Proteus like syndrome was previously reported in association with a germ line and
tissue-specific somatic mutations in the PTEN [48, 49, 50] These PTENS genes are usually mutated in Cowden syndrome and in Bannayan-Riley-Ruvalcaba syndrome (BRRS)
Confounding diagnostic difficulties with PS due to other overlapping syndromes.
Some of the phenotypic features of this syndrome overlap with those of the other
overgrowth syndromes such as the Klippel-Trenaunay-Weber syndrome, the
Ollier disease and the Maffucci syndrome occasionally posing some diagnostic
challenges. [51]Although chromosomal alterations have not been demonstrated with routine cytogenetic studies as such in Proteus syndrome, however the karyotype in two patients with Proteus syndrome showed structural abnormalities of chromosome 16 and chromosome 1 in a mosaic distribution, further supporting the hypothesis of somatic mutation. This concept of a somatic mutation that involves tissue growth factors or their receptors could explain several aspects of Proteus syndrome, such as the mosaic distribution of lesions, its sporadic occurrence, the unaffected offspring from the affected individuals, and the existence of discordant twins. Despite the evidence implicating a somatic mutation, no causal gene mutations were known for Proteus syndrome.
However, ongoing advances in the comprehension of the aetiologic-phenotypic
correlations in the context of supposedly genetic defects appear to make the future more
certain. [52]
Striking a balance in the diagnostic criteria for PS.
The most appropriate criteria for the exact diagnosis of Proteus syndrome with much certainty were controversially discussed. Although under diagnosis could be more common, most stringent diagnostic criteria is commonly advocated over simplistic
overlapping generic ones to facilitate appropriate research investigations and utmost
clinical care.The strengths of most stringent criteria are that they define an entity with an
apposite level of congruity in clinical features and outcome which could lead to an
enhanced comprehension of the inherent putative pathogenic mechanisms in these cases [41,33,34 ]
Plausible diagnostic, prophylactic and therapeutic interventions for PS
The aggressive and disproportionate overgrowth in PS is progressive involving mostly the musculo-skeletal systems and these orthopaedic complications could be difficult and challenging to manage. An optimal intervention will imply an adequate knowledge of a spectrum of manifestations and complications of the disorder and a multidisciplinary approach involving the geneticist, surgeons and other specialists.
The diagnosis of Proteus syndrome could be achieved without routine cytogenetic
studies, which have demonstrated inconsistent chromosomal aberrations.
The determination of the molecular aetiology of this syndrome will be difficult, given its
extreme rarity, the abbreviated life span in affected subjects, and its sporadic occurrence,
making the conventional approach of traditional cloning less useful.
Novel molecular genetic approach for the diagnosis of PS.
The aetiology of this disorder could however be studied using various comparative molecular biological techniques such as c DNA arrays, genomic arrays, subtractive techniques, testing of candidate genes and other appropriate techniques.
In addition to testing for dysregulations of growth hormones and binding proteins in vivo.
A novel systematic diagnostic classification scheme for PS
An updated revised criteria was adopted for PS in 2014 [45], differing from that undertaken in 1999. [33] and other more generic less stringent ones which allowed the diagnosis of PS to be made in patients presenting ordinarily with hemi-hyperplasia. [34,53, 54].Its diagnosis requires the presence of all general criteria and various specific criteria, including the presence of the category A criterion or two category B criteria or three category C criteria. The affected subjects should have the following general criteria, mosaic distribution of the lesions, progressive course, and a sporadic occurrence.
The specific PS diagnostic criteria are as follows:
[I] +Category A-Cerebriform connective-tissue nevus.
[II] +Category B-Epidermal nevus; asymmetric, disproportionate overgrowth (limbs,
skull, external auditory meatus, vertebrae, and/or viscera); and specific tumours that
occur most commonly in the second decade (i.e., ovarian cystadenoma, parotid
Monomorphic adenoma)
[III]+Category C-Dysregulated adipose tissue (lipomas, regional lipohypoplasia)
, vascular malformations (capillary, venous, and/or lymphatic), lung cysts, and facial
phenotype..
The argument for a high index of suspicion for the diagnosis of PS
The variably mild form of this syndrome with a less eloquent phenotypic expressivity could be missed initially or entirely until catastrophic complication of DVT or PE renders it suspicious and the diagnosis now made in retrospect. Neuroimaging with Skull X-ray, CT or MRI will be most informative for those with significant facial dysmorphism, mental subnormality or epileptogenesis.Compressive neuropathies could follow tumourigenesis or bony exostosis.
April 2014 Supplement 1Classics and Revisits in Scientific Interdisciplinary Medical Themes
PS is a progressive and dynamic overgrowth condition.
Occasionally, the mild or moderate effects of Proteus syndrome, such as bening tumours, are present at birth. As the child grows and develops, the tissue overgrowth progresses and changes. This progression is often irregular, it is characterized by periods of major overgrowths and other periods of absent overgrowth.The effects therefore change over the course of a time.
PS is associated with ambulatory difficulties and compressive neuropathies.
However, most changes occur before adolescence, since tissue over-growths tend to plateau at that time.Patients with Proteus syndrome have difficulty ambulating because of toe macrodactyly, scoliosis, and joint instability, with frequent dislocations, expansive subcutaneous tumours and compressive neuropathies due to intraneural hamartomas.
Some patients may have persistent atelectasis, pneumonia, or symptoms of pulmonary
Strict diagnostic criteria, guidelines and algorithms has been proposed and accepted. Those features which are considered to have diagnostic values are considered because of the tremendous variability in the manifestations of Proteus syndrome.
These features could be classified as cutaneous or noncutaneous.
Cutaneous manifestations
Almost always, all patients with Proteus syndrome have at least one type of cutaneous
lesion, of which several different types have been reported. These skin lesions could
generally be classified into two groups, those that are congenital or neonatal in onset and are stable in the first group and those that are post neonatal and progressive in the second
group. In some patients, the presenting symptom may be one or more of the many types
of heterogeneous skin lesions that characterize Proteus syndrome.
These include cerebriform connective –tissue nevi, epidermal nevi, vascular hamartomas, lipomatas and hyperpigmented or hypopigmented areas. Epidermal nevi and vascular malformations are considered group one lesions, while lipomatas and cerebriform connective tissue nevi generally occur latter and are considered group two lesions.
Group 1 cutaneous lesions are characterized as follows:
Epidermal nevi are a common finding in patients with Proteus syndrome.
These manifest at birth as tan-to-brown, flat topped, hyperkeratotic or verrucous papules,
which run in a linear or whorled pattern along the lines of Blaschko.
Epidermal nevi are found asymmetrically, scattered around the body.
Histologic features are acantholysis and hyperkeratosis in a clinical lesion consistent with an epidermal nevus.
Congenital Vascular malformations are other common cutaneous features in PS.
These anomalies can be of venous, capillary, or lymphatic origin and include nevus
flammeus, angiokeratomas, cavernous hemangiomas, superficial and deep lymphangiomas, and varicosity of the superficial veins. [55, 56]
These lesions are developmental abnormalities and they grow proportionately through a
patient’s lifetime without regression .In an occasional patient these vascular malformations have been noted to expand beyond proportionate growth.
Prominence of veins may be enhanced in areas of regional lipodystrophy.
April 2014 Supplement 1 Classics and Revisits in Scientific Interdisciplinary Medical Themes.
Group Two cutaneous lesions are characterized by the presence of cerebriform nevi
Cerebriform connective-tissue nevus is one of the most common and characteristic
features of Proteus syndrome, although its presence is not required for diagnosis and its
presence alone is not pathognomonic for Proteus syndrome, although its presence is not
required for diagnosis and its presence alone is not pathognomonic for Proteus syndrome.
An occasional patient developed an isolated plantar cerebriform collegenoma who lacked the general diagnostic criteria for Proteus syndrome. These nevi have a relatively delayed onset, making the diagnosis of PS difficult in neonates and infants.
Cerebriform nevi occur as well-demarcated, skin coloured plaques with a cerebriform or
rugated appearance. Most often they occur in the cutaneous and subcutaneous tissues on
the palms or soles but have also been noted on the forearm, on the trunk, and inferior to the ala nasi.Lesions on the sole typically cause the most morbidity because they cause ambulation difficulties, are usually prone to ulceration and infection, and are malodorous.Histopathologically, the lesions consist of an irregular whorly non-lipomatous proliferation of highly collagenised cellular fibrous tissue.
Lipolymphohaemangiomas are characteristic features in PS
In PS adipose tissue abnormalities are common, resulting in lipomatas and areas of lipohypoplasia.Lipomatas occur as hamartomatous masses consisting of subcutaneous
tissue or a variable combination of adipose, lymphatic, and hemangiomatous components
(eg, lipolymphohaemagiomas) and are the second most frequent type of skin findings that
characterize Proteus syndrome. The soft subcutaneous tumours can affect any area of the
body but are most common on the head, abdomen, groin, or legs. They appear as soft,
skin coloured nodules or tumours.Patchy areas of lipohypoplasia or dermal hypoplasia
are also observed in some patients.Lipohypoplasia occurs as regions of skin with minimal
fat, while dermal hypoplasia appears depressed, red plaques in areas with prominent
Other non-lipomatous growths in PS
Cutaneous manifestations also include striking overgrowth anomalies, including
asymmetric hypertrophy of the face, part or all of one or both limbs (particularly
digits), the trunk, or any combination of these; often, hemihypertrophy of one side of the
body results. These abnormalities could also be the first to attract the parent’s attention
and cause considerable anxiety.Cafe au lait spots and areas of hypopigmentation or
hyperpigmentation with a linear or whorled arrangement are also hallmark skin findings
in persons with Proteus syndrome.Hypertrichosis and nail abnormalities are also seen.
Non-cutaneous manifestations in Proteus syndrome:
The most characteristic non-cutaneous findings in patients with Proteus syndrome involve skeletal overgrowth and include corporal hemihypertrophy, partial gigantism of the hands and or the feet, and skeletal anomalies such as long-bone overgrowth and scoliosis. Overgrowths of the hands, feet, or both were universal in one series of twenty four patients with Proteus syndrome. Arm or leg over growth is nearly always a constant feature of PS.
April 2014 Supplement 1 Classics and Revisits in Scientific Interdisplinary Medical Themes,
Cranio-facial abnormalities
These are fairly common and progressive and include cranial hemihyperplasia, hyperostosis of the skull or external auditory canal, craniosynostosis,
and unilateral condylar hyperplasia. Hyperostosis and unilateral condylar hyperplasia
were common, while cranial hemihyperplasia and craniosynostosis were rare.
A facial phenotype with dolichocephaly, long face, down-slanting palpebral fissures,
ptosis, low nasal bridge, wide or anteverted nares, and open mouth at rest has been
associated with mental retardation and seizures.
The other commonly associated neoplasms in Proteus syndrome
Specific tumours have been noted to occur with an increased frequency in patients with Proteus syndrome. In addition to being found subcutaneously lipomata could also infiltrate muscles and internal organs, including the heart, pancreas, spinal cord, and the pharynx. Other tumours noted to occur in several patients include ovarian cystadenomas, testicular tumours, and parotid adenomas. Other benign neoplasms, such as bronchial hamartomas and multiple meningiomas have been reported. Cystic lung disease is found in approximately 1:10 of patients with Proteus syndrome and could lead to a significant morbidity, with rapid progression, fatality could result. Renal cysts in PS have been previously described. Visceral overgrowth was reported previously, especially involving the spleen or the thymus. Internal vascular malformations of the gastrointestinal tracts, spleen, kidneys, and the testicles have been reported previously.Other occasional reports of intravesicular venous lesions associated with life threatening haematuria was previously proffered.
Other ophthalmopathies in PS
Many ocular manifestations were previously reported in patients with Proteus syndrome. The most commonly recorded of these ocular involvements included strabismus, nystagmus, and epibulbar tumours.Other ocular involvements include an over representation of a high degree of myopia, retinal pigmentary abnormalities, retinal detachment, cataracts, posterior segment hamartomas, retinal colobomas, heterochromia irides, and glaucoma.
Associated myopathies, cardiopathies and Immuno-deficiency states in PS
Other additional features in PS include asymmetric myopathies, congenital cardiac defects, malocclusions, hypodontias, and hypoplastic enamels. A congenital immunodeficiency of some sort was previously reported in a case of Proteus syndrome.
HHML is a differential of PS with much clinical consequence.
A very important differential diagnosis to be considered in these is hemihyperplasia
and multiple lipomatosis syndrome (HHML), because of the higher association with
nephroblastoma (Wilms tumours and possibly hepatoblastomas) in HHML.
The diagnosis of HHML with certainty will have implications for apposite screening of these patients for malignancies using standard screening protocols. [57]
April 2014 Supplement 1 Classics and Revisits in Scientific Interdisciplinary Medical Themes.
Early prophylactic surgical management are more appropriate interventions.in PS
The management of PS has been an inescapable but crucial medical dilemma,
especially with regards to the prolonged multiple staged orthopaedic interventions.
The rapidly progressive overgrowths are quite cosmetically disfiguring, unsightly and
probably mechanically and functionally incapacitating, seldomly or occasionally causing
compressive aerodynamically significant airway defects or neuropathies and rarely
malignant transformations. Despite several previous descriptions of early, intermediate or
delayed modified surgical interventions for these defects, overall short, medium or long
terms outcomes for these defects appear to be discouraging and unrewarding.
However, the evidence for a primordial intervention before the onset of catastrophic or
debilitating complications appears more convincing and satisfactory.
Interventions for PE in PS
The other factors of interest in the management of PS are the continual concerns with
deep vein thrombosis related thrombo-embolic phenomenology especially with regards to
its frequent occurrence peri or post operatively. The importance, strengths and the morbidity and mortality implications of these associations raised and buttressed the
argument for routine pre or perioperative anticoagulant prophylaxis and monitoring
of PS cases. However, this practice is more or less anecdotal and could occasionally be a
complex decision in the context of a sequence with a supposedly universal vascular
anomalies, real risk of bleeding especially in the association of Marriet Kaselbach
syndrome which is a potential complication of platelet consumption by the meshwork of
the vascular malformations.
Screening for Neoplastic conditions as plausible prophylactic strategy for PS
The other area worthy of adequate attention and consideration in the overall management of PS patients which were contentiously reviewed and addressed in the literature are those dealing with the most safe, cost effective and clinically useful way of detecting the tumourous events in PS early enough for appropriate interventions and outcomes to be most therauptically and prognostically rewarding. However, this decision is compounded by the fact that the list of plausible neoplasms which could complicate a case of PS are quite diverse and there appears to be no robust figures to suggest that earlier detection of these tumours or interventions by any means is actually rewarding.As such the time for active surveillance of these tumours should be weighed in favour of clinically and symptomatically suspected cases as against a routine procedure.
Genetic Counseling
Also issues concerning the genetic counseling of PS patients seem anecdotally based on
the mutational, nonmosaic lethality principle which infers a low recurrence risk for PS.
Finally, the physician investigators concerns of the most appropriate way of PS gene
mapping, identification and elaboration appears to be future challenges given its sporadic
Conclusions:
For a long time in the medical literature, issues concerning the exact diagnostic criteria of
PS in suspected cases have been topics of much debate, were controversially discussed,
inconclusive and not uniformly accepted. Although recently, these criteria were consensusly fairly well elaborated on, with some convincing recommendations derived. However, it is likely that these criteria will need to be modified from time to time as it has always been, to circumvent underdiagnosis or over diagnosis, the extensiveness and expressivity of the increasing number of overlapping syndromes make these diagnostic challenges likely, especially with further work in these areas or longer term follow up of putative PS probands and their families.
Relevance and Importance.
These editorial review discusses an unusual hitherto previously unaddressed co-existence and associations of embroyomas in PS.The diversity in its phenotypic expressivities and the variabilities in the chronology and temporality of their manifestations were reiterated.The rarity and strengths of its associated features as against its differentials especially hemihyperplasia and multiple lipomatosis syndrome (HHML) which is associated with embryomas were examined.[58] The other available literature on the other putative tentatively peculiar overlapping syndromes could be reviewed as a topic for debate in the near future.
Guest Editors: CRS [Med] Consulting Academic Clinical Editorial Experts.
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[19]-Cohen, M.M.Jr.: Understanding Proteus syndrome, unmasking the Elephant Man, and stemming elephant fever. Neurofibromatosis 1:260-280, 1988.
[20]-Cohen, M.M., Jr: Further diagnostic thoughts about the Elephant Man.Am.J.Genet.29:777-782, 1988:
(21]- Bailer, M.G.; Reidy,M.J; Wilson, W.G.Proteus syndrome versus Bannayan-Zonana syndrome: a problem in differential diagnosis .Europ.J.Pediat.148:122-125, 1988.
[22]-Samlaska,C.P.;Levin,S.W.;James,W.D.;Benson,P.M.;Walker,J.C.;Perlik,P.C.:
Proteus syndrome .Arch.Derm.125:1109-1114,1989.
[23]-Malamitsi-Puchner,A,;Dimitriadis,D.;Bartsocas,C.;Wiedemann,H.-R.:Proteus syndrome: course of a severe case.Am.J.Genet.35:283-285,1990.
[24]-Hotamisligil, G.S.; G.S.; Ertogan, F.: The Proteus syndrome: association with nephrogenic diabetes insipidus.Clin.Genet.38:139-144, 1990.
[25]-Goodship,J.;Redfearn,A.;Milligan,D.;Gardner-Medwin,D.;Burn,J.:Transmission of Proteus syndrome from father to son?Med.Genet.28:781-785.
(26)-Kruger, G.; Pelz, L.; Weidemann, H.-R.: Transmission of Proteus syndrome from mother to son? (Letter) Am.J.Med.Genet.45:117-118,1993.
[27]-Skovby,F.; Graham,J.M.; Sonne-Holm,S.; Cohen,M.M.,Jr.:
Compromise of the spinal canal in Proteus syndrome.Am.J.Med.Genet.47:656-659.
[28]-Cohen, M.M., Jr.: Proteus syndrome: clinical evidence for somatic mosaicism and selective review.Am.J.Med.Genet.47:645-652,1993.
[29]-Smeets, E.; Fryns, J.-P.; Cohen, M.M., Jr.Regional Proteus syndrome and somatic mosaicism Am.J.Med.Genet.51:29-31.
[30]-Gordon,P.L.;Wilroy,R.S.;Lasater,O.E.;Cohen,M.M.,Jr.:Neoplasms in Proteus syndrome.Am.J.Med.Genet.57:74-78,1995.
[31]-Lacombe,D,; Battin,J.: Isolated macrodactyly and Proteus syndrome. (Letter)Clin.Dysmorph.5:255-257.
(32]-Ceelen, W.; de Waele, J.; Kummen, M.; de Hemptinne, B: Non-operative management of a splenic laceration in a patient with Proteus syndrome.J.Accid.Emerg.Med.14:111-113,1997
[33]-Biesecker,L.G.;Happle,R.;Mulliken,J.B.;Weksberg,R.;Graham,J.M.,Jr.;Viljoen,D.L.;
Cohen, M.M., Jr.: Proteus syndrome: diagnostic criteria, differential diagnosis, and patient evaluation.Am.J.Med.Genet.84:389-395.
[34]-Biesecker, L.G.; Peters, K.F.;Darling, T.N.; Choyke, P.; Hill,S.; Schimke,N.;
April 2014 Supplement 1 Classics and Revisits in Scientific Interdisciplinary Medical Themes.
Cunningham,M,;Meltzer,P.;Cohen,M.M.,Jr.:Clinical differentiation between Proteus syndrome and hemihyperplasia:description of a distinct form of hemihyperplasia.Am.J.Med.Genet.79:311-318,1998.
[35]-Happle,R.: Elattoproteus syndrome: delineation of an inverse form of Proteus syndrome.Am.J.Med.Genet.84:25-28,1999.
[36]-De Becker,I.;Gajda,D.J.; Gilbert-Barness,E.;Cohen,M.M.,Jr.:Ocular manifestations in Proteus syndrome.Am.J.Med.Genet.92:350-352, 2000
[37]-Hodge,D,;Misbah,S.A.;Mueller,R.F.;Glass,E.J.;Chetcuti,P.A.J.:Proteus syndrome and immunodeficiency .Arch.Dis.Child.82:234-235,2000.
[38]-Gilbert-Barness, E.; Cohen, M.M.,Jr.;Optiz, J.M.: Multiple meningiomas, craniofacial hyperostosis and retinal abnormalities in Proteus syndrome.Am.J.Med.Genet.93:234-240.
[39]-Slavotinek, A.M.; Vacha, S.J.; Peters,K.F.; Biesecker,L.G.: Sudden death caused by pulmonary thromboembolism in Proteus syndrome.Clin.Genet.58:386-389,2000)
[40]-Cohen.M; Causes of premature death in Proteus syndrome (Editorial) Am.J.Med.Genet.101:1-3, 2001.
[41]-Biesecker,L.G.: The multifaceted challenges of Proteus syndrome.JAMA 285:2240-2243,2001.
[42]-Barker,K.;Martinez,A.;Wang,R.;Bevan,S.;Murday,V.;Shipley,J.;Houlston,R.
;Harper, J.: PTEN mutations are uncommon in Proteus syndrome.J.Med.Genet.38:480-481,2001.
[43]-Mackay, G.; Spitz, L.; McHugh,K.: Lipomatosis of the colon complicating Proteus syndrome.Arch.Dis.Child.86:265 only,2002.
[44]-Mohamedbhai,A.G.;Miyan,A.M.H.;Lacombe,D.:Neonatal Proteus syndrome?(Letter)Am.J.Med.Genet.112:228-230,2002.
[45]-Turner JT, Cohen Jr MM, Biesecker LG: Reassessment of the Proteus syndrome literature: application of diagnostic criteria to published cases. Am J Med Genet 2004; 130A:111-122.
[46]-Thiffault, I.; Schwartz, C.E.;Der Kaloustian, V.;Foulkes,W.D.: Mutation analysis of the tumor suppressor PTEN and the glypican 3 (GPC3) genes in patients diagnosed with Proteus syndrome.Am.J.Med.Genet.130A:123-127, 2004.
[47]-Brockmann, L.G.; Happle,R.;Oeffner,F;,Konig,A.: Monozygotic twins discordant for Proteus syndrome.Am.J.Med.Genet.146A:2122-2125,2008.
[48]-Smith,J.M;Kirk,E.P.E;Theodospoulos,G;Marshall,G.;Walker,J;Rogers,M;Field,M;
Brereton,J.J; Marsh, D.J.Germline mutation of the tumour suppressor PTEN in Proteus syndrome. (Letter)J.Med.Genet.39:937-940,2002.
[49]-Zhou,X;Hampel H;Thiele,H;Gorlin,R.J;Hennekam,R.C;Parisi,M;Winter R.M;Eng,C.:Association of germline mutation in the PTEN tumour suppressor gene and Proteus and Proteus-like syndromes. Lancet 358:210-211, 2001
[50]-Zhou, X.-P.;Marsh,D.J.;Hampel,H.;Mulliken,J.B,;Gimm,O.;Eng,C,:Germline and germline mosaic PTEN mutations associated with a Proteus-like syndrome of hemihypertrophy,lower limb asymmetry,arteriovenous malformations and lipomatosis.Hum.Molec.Gent.9:765-768,2000
[51]-Cohen JrMM, Neri G, Weksberg R: Klippel-Treaunay syndrome, Parkes Weber Syndrome, and Sturge-Weber syndrome: Overgrowth syndromes. New York: Oxford University Press, 2002, pp111-124.
[52]-Robin NH, Biesecker LG: Considerations for a multiaxis nomenclature system for medical genetics. Genet Med 2001; 3:290-293.
[53]-Darmstadt GL, Lane AT: Proteus syndrome.Pediatr Dermatol 1994; 11:222-226
[54]-Hotamisligil GS: Proteus syndrome and hamartoses with overgrowth.
Dysmorphol Clin Genet 1990; 4:87-107.
[55]- Tweed JV, Turner JT, Biesecker LG, Darling TN: Cutaneous manifestations of Proteus syndrome: correlations with general clinical severity. Arch Dermatol 2004; 140:947-954
[56]-Nguyen D,Turner JT,Olsen C,Biesecker LG,Darling TN:Evolution of skin lesions in Proteus syndrome.J. Am. Acad. Dermatol. 2005; 52:834-838.
[57]-De Baun MR, Brown M, Kessler L: Screening for Wilms tumor in children with high-risk congenital syndromes: considerations for an intervention trial. Med Pediatr Oncol 1996; 27:415-421.
April 2014 Supplement 1 Classics and Revisits in Scientific Interdisciplinary Medical Themes.
Cervical haemangiomas with intracerebral extensions in childhood.
Guest Editors: CRS [Med] Consulting Academic Clinical Editorial Experts.
Background and Purpose
The need for a multidisciplinary intervention in most cases in clinical medicine is further reiterated by another editorial annotation and perspective from the CRSM consulting guest editorial experts by cases of exophytic cervical haemangiomas with intracerebral extensions observed in some young children,
Editorial Annotation and perspective.
In this correspondence, unusual presentations of congenital Cervical Haemangioma with intracerebral corticomedullary extension, with acute respiratory distress, hypochromia and thrombocytopaenia in children is discussed. In such instances, early and timely interventions will have positive implications for guarding against occult Marriet-Kaselbach syndrome, high output cardio-respiratory decompensation, neurologically significant and multiple vital organic compressive-obstructive effects. In these circumstances, application of intralesional corticosteroids appears to have arrested the progressive course of these lesions, until interval extramural instrumentations were achievable.
Introduction
The descriptive epidemiology of Haemangiomas intimates that they are relatively common childhood tumours occurring in 1:10 to 1:20 infant’s .Female gender and extreme prematurity appears to be significant features of its epidemiologic associatiions.They are usually localized to the cutaneous or subcutaneous tissues. Although almost universally congenital, they are rarely fully developed at the time of birth. About 2 in 3 cases of haemangiomas are localized to the head and neck region. Most haemangiomas are present or appear during the neonatal period as soft rapidly growing solitary unifocal lesions or occasionally multifocal lesions.
Complications of Haemangiomas.
In haemangiomas rapid growth over six to nine months may occur to produce severe cosmetic disability, orificial obstructions, threatened viability of vital structures such as the eyes and multiple ulcerations. In less than 1: 10 cases, rapidly growing hemangiomas of the head and neck region could produce catastrophic complications, with aero-digestive tract obstructions especially following a sudden increase in size related to haemorrhages, causing visual, auditory, deglutinatory and vocal difficulties. Also the tumefactions could get secondarily infected through the ulceration of the overlying skins .If an arteriovenous malformative communications of a considerable capacity with a haemodynamic significance or consequence develops an intractable high output congestive cardiac failure could follow.
April 2014 Supplement 1 Classics and Revisits in Scientific Interdisciplinary Medical Themes.
Other deleterious consequences of Haemangiomas.
There are other several complications of great concern such as the Kasabach-Merrit syndrome. In this complication, an inordinate bleeding diathesis and purpura may result from platelet consumption, DIC, microangiopathic haemolytic anaemia.
Previously, internal organs have been reported to be occasionally involved, in
haemangiomas, however, intracerebral involvement in haemangiomas are rather an
unusual and rare phenomenon.
Thrombocytopenia, thrombocytosis, respiratory distress and occult bleeding diathesis from coagulapathy and thrombocytopaenia and severe recurrent iron deficiency anaemia appears to be real major concerns with significant lesions.
Associated naevus may initially be considered to be inconsequential but could occasionally pose cosmetic and aesthetic difficulties with the passage of time.
The natural history of haemangiomas and its simulating lymphogiomas
The natural history of haemangiomas peculiarly involves an initial proliferative growth
phase that lasts about half to one year and an involutional phase which is associated with
a slow regression of the haemagiomas, with about one half of the lesions resolving by sixty months and about 9/10th resolving by the age of ten years. A very important differential to consider in this case is a lymphangiomatous lymphatic malformations which could be unifocal or multifocal and commonly occurs in the facio-cervical region, axilla or thoracic, they are similar to haemangiomas in that they appear in infancy and childhood usually before thirty six months. However, they differ from them by the lack of spontaneous involution. They are equally obstructive and haemmorragic tendencies.
These lesions are quite extensive and diffuse making a stage evaluation for the exact
determination of the extent of the lesion and appropriate stage dissections imperative for
an utmost outcome.
Management
Intralesional steroids appeared to have arrested the progression of these lesions in some instances.In occasional circumstances however, management could be expectant, given that spontaneous resolutions has been known to occur. By the age of ten years 9 out of 10 cases would have resolved completely.
Systemic steroid applications would have been indicated otherwise if there were concerns with ocular integrity or ongoing concerns with airway obstructions, difficulties with feeding or defecation or in the Kasabach-Merrit syndrome
Although some recalcitrant cases may not be very steroid or radio therapeutically responsive. Administration of interferons appears to be very useful in catastrophic situations. Also Laser coagulations or sclerotherapy could be useful in haemangiomas but not usually in Lymphangiomas..Other agents like OTK 145 have been employed in selected cases with significant beneficial effects. In Kasabach-Merritt syndrome, treatment is supportive, platelets, cryoprecipitate and fresh frozen plasma, heparin and antifibrinolytic agents such as aminocaproic acid and traxenamic acid may be used.
In other instances, life threatening compressive effects such as severe respiratory distress or rising intracranial pressure with cardiopulmonary instability and intrabdominal hypertensive events has made urgent extramural instrumentations imperative.
Guest Editors: CRS [Med] Consulting Academic Clinical Editorial Experts.
April 2014 Supplement 1 Classics and Revisits in Scientific Haematology.
Fanconi anaemias in Childhood.
Guest Editors: CRS [Med] Consulting Academic Clinical Editorial Experts.
Background and Context:
Fanconi Anaemia-FA is a severe debilitating disease associated with a compromised life expectancy. Specific review on FA is crucial. This expert guest editorial review article by the CRSM academic clinical consulting editors is based on observational reports on a spectrum of compatible childhood symptomatic characteristic Fanconi akylating aplastic anaemias.
In the various subsets, overall aggregate annotations suggest that growth failures were universal, whereas dysmorphologies such as microcephaly, polydactyly, hypopigmented dermatoses were common associations.Rhizomelic short limbed dwarfism, hypodontias and caput quadratum were occasionally encounted defects. Bleeding diathesis, febrile neutropaenias, and pallor were predominantly featured phenomena.
Another notable feature of interest is that in individual cases, recurrent symptomatologies were invariable and somewhat predictable.
Multiple blood transfusions, antimicrobials, steroids. , testosterone, acyclovir and fluconazole were the most commonly applied interventions which lead to a partial remission in some cases.. Doubtful malignant transformations and acute catstrophic bone marrow failures were mutually exclusive events. Unprecedented demise was a notable phenomenon. Occasionally, recovery from FA could be spontaneous.
Case Definitions..
Fanconi aplastic anaemia is an inherited autosomal recessively transmitted, genomically determined instability syndrome, associated with multiple developmental structural and functional abnormalities, progressive bone marrow failure, leading to pancytopaenia and the requirement for haematopoietic bone marrow stem cell transplantation or increased morbidity or mortality as a result of defective haemopoesis and malignant predisposition to earlier onset acute myeloid leukaemias and latter onset solid stromal tumours. [1, 2]In Fanconi anaemia, cellular chromosomal instability, breakage and hypersensitivity to bifunctional DNA cross-linking agents especially mitomycin C is usually demonstrated, which usually forms one of the basis for its diagnostic criteria. [3]
Complementation somatic-cell hybridization genomic analysis in FA
For a long time, the relationship between congenital malformations, bone marrow
failures, cancers, chromosomal breakages and their derived complementation groups in
April 2014 Supplement 1 Classics and Revisits in Scientific Haematology
Fanconi anaemia syndromes were poorly understood. [4 ]
The principal pathophysiology in Fanconi anaemias, the most common of the familial congenital constitutional aplastic anaemias have been fairly well elucidated through major advances in complementation somatic-cell hybridization genomic analysis of FA cell lines which demonstrated and provided evidence for alphabetically designated complementation groups (FA-A, FA- B, FA-C, FA-D1, FA-D2, FA-E, FA-F, FA-G, FA-I, FA-J, FA-L) with implications for abnormal haemopoiesis and apoptosis in their mutant states especially for the FA-C [2] in addition to linkage to a specific pathognomonically genomic disease entity.
The distribution of the complementation subgroup subtypes.
The distribution of the complementation subgroup subtypes of FA is geographically distinct, in some regions the FA-A was the most frequently represented at 59%, where as in the other, FA-C was the predominantly featured subtype at 67%.
This distributional in homogeneity could have implications for mutation-screening
strategies in continental FA patients. [5]
The extensive genetic heterogeneity in FA and its influence on linkage analysis.
The implications of this extensive genetic heterogeneity for classical linkage analysis were further discussed in previous investigations. [6, 7, 8] however, a good part of its pathophysiology remains elusive.
Although it is a genetically inherited form of bone marrow failure syndrome, its presentation may not necessarily be congenital [9, 10], until the abnormal immune
responses implicated in its pathogenesis is triggered by genomic instability or by
environmental exposures to chemicals, drugs or viral infections or perhaps by
endogenously generated altered bone marrow cells, which then heralds its more florid
The molecular and geographic epidemiology of FA.
The pathobiology, molecular and geographical epidemiology with the relevant therapeutic options in Fanconi aplastic anaemia syndromes have been previously investigated, described, replicated and revisited in several settings [11,12,13,14] Previous occasional case studies and series suggested hospital and community based high incidence rates, however overall the disease prevalence was relatively low, such that longitudinal single city epidemiological studies were not logistically feasible, however more recent investigations achieved higher sample sizes but most of those were in adults.[15]Overall, FA is a relatively uncommon disease with an estimated prevalence of 1 to 5 per million and an approximated carrier frequency of 1 in 200 to 1 in 300 in most population studies [16] Although its diagnosis is probably underestimated, because its haematological features may be moderately expressed or they could present latter.
The influence of the Founder effect on the incidence of FA
Of growing interest in the elucidation of the drive behind the unusual preponderance of FA in some ethnic groups compared to others is the concept of founder effect associated with genetic and reproductive isolation. This phenomenon implies an increased risk of inheriting rare genetic disorders such as FA following the development of a high frequency of a mutant allele introduced into communities which are genetically and reproductively isolated and therefore consanguineously and endogamously married .
The carrier frequency in this subset was estimated to be 1 in 90 to 1 in 92[17,18]
Other previously described epitomes of the phenomenon of founder effect were demonstrated for a subset , where the carrier frequency was estimated to be about 1 in 77 [19,20]
Further recent investigations demonstrated the inordinately clustered overrepresentation
of the incidence of FA in the subjects of particular ancestry from heterogeneously
distinct descent, abode and ethnicity. However, the figures from those of subsets
with homogenous ancestries mostly of the complementation group A subset were
astonishingly extreme, probably the most globally at 1 in 64 to 1 in 70[21, 22]
Haplotype linkage disequilibrium as a causality factor in FA.
Other prominent clusters were demonstrated elsewhere, where the populace in
genetic isolation revealed clinical characteristics of the patients showing both intra-and
interfamilial heterogeneity suggestive of haplotype linkage disequilibrium for markers
flanking the FAA gene. This hypothesis may have implications for positional
cloning of this gene through haplotype disequilibrium mapping. [23]
The case for further investigations in FA
In most circumstances however, accurate estimates of childhood characteristic
Aplastic anaemia (Fanconi subset) Fanconi anaemia, its clinical profile, peculiarities, heterogeneity, complexities and the intricacies in its diagnosis and the feasible therapeutic options have not been previously analyzed, revisited or completely understood.
Reliable inference or reference review on Characteristic Fanconi akylating aplastic anaemia syndromes are crucial for interventional planning, hypothesis generation, research directions and focused hospital or population based interventions.
It could also offer some contribution to a better definition of its natural history. Furthermore, this annotation offers the opportunity to highlight and briefly discuss some of the commonly associated structural and functional anomalies in FA as was previously proposed [24] , given the relevance of a more lucid definition of these commonly associated anomalies in expediently advancing a rational prognostic scoring index which predicts the likelihood of progression to catastrophic bone marrow failures or malignant transformations as was determined previously[2,25,26] This editorial review article is a step to describe and characterize some of these aspects.
Case definitions, Observations and follow up of compatible FA cases.
On observation and follow ups, suspected cases with the diagnosis of compatible Characteristic Fanconi akylating aplastic anaemia syndromes would have achieved the clinical, radiological and pathological inclusion criteria of FA for descriptors, clinical features, predominant symptomatologies, laboratory features as was previously described by Camitta: [27]
With severe disease defined as the presence of two of three blood component counts
criteria of an absolute leukocyte count of<0.5×10 (9), platelet count of <20×10 (9) and a
reticulocyte count of < 1%.Extreme neutropenia (<0.2×10 9/L) defined very severe
aplastic anaemia, the other cases could be described as moderately severe aplastic
anaemia.The bone marrow biopsy had to be compatible with the diagnosis of aplastic
anaemia.Cases had to be analyzed for demographics, historical and medical information, especially with relevance to clinical features, recent and previous drug use and related infective episodes.
Clinico-Epidemiological diagnosis in FA
Further basic epidemiologic features of Fanconi aplastic anaemia bone marrow failure syndromes could be achieved by characterizing cases in terms of demographic variables, age, sex, medical history, clinically significant manifestation, proof of diagnosis, and prognostic criteria. Associated dysmorphologies were evaluated, studied, validated, analyzed and reported in a standardized manner using standard normograms from the dysmorphology databases.
Clinical features and associations of FA
Overall, presentations in FA were associated with the male factor, consanguinity, latter presentation,non-familial incidence,infective episodes, unusual cranio-facial features or inconsistent physical characteristics Bleeding diathesis especially with epistaxis was a common but less florid mode of presentation, where as massive haemoptysis.frank pulmonary haemorrhages and haematemesis,where less frequent, but clinically more alarming and deleterious.Lost cases were mostly related to massive haemorrhages or overwhelming infective events related to febrile neutropaenias.
Outcome of observed FA cases..
The presence of at least one associated anomalous dysmorphological defect is quite usual.Rhizomelic short limbed dwarfisms with delicate statute, microcephaly
pre-axial and post axial polydactyl, caput quadratum, absent radii,
hypodontias and abnormal gingival morphology were the most predominant dysmorphological phenomena. Abnormal pigmentation of the hair, the skin and the mucous membranes especially the lips were the most common intergumentary defect noted.
April 2014 Supplement 1 Classics and Revisits in Scientific Haematology
The diagnostic and the therapeutic aspects of FA.
For the suspicion of Characteristic Fanconi anaemia to be evoked, the blood film and bone marrow morphologies has to be compatible with that of bone marrow failure syndromes.
Undertaking radiological evaluations would be expedient to exclude respiratory tract infections or complicated organizing pneumonias or features suggestive of pulmonary infarctions or haemorrhage, it may equally exclude or suggest erythrophagocytic lympho-histiocytosis-X rather than FA as the plausible diagnosis to be pursued.
X-ray radiological surveys could be worthwhile from the context of suggesting absent radii. Or other more covert musculoskeletal defects.
Transthoracic and abdominal ultrasonic echographies or tomograms could be useful to exclude the possibility of other linked congenital visceral abnormalities.
Routine cultures and biochemical studies would have positive prophylactic, therapeutic and prognostic implications for those with persistent febrile episodes despite applications of broad spectrum and specific antimicrobial. Most patients would be hospitalized at a point, and most of the hospitalizations will be associated with whole blood transfusions, and most cases could be managed conservatively at the first instance.
The historical perspective of FA.
Fanconi aplastic anaemia syndrome, a form of Constitutional aplastic anaemia was
earlier described by Erlich towards the end of the 19th century. [28]
Constitutional aplastic anaemia could be associated with congenital dysmorphology
(Fanconi subset) or it may not be associated with it. In 1919 Smith reported aplastic
anaemia in a six year old boy associated with marked nuchal, genital, umbilical and areolar skin pigmentation. [29] Most cases of constitutional aplastic anaemias were usually autosomal-recessively transmitted, although an occasional case report suggested the possibility of an autosomal familial dominant inheritance pattern [30]
Fanconi in 1927 described three brothers aged five, six and seven with hyperchromic
anaemia, leucopaenia, thrombocytopaenia, haemmorragic diathesis, microcephaly, testicular hypoplasia, convergent strabismus, exaggerated deep tendon reflexes and generalized brown, melanin like pigmentation of the skin but not of the buccal mucosa. [31]
The Haemopoetic stem cell line defect in FA and its therapeutic implications.
Fanconi anaemia bone marrow syndromes usually arise as a result of damage to precursor cells so that they cannot supply an adequate quantity of haemopoetic cells .This damage could also be at the bone marrow stromal level (i.e.the bone marrow microenvironment) which is now regarded to be equally important in the pathogenesis of aplastic anaemia.The spectrum of clinical and laboratory features are implicit on the stage of the haemopoiesis where the maturational sequence was disrupted. Earlier damage will result in uniform pancytopaenia, whereas latter disruptions after haemopoetic progenitor cells differentiation will result in predominantly red blood cell aplasia, agranulocytosis or thrombocytopenia. These differential interruptions may have implications for variabilties in its symptomatologies, which if predictably consistent could be exploited for a
more focused and targeted blood component and adjunctive supportive therapeutic interventions with overall positive outcome, toxicity and costs implications.
April 2014 Supplement 1 Classics and Revisits in Scientific Haematology
Irradiation and toxicity induced perturbations implicated as causality factors in FA
The association of the onset of the symptomatology of Fanconi aplastic anaemia with several putative inciting agents such as viral infections, drugs, toxic agents, irradiation,
autoimmune diseases and idiopathic- cryptogenic factors, intimated the probability of
several mechanisms for the non-specific triggers and amplifications of the disease
processes. [32] The effect of irradiation on the cells of characteristic Fanconi akylating aplastic anaemias were controversially discussed, although the results suggested that
there were no increases in radio-sensitivity to all cell types from characteristic Fanconis
akylating aplastic anaemia patients, however an apparent increase in chromosomal
radiosensitivity may be seen in the lymphocytes from an occasional case. [33]
Defects of Haemopoetic stem cell line and immonopathology suggested as FA causality factors.
Previous investigations proposed several hypotheses for the pathophysiology of Fanconi aplastic anaemias, which were derived from the results obtained after several systematic laboratory investigations. These results suggested the convincing evidence of the efficacies of several therapeutic options which linked it to some plausible aetiological mechanisms. The successful use of bone marrow transplantation to cure Fanconi aplastic anaemia implicated a stem cell deficiency, where as the significant response to immunomodulant therapies pointed more to an immune mechanism of haematopoietic
Clonality inconsistencies suggested as a possible causality factor for
FA and other associated diseases haematological dyscrasias.
The relationship of the Fanconi aplastic anaemia syndrome to other haematological dyscrasias associated with clonality has been suggested, such haemato-pathologies include large granular lymphocytosis, and single haematopoietic lineage deficiency states such as agranulocytosis, pure red cell aplasia and amegakaryocytic thrombocytopenia. [34] Other closely related entities to FA are Dyskeratosis congenita and other forms of Idiopathic cryptogenic unclassifiable forms of constitutional aplastic anaemias, like FA they are associated with a high incidence of haemopoetic clonal disorders, single-or multiple lineage cytopaenias, severe aplastic anaemia, myelodysplasia, and malignant transformation, identification of these associated mutant genes in such pathologies will have implications for elucidating the exact pathophysiology involved, and proffer relevance for deducing apposite novel therapeutic options. [35, 36, 37]
Chromosomal breakage points on peripheral lymphocytes may suggest the diagnosis of FA.
The diagnosis of Fanconi aplastic anaemias could be inferred from chromosomal breaks observed in peripheral blood lymphocytes or dermal fibroblasts exposed to mitomycin in the appropriate clinical and haemato-pathological setting.Fanconi anaemia cells will normally show excessive chromosomal breaks. [38, 39] In this way some young adults have been diagnosed to have Fanconi anaemia.
Myodysplastic syndromes and paroxysmal nocturnal haemoglobinuria as FA differentials
Some less common congenital marrow failures syndromes, without specific markers,
could be more difficult to exclude.Myelodysplastic syndromes could be ruled out by
appropriate marrow haemato-pathologic and cytogenetic analysis.The distinction
between Fanconi anaemia, Myelodysplasia and other forms of bone marrow aplastic
anaemia is critical, because therapeutic options differ. [40, 41] Also, there are considerable overlaps between the pathogenesis, laboratory features and therapeutic options for the syndromes of paroxysmal nocturnal haemoglobinuria and Fanconi aplastic anaemia because many patients with FA bone marrow failure syndromes
have an increased population of abnormal cells [42]
Although FA is congenitally acquired, its onset is delayed till early childhood.
Interestingly, the onset of the aplastic anaemia is usually between the ages of four and seven years in boys and six and ten years in girls, however it may occur at an earlier age.
Results proffered by one investigation noted a male preponderance and a younger age at onset [43]
April 2014 Supplement 1 Classics and Revisits in Scientific Haematology
The cryptic onset of the clinical features of FA in some instances and its diagnostic implications.
The onset of FA could be cryptic with pallor, skin bruises and petechiae.Easy fatigability
and anorexia then follows, with progression of the disease, bleeding could occur from
almost any site and a sore throat with ulceration of the mucosa due to leucopenia ensues.
Physical examination is more informative for the associated defects, the usual
pallor and occasionally for the related cutaneous bleeding diathesis. Also FA could
present in a more occult way as bone marrow hypoplasia or aplasia following further bone marrow studies of an incidentally or co-incidentally discovered abnormal blood film
morphology. Multiple sites aspirates and a trephine will be relevant for making a more
substantive diagnosis in indeterminate cases.
The therapeutic aspects of FA.
Whole blood, blood component therapy with platelets and chemoprophylaxis will be
required until there is a compatible bone marrow donor.Allogeneic bone marrow stem
cell transplant in children is a very rewarding intervention. However, this may not always
be achievable in all cases due to lack of a suitable donor. In such cases
immunomodulation and antithymocyte globulin therapy could be considered in the interim. [44]However, there are real concerns of longer term complications of hypocellular myelodysplasia compounding its diagnosis and transformation to acute leukaemias in longer term survivors even after bone marrow transplantation. [45]
The variations in the clinical course of Fanconi anaemia and the response to
immunosuppressive treatment could be explained by variations in the balance between
its primary defect and the secondary immune responsiveness, the co-involvement of other
accessory cells in the primary disease, the relative time course of the two components and
the efficiency of the repair mechanisms. [32]
April 2014 Supplement 1 Classics and Revisits in Scientific Haematology
A rational and conservative approach to circumvent some complexities associated with some optimal interventions for FA
The inherent complexities of these interventions for FA imply that they will not always be rapidly achievable, in most instances or circumstances.
However, since previous investigations intimated that symptomatologies appear when the stem cell and progenitor cell populations are less than one percent, given the inherent high marrow reserve potential for haemopoiesis.There fore, this positive feature of an immense marrow reserve capacity for haemopoiesis could be exploited in more selective , specific and focused haematopoietic cell line conservative supportive interventions with colony stimulating factors and/or defined blood component therapies, which offers initial interim tentative options for well selected cases in restricted circumstances, while arrangements for an appropriate compatible umbilical or bone marrow stem cell donor are being made, however given its negative cost and systemic toxicity implications a more rational, directed, focused and informed use of these options will be imperative for an utmost outcome. [46]
Predictive scoring systems to determine progression to catastrophic bone marrow failures or malignant transformations in FA
Previous investigations intimated that haematological indexes after interventions predict
prognosis [47].This may have implications for major decisions concerning bone marrow
stem cell transplantations. [48]A scoring system proposed previously, to predict the
likelihood of the onset of an early catastrophic bone marrow failure or progression to latter acute myelogenous leukaemias and stromal solid organ tumours which are it’s commonly associated mutually exclusive or rivalry phenomena, speculates that abnormal or absent radii and a five-item congenital abnormality score were hypothetically significant predictor of early symptomatic catastrophic bone marrow failures, [25] where as those with latter onset less overt bone marrow failures syndromes are likely to survive longer enough to progress to malignancies such as acute leukaemias mainly, acute myelegenous leukaemias in about nine of every ten cases,anaemic myelodysplastic syndromes in one of every other fifteen cases, solid tumours in one of every twenty cases which were mainly gastrointestinal and gynaecological stromal tumours and hepatic tumours both hepatocellular carcinomas or hepatic adenomas in one of every thirty three cases, although the age at onset of the malignancies were considerably much lower compared to the general population ..In a quarter of these cases the malignant features preceded the diagnosis of Fanconi aplastic anaemia syndrome. [26]
Epidemiologic figures for the haematological and oncological complications of FA.
On the basis of these predictors, a previous series demonstrated the cumulative incidence of bone marrow failures by the age of ten years to be quite heterogeneous from one of every eight cases to about four of every five cases, according to associated risk scores, whereas the ratio of the statistically significant, observed versus expected malignancies were demonstrated for all cancers, all solid tumors and acute myelogenous leukemia’s. Other significantly relevant observed versus expected ratio elevations were noted in oesophageal, vulval, head and neck, mammary gland and cerebral neoplastic conditions respectively. [2]However, the competing events of a possible occasionally synchronous or usually metachronous acute myelogenous or invasive stromal solid tumours could be completely overwhelmed by overtly acute symptomatic catastrophic bone marrow failures syndromes, which indeed validated the diagnosis of Fanconi anaemias in most instances
The five- point congenital dysmorphology score for predicting catastrophic BMF in FA.
The five-item congenital abnormality score with microcephaly, absent or abnormal radii,
polydactyly, abnormal pigmentary skin defects and growth failures predictive of
catastrophic bone marrow failures has been determined to be fairly well reproducible by several investigations compared to that suggested for malignant transformations.
This is unlike the result reported by one investigation, where there were progressions to acute myelomonocytic leukemias [43] where as Leukaemias contributed immensely to the mortalitities in one study [14], malignant transformations to a miscellaneous subset of
stromal solid tumours was the implicated culprit in another investigation [2]
Blood Transfusions as an inescapable intervention in FA and its major concerns
Blood transfusions are almost always a necessary adjunct to the therapy of bone marrow failures, and its indication in Fanconi aplastic anaemias syndromes is a spectra as indicated by the reports from several series, however the major concern with multiple blood transfusions are the hazards related to iron overload and transfusion related infectious phenomena. The other major concern of multiple pretransplant platelets transfusions is, its negative impact on reducing the chances of allogeneic umbilical cord and bone marrow stem cells transplants from an HLA genotypically identical sibling, which is the mainstay of a substantive definitive intervention for the Fanconis anaemias related pancytopaenias and the most predictive prophylactic option for new onset leukaemic evolution.[49]
Immuno-modulation as a therapeutic option in FA
In such instances or circumstances where multiple blood transfusions are contraindicated, non-beneficial or hazardous immunomodulators and antithymocyte globulin could be an interim therapeutic option. [44]However, there are still further concerns of longer term complications of hypocellular myelodysplasia compounding this diagnosis, transformation to acute leukaemias in longer term survivors especially following bone marrow transplantation. [45]
Other pragmatic diagnostic options for FA when chromosomal studies are unachievable or inconclusive.
In FA most cases will show a predominance of either a severe to profound defect in granulopoiesis or thrombopoeisis with an erythropoetic insufficiency of moderately severe to very severe degree being fairly constant among these subsets.
Although traditionally, a significant contribution to the diagnosis of Fanconis anaemias have been the description of consistent chromosomal abnormalities in a high proportion of cases,however,chromosomal studies assist in achieving the diagnosis of Characteristic Fanconi akylating aplastic anaemia syndromes with some restricted degree of certainty, given the commonly encountered inconsistencies appearing in a high proportion of FA cases.
The exploitation of the associated dysmorphologies in FA for achieving a therapeutically useful diagnosis in restricted, equivocal or ambiguous diagnostic circumstances.
However, review of extant and modern literature intimates that the diagnosis of Fanconi anaemia was occasionally achieved on the basis of compatible clinical and
haemato-pathological features in addition to its associated congenital malformations. Commonly associated congenital abnormalities noted in characteristic Fanconi akylating aplastic anaemias include a variety of skeletal abnormalities (especially involving the thumb), absent or abnormal radii, short stature, abnormal skin pigmentation, microcephaly, triangular shaped face, micrognathia, adrenal abnormalities.
The inconsistencies of chromosomal studies lends an argument for other supporting diagnostic armamentarium for FA
In some cases of Fanconi characteristic akylating aplastic anaemias, associated with bone
marrow syndromes, the chromosomal analyses will not demonstrate a high number of
chromosomal breaks, chromatid exchanges and endoreduplications, but could be
essentially normal and stable with unperturbed structural integrity which would not fit in
entirely with the diagnosis of Fanconi anaemia as was proposed by Swift and Hirschhorn [50] and Bloom et al [51],where as in other cases these chromosomal inconsistencies could be demonstrated without any obvious associated skeletal or organic dysmorphologies or malformations, in a few cases, eloquent associated malformations were the principally overwhelming features noted.
Exceptionally, the presentations of FA were typical and the diagnoses were made on the basis of consistent clinical, haemato-pathological, radiological and typically associated anomalies and by excluding its differentials.
Revisiting the dysmorphological associations of FA to butress its diagnostic use for FA
A recap of the classically associated dysmorphological features and
anomalies in this syndrome will be expedient to demonstrate the validity of the diagnosis
of compatible symptomatic characteristic Fanconi akylating aplastic anaemias in those
instances when the possibility of this diagnosis was evoked .
In most reported series abnormal pigmentary lesions were the most consistent
abnormality occurring more regularly in certain areas such as the axillae, groins,
umbilicus, areola and nuchal areas, although any part of the body could be affected.
The pigmentation may appear before or after the haematological abnormalities have manifested. An early detection of these pigmentary skin defects could offer timely suggestive clues for the diagnosis of FA with positive implications on generic and specific interventions with overall good outcome;however its presence is not invariable in all FA cases. [52] .
Other relevant associated dysmorphologies in FA.
The other commonly associated skeletal anomalies observed in FA involved the thumbs, such as hypoplasia of the phalanges, complete absence of one or both thumbs, supernumerary thumbs, atrophy of the thenar eminence, immobility of one or both joints of the thumb, a double distal phalanx and thumbs attached to the hand only by soft tissues. Other skeletal abnormalities reported occurs frequently enough to be considered an associated feature of the syndrome, viz.complete absence of one or both radii.
The other incidental or co incidental skeletal abnormalities which were previously described skeletal inconsistencies include a saddle nose deformity, spinal alterations, metaphyseal striation syndromes .The incidence of abnormalities of the renal tract in Fanconis anaemia is over 1 in 4 .The most common anomalies are structural renal defects such as absence of one kidney, horseshoe kidneys, ectopy, hydronephrosis, and impaired excretion by one or both kidneys. Cardiovascular anomalies occur but are not nearly as common as renal anomalies. They include Patent ductus areteriosus, and marked arteroma at an early age. Other associations include abnormal ears and hearing difficulties.
April 2014 Supplement 1 Classics and Revisits in Scientific Haematology.
On the average, overall intelligence is usually adequate to make a realistic educational and career development planning achievable. Many other miscellaneous anomalies have been described, the most common being hypogonadism,[43]Failure to thrive and growth retardation are universal findings in children with Fanconi aplastic anaemia syndromes. The Short statures in these patients are usually proportionate. micropthalmia, microstomia, strabismus and hypereflexia were not noted in this series. Other previous occasional case reports have proposed other very uncommonly encountered developmental anomalous associations.
The sporadic occurrence of VACTERRL syndrome in FA subsets.
Although most VACTERRL associations occur sporadically, the associated anomalies in VACTERRL syndrome and FA are so overlapping especially in the presence of a radial ray defect that, a systematic cytogenetic diagnostic tests of FA should be pursued in all cases of VACTERRL, because in patients with such malformations an early diagnosis of Fanconi anaemia could offer positive implications for genetic counseling and specific interventions. [53] In the absence of these associated dysmorphologies, it would be difficult to propose the diagnosis of Fanconi anaemias in most instances.
Idiopathic cryptogenic aplastic anaemia as a differentials of FA
A diagnosis of Idiopathic Cryptogenic aplastic anaemia could however be suggested, in that case, chromosomal studies with Mitomycin as suggested previously would be relevant to suggest these diagnoses with some degree of certainty. [54] and exclude or include the idiopathic cryptogenic groups.
Plausible therapeutic interventions for FA
In most cases of FA, although an adverse implication of the three haemopoetic cell lines is universal, however in any one instance and case, the extent and severity of this implication for each haemopoetic cell line is relatively quite variable.
These differential haemopoetic cell lineage affectations will have positive cost and toxicity implications for a defined colony stimulating factor or blood component therapy application, which could be selective, focused and directed to the most adversely affected haemopoetic cell line with more frequent and consequential symptomatologies.
Generally, children with Fanconi anaemia could be managed on out patient basis,
however, inpatient care could be warranted occasionally for the management of
complications of bone marrow failures such as bleeding diathesis or severe infections.
Whole blood or blood component therapies and transfusions were occasionally achieved
on outpatient basis. Focused evaluations of blood counts and film morphologies are recommended at three-monthly intervals or more often as needed or more frequently if warranted as an out patient procedure.Haemopoetic stem cell transplant is currently an inpatient procedure.
Traditional and recent evidence proposed a combination of androgens and corticosteroids as an initial feasible and achievable treatment options in all forms of Characteristic Fanconi akylating aplastic anaemia syndromes. [9]
The occasional shortcomings of the therapeutic interventions for FA
The shortcomings of frequent relapses in a quarter to half instances and steroid related side effects are known major concerns, hence this treatment modality though sustainable could not meet the criteria for a specific, satisfactory or definitive therapeutic option.
Occasionally, results from some investigations were not very positive, the response rate was thought to be generally unsatisfactory due to several contributory factors leading to higher mortality figures and inexorable progression to exigent bone marrow transplantation, which remains an important option for patients with Fanconi Aplastic anaemia syndromes.[14]
The promising outcome of other therapeutic interventions in FA
However a previous investigation demonstrated a sustainable beneficial effect of androgens in constitutional aplastic anaemia as a group. [55]
In childhood Characteristic Fanconi akylating aplastic bone marrow failure syndromes, immunomodulation with anti-thymocyte globulin and cyclosporine could be considered as an interim interventional option, until bone marrow stem cell transplantation from a human leucocyte antigen compatible sibling, which is the main stay of management in all severe cases could be achieved .A longer term more certain amelioration of symptomatologies with improved quality of life was described for this intervention by several authors [27]
Therapeutic concerns with Immunomodulation.
Favourable prognostic indicators for immunomodulation therapy in FA are a younger age and a short interval from diagnosis. However, the shortcomings of immunomodulation therapy with an enhanced risk of developing clonal bone marrow diseases such as leukemia or myelodysplasia continue to be major concerns. In the presence of an empty marrow, pancytopaenia and transfusion dependence, the severity of the disease is based on polymorphnuclear counts. However with the use of ATG/cyclosporine this may no longer be the case. Determination of intracellular interferon gamma in blood samples of FA cases may correlate with responsiveness to immunomodulation therapy and may suggest those patients who are likely to relapse.[56] Also, the quantitative compromise in the white blood cell counts is commonly associated with important qualitative consequences as depicted by the shorter telomere diameter of the polymorphnuclear cells in this pathology further predisposing them to multipathogenic invasive infections
.[57] and posing further diagnostic, prognostic and therapeutic difficulties.
Malignant transformations related to prolonged immuno-modulations.
Reasonably lengthy investigations recorded a death rate of about sixty eight percent, with eight percent of the deaths attributed to acute leukaemic events.
However, the mean age at the death of patients, the time from presentation to death,
and the duration of the studies were about ten years, two and half years and eleven years respectively as proffered by one investigation in a group of FA children living between the tropics on cancer and capricorn [14]In most series, the presence of more than one cell line symptomatology was associated with an increased morbidity and mortality.
The relative mortality impact of the features and complications of FA.
Bleeding diathesis and overwhelming septicaemias could be associated with mortality more than erythrocytopaenias.Aleukaemic prodromes of childhood acute lymphoblastic leukaemias should be searched for in all suspected cases. The positive outcome implication of searching for this association was reiterated by previous investigations. [58]
Surveillance programme is a cost effective and crucial interventional approach for FA.
One of the major concerns in the follow up of children with FA is the early detection of
malignancies through structured surveillance programmes, because children with
autosomally dominant Fanconi anaemias have one of the highest predictable risks for
developing catastrophic bone marrow failure syndromes with subsequent transformation
to acute myelogenous leukaemia. [59]
Relative and absolute indications for hospitalizations in FA
In FA, hospitalizations will be imperative for the treatment of malignant transformations
In aplastic anaemia, with pharmacotherapeutic interventions, the application of blood products adjuncts, and bone marrow stem cells transplantations, the putative life expectancy could be prolonged beyond the projected median of thirty year or its environ..
The prophylactic interventional approach in the management of FA.
The earlier gloomy prognosis in Fanconi anaemia has been remarkably improved by cancer screening and preventive strategies, aimed to identify early malignancies and apply appropriate oncological therapeutic interventions, which supposedly will reduce the cancer related mortality events. Although major interventions are usually required in the definitive management of FA, however, basic health seeking habits such as, eschewing behaviours and activities with much risk of injuries and bleeding, as well as maintenance of optimal hygienic standards to reduce chances of acquiring infective events, reiterating the need to adhere to relevant pharmacotherapies, transfusions, and other interventions, aimed at cancer screening such as bone marrow, oropharangeal, and
gynaecological examinations and preventions, in addition to keeping away from smoking or excessive alcohol ingestion have been inferred to be rewarding.
Screening for FA in children with malignant conditions with no known risk factors.
Also children and adolescents who present with tumours that
are characteristic of Fanconi aplastic anaemia syndrome but who are not having the usual
risk factors associated with those tumours need to be screened for Fanconi aplastic
anaemias, such tumours include acute myeloid laeukaemias, tumours,of the brain oropharynx, esophagus, vulva, brain, skin (non-melanomatous, cervix, breast, kidney, lung, liver (adenomas and hepatomas), lymph nodes (lymphoma),stomach, colon, osteogenic sarcomas, retinoblastomas, Wilms tumours and myelodysplastic
syndromes related malignancies.
Genetic Counseling
Nuclear and extended familial genetic counseling aimed at providing apposite genetic counseling to parents, caregivers, and other carriers or potential carriers with regard to the risk of recurrence and discussion of phenotypic variability within a family will be feasible deterrent options. In families, in which a mutation has been identified in a proband or through carrier screening, invitro fertilization and preimplantation genetic diagnosis could be proffered and management options suggested to the parents for an informed decision. A founder effect or an unduly high carrier frequency will also merit similar interventions.In families with affected probands, cord blood may be saved for future use as a source of hematopoietic stem cells at the birth of a sibling. Assisted conception and preimplantation genetic diagnosis could be used to identify a fetus that is a human leukocyte adhesion (HLA) compatible sibling, who does not have Fanconi anaemia.
The descriptive epidemiology of malignancies in FA subsets.
The incidences of acute myeloid leukaemias and certain solid tumours are
greatly increased in homozygotes FA syndromes. [60]
The issues concerning the associations and predispositions to malignancies in children
who are heterozygous asymptomatic carriers of FA mutations and were inferred to be at
an increased risk of developing Acute Myeloid Leukaemia from environmenental
carcinogens were contentiously discussed, and malignant transformations were raised as possibilities in these subsets by previous investigations
The initial study on this debate by Swift et al analysed one hundred and two deaths in the relatives of eight FA families and found a higher rate of leukaemia, gastric, colorectal and tongue cancers [61] but this was not confirmed by latter investigations by the same authors when the study sample was expanded to include twenty five families no overall or specific excess of malignancies could be demonstrated, in these subsets, indeed there were fewer leukaemic events than expected [62]
A separate study of one hundred and twenty five relatives in nine FA families also failed to show any statistically significant or superior difference between these subsets. [63]
FANCC mutations carriage in FA and FA complementation group C as a risk factor for breast cancer.
Where as molecular genetic studies investigating cancer risk in heterozygotes have been mostly inconclusive. [64], A recent molecular genetic study investigating cancer risk in nine hundred and ninety four relatives of FA probands did suggest an increased risk of breast cancer in FANCC mutation carriers. [65]
However, other previous investigations suggested a significantly increased frequency of FA complementation group C sequence variants in children with Acute Myeloid Leukaemia compared to controls which lends plausible support to the hypothesis that speculates on the association that small variations in the FAC protein might be sufficient to render haemopietic stem cells more sensitive to certain environmental DNA cross-linking agents, leading to an increased risk of Acute Myeloid Leukaemia. [66 ]
This hypothesis of increased susceptibility of the heterozygotes family members of FA cases to malignancies could be further partly explained by putative inherent grading of pre-existing immune defects demonstrated in parental heterozygotes and their FA proband off springs. [67]
The positive therapeutic and the toxicity implications of achieving the diagnosis .of FA with a reasonable degree of certainty.
Issues pertaining to the aetiopathogenesis, specific interventions and
overall management of Fanconi aplastic anaemia have been extensively reviewed,
understanding these new concepts will be invaluable in formulating future management
strategies and research directions. Failure to diagnose Fanconi aplastic anaemia syndrome
may lead to delays in treatment. Making a diagnosis of Fanconi anaemia with certainty
against its other differentials will have positive implications for an appropriate use of
immunosuppressive therapy in such instances, and positive pharmaco-vigilance and anti-toxicological implications for the avoidance of the inappropriate use of intensive chemotherapeutic or
April 2014 Supplement 1 Classics and Revisits in Scientific Haematology
radiotherapeutic interventions in leukaemias or solid stromal tumours, or unduly enthusiastic marrow cytoablative therapy or other very toxic types of preparations for bone marrow stem cells transplantations.
A high index of suspicion is needed for early detection of FA cases with minimal symptomatologies and/or phenotypic expressivities.
More benign forms of Fanconi anaemias with less phenotypic expressivities may be missed in sibs of probands, so suspected or implicated cases should be evaluated for possibly associated birth defects or haematological inconsistencies suggestive of occult Fanconi aplastic anaemias and considered for a definitive evaluation. The diagnosis of Fanconi aplastic anaemias syndromes in relatives of probands will have further implications for excluding those cases as potential donors, which will contribute positively for a more successful and sustainable transplantation programmes to be achieved.
The health economics aspect of interventions for FA.
The outlook of children with FA could be considerably improved by the availability of these novel interventional options, given the concerns with costs and toxicities a more focused and directed committed colony line stimulating factor use and/or blood component therapies could be more appropriate treatment options with overall positive costs and toxicities implications.
The benefits of earlier interventions for FA.
The benefits of earlier detection of Fanconi anaemia at its presymptomatic stage in affected children using these dysmorphologies as a clue and predilection to FA will be worth exploring, since this will offer an opportunity for earlier prophylactic interventions and time for the search for a compatible bone marrow stem cell donors and appropriate pre-transplantations evaluations and arrangements.
Early detection of FA could also assist research directions, by proffering the opportunity for a more primordial comprehension of its pathogenesis and natural history.
Fanconi aplastic anaemia of childhood is a grave disease, its incidence
varies considerably globally. Fanconi anaemia syndromes meet the criteria of a
voluntary, confidential and consented screening program, because it poses a significant
burden to the population, a reliable means for its diagnosis exists, the test is cost
effective, and there are options of interventions that could alter its clinical
course and outcome considerably.
FA is a significant cause of mortality and morbidity globally.
Overall, bone marrow failures are a significant cause of
morbidity and mortality globally. The case fatality of a severe disease is considerable.
However, in ideal circumstances, earlier detection, timely presentations and appropriate
interventions with regards to allogeneic haemopoetic stem cell transplantation or multimodal immunomodulation has dramatically improved the prognosis and outcomes over the last three decades, with four fifths of the cases having a longer term complete or partial remission with either or both therapeutic options. However in some instances,
given the limited number of investigations in these aspects, and paucity of comparable
clinical and investigative scenes, many aspects of its pathological impact remains elusive or obscure..
The challenges with FA and the need for a systematic approach to a complex issue.
The partial response to androgens and steroids, the high mortality events, and early mean age at demise will suggest that timely bone marrow transplantations in these children would be beneficial and worthwhile. Continued concerns about the grave consequences of these rapidly fatal progressive pathologies could raise and support an argument for screening children with these structural and functional inconsistencies and their relatives in a non invasive way for incidental or coincidental bizarre haematological features including bone marrow hypoplasia, and occult malignancies.
A structured hospital and population based screening as a plausible interventional strategy for FA
A structured hospital and population based screening especially in children with familial history of congenital defects, those with bone marrow failures, with emphasis focused on trends of incidence, morbidity, mortality and survival.
Also an evaluation of the economics and cost benefit ratios of the various options of interventions will be rewarding.
The Need for further reappraisals and literature review.
Selected relevant extant and current global and regional literatures could be reviewed to examine the progress in the interventions for FA and to evaluate the diagnostic rarity and strength of the association between FA and these morphological or functional defects.
Other plausible therapeutic and deterrent options in addition to other methodologic issues could be discussed further in the near future.
Guest Editors: CRS [Med] Consulting Academic Clinical Editorial Experts.
April 2014 Supplement 1 Classics and revisits in Scientific Haematology
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[48]-Sleijfer D.Th, Mulder N.H, Nieweg H.O.The value of prognostic indices in aplastic anaemia. Annals of Haematology 1989; 42:2, 69 -78.
[49]-Gluckman E, Auerbach AD, Horowitz MM, et al.Bone marrow transplantation for Fanconi anaemia.Blood 1995; 86:2856-2862.
[50]-Swift, M.R.and Hirschorn, K. (1966):Ann.Intern.Med. 65.496.
[51]-Bloom, G.E., Warner, S., Gerald, P.S and Diamond.L.K. (1966 )New Engl.J.Med.274; 8.
April 2014 Supplement 1 Classics and revisits in Scientific Haematology
[52]-Giampietro PF, Adler-Brecher B, Verlander PC, et al.The need for more accurate and timely diagnosis in Fanconi anaemia: a report from the International Fanconi Anaemia Registry.Paediatrics 1993; 91:111 6-1120.
[53]-Perel Y, Butennandt O, Carrere A, Saura R, Fayon M, Lamireau T, Vergnesd P, Oesophageal atresia, VACTERL association:Fanconis anaemia related spectrum of anomalies. Arch Dis Child (April) 1998; 78:375-376
[54]-Cervenka J, Arthur D, Yasis C.Mitomycin C test for diagnostic differentiation of idiopathic aplastic anemia and Fanconi anemia. Pediatrics 1981; 67:119-27.
[55]-Windass, B: Vowels, MR: Hughes, DO: White, L.Aplastic anaemia in Childhood: prognosis and approach to therapy.Med-J-Aust.1987 Jan 5; 146(1):15-9.
[56]-Sloand EM Maciejewski JP Kirby M Kim S Young NS.Bone marrow and peripheral blood lymphocytes of patients with severe aplastic anaemia contain interferon gamma detectable by flow cytometric analysis (Abstract) Blood.1997.90 (suppl).20 b.
[57]-Ball SE,Gibson FM,Rizzo S,Tooze JA,March JC,Gordon Smith EC.Progressive telomere shortening in aplastic anaemia .Blood 1989;91.3582 -92.
[58]-Reid MM, Summerfield GP.Distinction between aleukaemic prodrome of childhood acute lymphoblastic leukaemia and aplastic anaemia.The Journal of Clinical Pathology 1992; 45:697-700.
[59]-Butturini A, Gale RP,Verlander PC,Adler-Brecher B,Gillio AP,Auerbach AD:Haematologic abnormalities in Fanconi anaemia:An international Fanconi anemia Registry Study. Blood 1994; 84:1650.
[60]-Tischkowitz M, Dokal I: Fanconi anaemia and Leukaemia-clinical and molecular aspects.Br J Haematol 2004, 126(2):176-191.
[61]-Swift M. Fanconis anaemia in the genetics of Neoplasia.Nature 1971; 230:370.
[62]-Swift M, Caldwell RJ, Chase C. Reassessment of cancer predisposition of Fanconi anaemia heterozygotes.J Natl Cancer Inst 1980; 65:863.
[63]-Potter NU, Sarmousakis C, Li FP.Cancer in relatives of patients with aplastic anaemia.Cancer Genet Cytogenet 1983.9(1):61-65.
[64]-Matthew CC. Fanconi anaemia genes and susceptibility to cancer.Oncogene 2006.25(43):5875-5884.
[65]-Berwick M, Satagopan JM, Ben-Porat L, Cralson A, Mah
K, Henry R, Diotti R, Milton K et al.Genetic heterogeneity among Fanconi anaemia
heterozygotes and risk of cancer. Cancer Res 2007.67(19):9591-9596.
[66]Awan A, Malcolm Taylor G, Gokhale D A. Increased Frequency of Fanconi Anaemia Group C Genetic Variants in Children with Sporadic Acute Myeloid Leukaemia.Blood .1998; 12:4813-4814.
[67]-Giuseppe C,Ciro G.C,Maria Napolitanoa,AsciertoP.A.Immunological Phenotype analysis of Patients with Fanconis Anaemia and Their Family Members.Acta Haematol 1998;100:39-43.
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Classics and Revisits in Scientific Medicine CRS[Med.] April 2014Vol 1(1)
April 10, 2014 Leave a comment
Final Complet A4Final Complet A42Final Complet A43Final Complet A44
Profile of Specialty Focused Journal Supplements
Classics and Revisits in Scientific Cardiology.CRS[Card.]
A focused review of the Cardiac Anatomy
Angina Pectoris and Acute Chest pain of Cardiac origin
Classics and Revisits in Scientific Dermatology CRS [Derm.] A directed anatomical study for the dermatological structures
Classics and Revisits in Scientific Endocrinology CRS [End.]
Classics and Revisits in Scientific Gastroenterology.CRS [Gast.Ent.]
A precise Anatomical Data for the Gastrointestinal System
Profile of Specialty Focused Journal Supplements
Classics and Revisits in Scientific HaematologyCRS [Haem.]
Characteristic Fanconi Anaemias in Childhood.
Classics and Revisits in Scientific Hepatology.CSR [Hep.]
A precise Anatomical Data for
the Hepatobiliary System
Classics and Revisits in Scientific History of Medicine.CRS [His.Med.] Classics and Revisits in Scientific Infectious Diseases.CRS [Inf.Dis.]
Classics and Revisits in Scientific Medicine CRS [Med.]
April 2014Vol 1(1)
Profile of Specialty Focused Journal Supplements
Classics and Revisits in Scientific NeonatologyCRS [Neon.] Classics and Revisits in Scientific NephrologyCRS [Neph.]
Classics and Revisits in Scientific NeurologyCRS[Neur.] Classics and Revisits in Scientific Oncology CRS[Onc]
Classics and Revisits in Scientific Medicine CRS [Med.] April 2014Vol 1(1)
Profile of Specialty Focused Journal Supplements
Classics and Revisits in Scientific Preventive MedicineCRS[Prev.] Classics and Revisits in Scientific Pulmonology CRS [Pulm.]
Classics and Revisits in Scientific Psychiatry CRS[Psych.] Classics and Revisits in Scientific Radiology CRS[Rad.]
Classics and Revisits in Scientific RheumatologyCRS [Rheum.]
Osteogenesis Imperfecta fragilis with blue sclerotics in childhood
Classics and Revisits in Interdisciplinary Medical Themes.CRS [Int.Disc.Med.Them.]
Proteus syndrome
Cervical haemangiomas with intracerebral extensions
Contents
Background and purpose of Journal and Supplements 2 .
List of Specialty Focused Journal Supplements Profiles 3. Journal and Supplements Editorial policy 4
Specialty Focused Journal Supplements Profiles. 7
Classics and Revisits in Scientific Cardiology Supplement (1) 14
Angina pectoris and Acute Chest Pain of Cardiac Origin.
Classics and Revisits in Scientific Rheumatology Supplement (1) 22
Osteogenesis Imperfecta Fragilis with Blue Sclerotics
in Childhood
Classics and Revisits in Scientific Interdisciplinary Medical Themes.
Proteus Syndrome in Childhood 29
Cervical Haemangiomas with Intracerebral extensions. 47
Classics and Revisits in Scientific Haematology. 49
Fanconi Anaemias in Childhood.
April 2014 1(1) Classics and Revisits in Scientific Medicine.
Back ground
Classics and Revisits in Scientific Medicine CRS [Med.] is new.
CRS [Med] is a balanced portal of continuing medical education information ,
Purpose
The intent is that CRSM [Med] could provide a fairly well effective reciprocal communicative pathway for continuing medical education development.
The text delivers the details of good basic and clinical medical practice clearly and concisely .It is a unique contribution to medical specialty practice development nationally, regionally and globally, in the context of ongoing health trends.Although the current directions in health care developments were highlighted, an unpretentious emphasis to quite often unavailable advanced technological innovations and models were reasonably. weighted.
Focus and Theme
Throughout the series efforts were made to balance the emphasis on the scientific fundamentals and the basic principles of management and prevention with research directions and recent advances in investigations and interventions.
In order to make it more reader friendly and handy, efforts were made to minimize the volume while at the same time not compromising the contents.
On this basis, suggestions were made that it should be a regular and frequent topical medical periodical in scientific basic and clinical medicine.
It is hoped that these compendia will be found most rewarding by medical practitioners, health professionals undergoing their undergraduate and postgraduate medical trainings and other health conscious professionals and individuals.
This is a legendary textbook of scientific medicine in a journal format.
Issues and Future Trends
In this edition of the CRS [Med], a preamble of the format of the publications is suggested.
However, it would be worthwhile to hasten to say that it is likely that there may be considerable alterations in these serial publications with regards to their academics structure, print layout, constituent parts, focus and certainly contents and periodicity.Therefore suggestions and opinions on contents inclusions and modifications are welcome.
In principle, publications would normally follow positive peer and editorial reviews, revisions and decisions.
Specialty Focused Journal Supplements
For clarity sake, the other associated serial medical scientific publications and channels of classics and revisits in scientific medicine, which will normally be published periodically as attached, merged or adhoc as isolated supplements to the parent journal CRS [Med] are alphabetically itemized according to the relevant medical specialties focus as follows:
April 2014 Vol 1(1) Classics and Revisits in Scientific Medicine
April 2014 1(1) Classics and Revisits in Scientific Medicine.
Specialty focusedSupplements to JournalNumber Specialty focusedSupplements to Journals Fulland abridged Title Anticipated IssuesPer year/PublicationCycle Specialty focusedJournalSupplementClassification
01 Classics and Revisits in Scientific Cardiology- CRS [Card.] 12Monthly Academic, clinical.professional&Scholarly
02 Classics and Revisits in Scientific Dermatology. CRS [Derm.]. 12Monthly Academic, clinical.professional&Scholarly
03 Classics and Revisits in Scientific Endocrinology. CRS [End.]. 12Monthly Academic, clinical.professional&Scholarly
04 Classics and Revisits in Scientific Hepatology . CRS [Hep.] 12Monthly Academic,clinical,professional&Scholarly
05 Classics and Revisits in Scientific History of Medicine. CRS [His.Med.] 12Monthly Academic, Informative.professional&Scholarly
06 Classics and Revisits in Scientific Infectious Diseases. CRS[Inf.Dis.] 12Monthly Academic, clinical.professional&Scholarly
07 Classics and Revisits in Scientific Interdisciplinary Medical Themes.CRS [Int.Disc.Med.Them.] 12Monthly Academic, Clinical, Informative.professional&Scholarly
08 Classics and Revisits in Scientific Neonatology. CRS [Neon.] 12Monthly Academic, clinical.professional&Scholarly
09 Classics and Revisits in Scientific Nephrology. CRS [Neph.] 12Monthly Academic, clinical.professional&Scholarly
10 Classics and Revisits in Scientific Neurology. CRS [Neur.] 12Monthly Academic, clinical.professional&Scholarly
11 Classics and Revisits in Scientific Oncology. CRS [Onc.] 12Monthly Academic, clinical.professional&Scholarly
12 Classics and Revisits in Scientific Preventive Medicine. CRS [Prev.Med,] 12Monthly Academic, clinical, Informativeprofessional&Scholarly
13 Classics and Revisits in Scientific Pulmonology. CRS [Pul.] 12Monthly Academic, clinical.professional&Scholarly
14 Classics and Revisits in Scientific Psychiatry. CRS [Psy.] 12Monthly Academic, clinical.professional&Scholarly
15 Classics and Revisits in Scientific Radiology. CRS [Rad.] 12Monthly Academic, clinical.professional&Scholarly
16 Classics and Revisits in Scientific Rheumatology. CRS[Rhem] 12Monthly Academic, clinical,professional&Scholarly
17 Grey Medical Literature. Ad-hoc Academic&Scholarly
18 News Letters,Brochures&Bulletins Ad-hoc Professional
19 Medical Health Updates Ad-hoc. Clinical &Professional
20 Other Miscellenous Publications Ad-hoc Clinical&Professional
April 2014 1(1) Classics and Revisits in Scientific Medicine.
Editorial Policy
We are pleased to consider papers in the following categories.
Original Articles from primary and secondary research:
The original articles could normally be up to 3000 words.
The documentations of the original papers should follow the traditional IMRAD pattern introduction, methods/ patients, results.discussion.conclusion, acknowledgements and references
The contents are expected to be precise, factual and as concise as possible.
Illustrative colour, black and white photographs and line drawings could be used if they could make the contents more lucid.
The abstract or the summary section should be structured and limited to 250 words or less.
Review Articles:
Review articles would generally be less than 3000 words.
It should be well researched and referenced.
Short Reports and Brief Communications:
Short reports and brief communications may contain up to 1000 words.
No summary or abstracts are required, but authors could decide to include them.
The contents should be logically ordered, but division into sections is optional and up to ten references may be used.
This may be suitable for the presentation of descriptive studies, some personal experience/observation and some field studies.
Case Reports and Case Series
This is the format most suitable for the presentation of individual case reports of particular interest or an illustrative series of cases.
Correspondence to the Editor
This could be on any topic related to the subject areas of the journal, which could be new topics or based on articles which have appeared in the series.
Current Topical Issues, opinions, view points, annotations and perspectives
This part of the series will provide a forum for expert opinion on topical issues in the area relevant to the series.
Appropriate submissions may include proposals for action such as:
[I]-Sound clinical judgment and good practice.
[II]-Appraisal or critiques of ongoing practice or principles.
[III-Responses to published statements.
The length of submissions in this section should normally be less than 1000 words. The presentations are expected to be precise, succinct and factual.
Usually about 20 references or less will suffice.
Occasional papers
Manuscripts of reasonable academic or clinical relevance belonging to this category could be published in the journal or in its supplements.
April 2014 1(1) Classics and Revisits in Scientific Medicine.
Research Letters:
This is a good medium for communicating research findings, which are too brief for short reports or brief communications.
Supplements:
Supplements to the series by Consulting Guest Editors on emerging topical issues will be published with a specialty based focus, periodically and on ad-hoc basis.
Normally these specialty focused supplements will be published as attachments, merged or isolated and detached supplements to the parent journal CRS [Med]
Technical Notes, Methods and Devises:
Papers describing procedures, techniques or equipment adapted by authors to their own conditions of work are welcome.
Medical Highlights and Forum Digest:
Summaries or Abstracts of ongoing and emerging topical medical issues will be published in the relevant sections of the journal or its specialty supplements.
Normally, these abstracts and summaries will be limited to 350 words or less and are best presented unstructured.
Editorials:
Normally, each series will be accompanied by an editorial perspective which as much as possible will aim to illuminate on the most significant contributions in the series.
Periodically, and on ad hoc basis editorials by consulting academic clinical guest editors will also be included, in the journal series or its supplement.
Book updates, Book Reviews and the Academic Clinicians Book shelf:
The series will consider book reviews, book updates relevant to the specialty series. A book shelf highlighting other updated books relevant to the specialty could be mentioned or displayed.
Report of the Highlights of Meetings and Conference Proceedings:
Proceedings of meetings and conferences relevant to the theme of the series could be published along the main journal series or as a supplement.
The specialty relevant highlights of appropriate medical scientific meetings will be published in the journal series or as supplements.
Academic Clinicians Web-Blog watch and alert:
Clinicians and medical scientists could be made aware of interesting Web sites and web-blogs in their relevant specialties.
Expert Opinions and View Points:
This involves a best evidence based expert advise and answer on a difficult or ambiguous academic or clinical encounter in any area relevant to the scope of the series. Normally the question or the scenario would be presented in a contextual format.with no more than 250 words.
The interventional answer or the solution to the difficulty will then be printed along with the question in the journal or a supplement following consultations with academic clinical experts in the topic and specialty.
April 2014 1(1) Classics and Revisits in Scientific Medicine.
Ongoing and forthcoming medical conferences, courses and pathways to medical career opportunities and progressions.
The series will from time to time and on adhoc basis publish a list of accreditated medical conferences, courses and achievable pathways to medical career progressions and opportunities.
Health News
The series will from time to time publish a balanced scientific perspective of the medical health literature on emerging clinical issue of public health importance.
Profile of Medical Journals and other related Publications
Editors of Medical Journals or other relevant scientific medical publications could
proffer copies of their publications to the Classics and Revisits in Scientific Medicines.
A compilation of these publications would then be sent to our consulting guest editors, referees and reviewers for short listing, vetting and selection.The publications will then be showcased in the upcoming issues of CRS [Med] or its supplements.
Clinical debates and Round Table discussions.
Logical and constructive debates and discussions on emerging topical health issues, on clinical issues which could influence practice positively will be published in the journal or its supplements periodically or on adhoc basis.
Text Presentation Format:
Text should be double spaced and all pages should be numbered.
The first page should include the title and the full names of authors with their academic and professional qualifications and/or job title and institutional affiliations at the time of writing, postal and E-mail addresses, telephone and fax numbers or other relevant contact co-ordinates for correspondence.
Tables, figures and text boxes should appear on separate pages at the end of the document and be appropriately labeled.
Text Boxes
Text boxes should be used for tangential information, such as contact details or background information of organizations relevant to the article, lists of very short case studies or descriptions of related research/projects and details of elements of a study or project, such as a survey, which for reasons of clarity are best removed from the main body of the text.
References and Acknowledgements:
References and acknowledgements should each start on a new page.
References should be outlined using the Vancouver referencing style, as originally published by the International of Medical Journal Editors (ICMJE)
References should be listed numerically in the order in which they appear in the text. The numbers of the references in the text could be in superscript.
A corresponding numerical list of the references is supplied at the end of the text after conclusive remarks.
April 2014 1(1) Classics and Revisits in Scientific Medicine.
References should be adequate, but redundant references should be avoided.
The full list of references should include the names and initials of the authors up to six authors could be listed then followed by et al for the outstanding authors, title of the paper, journal title, year of publication, volume number, first and last page numbers.
References to books should give the book title, place of publication, publisher and year, those of multiple authorship should also include
chapter title, first and last page numbers and the names and initials of the editors.
Papers accepted but not yet published should be included in the references followed by (in press)
Those in preparation (and any submitted for publication), personal communications and unpublished observations should be referred to as such in the body of the text.
Consent:
[I]-Original sentences and paraphrases replicated and quoted from other authors should be cited in a standard manner and be referenced appropriately.
[II]-The consent of patients and approval of the protocol by an ethical
committee or relevant authority on ethical matters should be confirmed for human investigations.
[III]-Any potentially defamatory statement or those construed to be defamatory must be eschewed.
[IV]-Any tables or illustrations previously published should be accompanied by the written consent of the copyright holder to republication, an acknowledgement included in the caption and the full reference should be included in the list.
Patient confidentiality:
Where a patient might be identified through an illustration or from the text, it is essential that written permission is obtained from the patient and forwarded with the manuscript.
Proofs and off prints
Proofs are sent to authors designated to receive them and corrections should be made within specified guidelines.
Covering Letter:
A covering letter signed by all of the authors must be submitted with the articles, and original or secondary research papers.
E-mail submissions should be sent to the Classics and Revisits in Scientific Medicine editorial section with record of all approved authors e-mail accounts.
The letter must contain the following information:
[I]-Why the submission is appropriate for publication in Classics and Revisits in Scientific Medicine or its specialty focused supplements and what it adds to the existing body of medical scientific knowledge.
[II]-The manuscript category that the paper is for.
April 2014 1(1) Classics and Revisits in Scientific Medicine.
[III]-Confirmation that the paper meets the requirements for the category as laid out in this document, stating word count and confirming that references were formatted in the Vancouver style as detailed below.
[IV]-Confirmation that the paper has not been published elsewhere.
[V]-Declaration of competing interests or the absence of competing interests and the disclosure of all sources of funding.
[VI]-Original research must declare ethical approval from an appropriate body and consent from participants.
[VII]-Name the corresponding author and provide full contact details.
[VIII]-Authors should also include written consents from those individuals being acknowledged in their paper.
Key Words
Authors are advised to include about five main key words before their introduction, this will facilitate the indexing of the article.
Language Support
Ideally, authors are advised to seek the assistance of a native English linguist for a linguistic editorial revision, however if this is unachievable, the Editors will provide the necessary linguistic support.
Copyright.
Before publication, authors would be asked to transfer the copyright of their contribution to the publishers of the Journal or its relevant supplement.
Check Lists for Authors:
Please ensure inclusion of the following before the manuscript submission:
.Manuscript in Microsoft word.
.Manuscript corresponding authors name, qualifications, degrees and/or job title
contact co-ordinates outlining their institutional affiliations, postal address,e-mail and telephone/ fax numbers.
.Submission covering letter.
Consent forms where applicable.
Papers may be submitted in one of the following ways: :
.Two copies of the manuscripts typed and double spaced.
.On a disk accompanied by one printed copy.
.Via E-mail to:
Dr.Emmanuel.G.U. Onyekwelu
Honorary Academic Clinical Editor
Classics and Revisits in Scientific Medicine.
P.O.BOX 2696
Serrekunda Post Office
The Gambia.
West Africa.
Tel: +220/9908295/2207357804.
E-mail:euonyekwelu@hotmail.com
Website: cottageclinicdoctors.wordpress.com
April 2014 Vol 1(1) Classics and Revisits in Scientific Medicine
Journal and Supplements Subscriptions and off prints enquiries.
Subscription enquires and Editorial communications about prints and off prints of the Journal or its supplements should be addressed to the above address.
The concessional subscription rate for this issue is the equivalent of Ten Euros.
CRS [Med]Classics and Revisits in Scientific Medicine.
This novel edition of CRS[Med] is a composite compilation of continual medical educational resource consisting of a profile of four supplements to the journal with a medical specialty focus with a considerable weighting given to their editorial aspects and themes:Classics and Revisits in Scientific Cardiology.CRS[Card]Classics and Revisits in Scientific Rheumatology .CRS [Rhem.]Classics and Revisits in Scientific Interdisciplinary Medical Themes.CRS[Int.Disc.Med.Them]
Classics and Revisits in Scientific Haematology.CRS[Haem,]
The Classics and Revisits in Scientific Cardiology.CRS[Card] journal supplement Presents a Guest Editorial Perspective on Angina pectoris and Acute chest pain of Cardiac origin by the CRSM Academic Clinical Consulting Guest Editors.
Classics and Revisits in Scientific Rheumatology .CRS [Rhem.] Classics and Revisits in Scientific Rheumatology .CRS [Rhem.]With a paediatric slant dilates on the peculiarities of Osteogenesis Imperfecta fragilis with blue sclerotics in childhood as a Guest Editorial review article by the CRSM Academic Clinical Consulting Guest Editors.a paediatric slant dilates on the peculiarities of Osteogenesis Imperfecta in negroid Africans as an Editorial short communication.
Classics and Revisits in Scientific Interdisciplinary Medical Themes.CRS [Int.Disc.Med.Them] with a paediatric slant.This supplement includes a guest editorial review article on Proteus syndrome in childhood and cervical haemangiomas in children with an intracerebral extension as editorial review articles by the CRSM Academic Clinical Consulting Guest Editors.
In this Classics and Revisits in Scientific Haematology.CRS [Haem] supplement with a paediatric slant characteristic Fanconis anaemia in childhood was discussed as an Editorial review article by the CRSM Academic clinical consulting Guest Editors.
Specialty Focused Journal Supplements Profile April 2014Supplements to Classics and Revisits in Scientific Medicine.Volume No. 1 Issue No.1
Classics and Revisits in Scientific CardiologyCRS [Car]
Angina Pectoris
and Acute Chest
pain of Cardiac origin
Classics and Revisits in Scientific Haematology
CRS[Haem]
Fanconi-anaemias
Classics and Revisits in ScientificRheumatologyCRS[Rheum]
Osteogenesis Imperfecta
Classics and Revisits in ScientificInterdisciplinary Medical ThemesCRS [Int.Disc.Med.Them.]
Cervical haemangiomas with intracranial extensions
and complications
Proteus Syndrome
Contents:
. Acute Chest pain of cardiac origin and Angina Pectoris.
. Osteogenesis Imperfecta fragilis with blue sclerotics in childhood.
. Proteus syndrome in childhood.
. Cervicalhaemangiomas with intracerebral extensions in childhood
. Characteristic Fanconi anaemias in childhood.
April 2014 Supplement 1 Classic and Revisits in Scientific Cardiology.
Angina Pectoris and Acute Chest pain of cardiac origin
Guest Editors: CRS [Med] Consulting Academic clinical Experts.
Definition:
Angina pectoris is an acute chest pain of cardiac origin due to a poor reception of oxygen supply by the heart muscles:
Aetio-Patho-physiology:
Angina pectoris usually ensues when the oxygen demands and requirements of the heart exceeds the capacity of the coronary arteries to supply an adequate amount of blood to the heart for the coronary cardiac perfusion.
In typical exertional angina pectoris, ischaemia results when myocardial oxygen demand is increased but supply is relatively compromised because of occlusion of more than fifty percent of the coronary arteries either directly through the obstruction by the eccentrically located arteriosclerotic plaque or following dynamic coronary artery vasoconstriction of the subjacent areteriosclerotically uninvolved vessel muscle wall.In the setting of atherosclerotic blood vessel pathology.
Usual Causes:
Artheriosclerosis, Coronary Artery Spasms, Anaemia and Erythrocytopaenia:
Coronary-Cardiac perfusion could be compromised or made ischaemic by a diminution in the caliber of the coronary arteries, erythrocytopaenia or anaemia.
The diminution in the caliber of the coronary arteries may be arteriosclerosis related or sympathetic dysautonomic resulting in coronary artery spasms.
The usual causes of erythrocytopaenia or anemia were reviewed elsewhere.
In intense anaemia or erythrocytopaenia, angina pectoris is likely because the number of red blood cells [which contains haemoglobin –the oxygen carrying molecule in the red blood cells or the amount of haemoglobin in themselves is below the normal range for the age and gender of the case leading to a diminution in the oxygen supply to the heart muscles and acute cardiac pain-angina. Other pathologies which cause an increase in the work of the heart and thus an augmentation of the oxygen requirements of the heart include:
Hypertension:
Intense to profound high blood pressure causes coronary artery vascular flow resistance following fibromuscular hyperplasia of the coronary artery wall and coronary artery fibromuscular spasms from reflex sympathetic dysautonomia.
Valvular Heart Disease:
Cardiac valvular stenosis or narrowing and valvular regurgitations or incompetencies especially of the aortic valves given its close anatomical relationship to the coronary arteries associated with rheumatic valvular heart disease or of other aetiological factors will lead to a net ineffective or insufficient aortic outflow and consequently coronary artery perfusion difficulties.
April 2014 Supplement 1 Classic and Revisits in Scientific Cardiology
Angina Pectoris and Acute chest pain of cardiac origin.
Cardiomyopathies:
Idiopathic heart muscles pathologies especially the hypertrophic obstructive cardiomyopathic forms leads to a thickening of the cardiac muscles -increasing the surface areas to be perfused, in addition to causing a compressive effect on the coronary arteries and a relative myocardial perfusion insufficiencies.
Arrythmogenic Right Ventricular Dysplasia or Type Four Cardiomyopathy could also cause arrhythmia related angina pectoris.
Hypercoagulable states:
The angina pectoris associated with hypercougulable states are usually a consequence of compromised ischaemic oxygenation of the heart muscles secondary to poor myocardial perfusion following sludging of poor blood flow due to its hyperviscocity.
Cardiac Dysarythmias:
Atrial and ventricular fibrillations are usually associated with angina pectoris because of the associated poor ventricular filling and hence cardiac output.
Aetiopathogenic Classification Scheme for Angina Pectoris:
Effort Angina Pectoris:
This is a chest pain or discomfort that typically occurs with activity or stress.
This pattern of angina is usually transient, but appears to be induced repeatedly by these activities or stress profile in a predictable and regular manner.
Postural Angina Pectoris
This is the form of angina that occurs on sudden recumbency due to the redistribution of the body fluid with an increase in left ventricular end diastolic pressure initiated increase in the cardiac work load and subsequent myocardial ischaemia.
Cardiac Syndrome X:
This form of angina pectoris usually affects middle aged menopausal women following hysterectomy and it is usually associated with ST segment depression on exercise thread mill test, but angiographically normal coronary arteries.
The prognosis for progression to coronary artery disease is the same as those for the normal population.
The cause of this pattern of angina is thought to be due to narrowing of the smaller coronary arteries or arterioles due to neurotransmitter or myocytes related chemical imbalance causing ventricular dysfunction or other forms of coronary arteriolar dysfunctions.
Hypersomniac Angina Pectoris:
This is angina related to sleep, especially the rapid eye movements [REM] sleep, it is probable that this form of angina is related to the relative hypoxia in this stage of sleep or the obstructive sleep apnea syndrome or associated nightmares.
April 2014 Supplement 1 Classics and Revisits in Scientific Cardiology
Variant or Prinzmetal Angina Pectoris:
This is an effortless Angina Pectoris and refers to episodic unpredictable angina which occurs during rest rather than during activities and it is usually accompanied by specific electrocardiographic changes such as transient ST segment elevation during the anginal attacks.
This form of angina is thought to be consequential to spasms of the large coronary arteries on the surface of the heart with or without atherosclerotic lesions.
Unstable Angina Pectoris:
This form of angina pectoris is metarmorphic.
It refers to angina pectoris with variable symptomatologies,given the constant symptomatology patterns of angina in any one individual, any augmentative change in its symptomatologies such as its severity, attack frequencies, uninduced events or whilst in the restive state should be taken seriously, and investigated, because the risk of a heart attack is usually considerable, because this may indicate a sudden narrowing due to a new cloth formation or rupture of an atheroma.
This form of Angina Pectoris is considered a form of Acute Coronary Syndrome.
Symptomatologies:
More often than not anginal pains are usually felt as a pressure or an ache beneath the breastbone-sternum.
This is usually interpreted as a chest discomfort or heaviness rather than pain.
The affected individual has an exertional chest discomfort assuaged by rest.
This discomfort may also occur in either shoulder or down either sides of the arm, the back, the throat, jaw or the teeth or as a burning sensation especially in the middle aged female.Anginal pains are usually transient, worse following cigarette smoking, emotional, exercise, exposures to cold or physical exertions especially after meals, walking in cold windy environment, sleep or nightmares
Patterns of anginal pains may be predictable or unpredictable.
Diagnosis:
Accurate historical details of the nature of the pain following the Ryles ten point algorithm questionnaire with its associated symptomatologies is very crucial for the exact diagnosis of angina pectoris. A physical examination in between events although mandatory may not be all that very useful. If an opportunistic examination is achievable during an event, a mild tachycardia, hypertension or dysarrthymia or specific or non specific ECG changes may be observable.
Coronary reserve is mostly compromised most in the subendocardium, ischaemia occurs initially in that region. If an ECG is undertaken during an episode of an anginal pain, ST segment depression suggestive of a subendocardial ischaemia will be observable.
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Angina Pectoris and Acute Chest Pain of Cardiac Origin.
Adverse prognostic features in Angina pectoris
The prognosis for angina pectoris is usually affected adversely by advancing age, extensive coronary artery disease, diabetes mellitus, hypertension, previous heart attacks, tobacco smoking, hypercholesterolaemia, events with excruciating chest pains, ventricular pump failures, the extent and severity of the coronary artery occlusions, abnormal ECG results, the prognosis for people with stable angina and efficient ventricular pump function, the prognosis is reasonably good.
Although the mortality rate for people with isolated angina pectoris is about 1.5%, it is considerably higher for those with the risk factors outlined above.
A good historical description of the symptoms may be enough to achieve the diagnosis of angina pectoris in most instances of the classically typically presenting cases.
Electrocardiogram
Electrocardiogram (ECG) will show ST segment changes as outlined above.
Interval ECGs are not usually very satisfactory, most useful information are usually achieved with the baseline and pharmacological exercise stress testing ECGs using a vasodilator.
Continuous ECG monitoring with a Holter monitor may detect abnormalities indicating symptomatic or silent ischaemia or prinzmetal angina which typically occurs at rest.
Exercise Stress Test.
An exercise stress test incites myocardial ischaemia, in patients with coronary artery diseases and offers some useful prognostic information to the clinician.
The patient who manifests ischaemia either clinically or electrocardiographically following the first few minutes of exercise is likely to have myocardial insufficiency related to coronary artery dysfunction or disturbances of its structural integrity.
An exercise stress test should be undertaken to define objectively the degree of exertion required to induce myocardial ischaemia due to coronary artery related haemodynamic disturbances. Patients with a positive exercise stress testing should be presumed to have a relatively considerable morbidity and should be referred for further angiographically gated imaging procedures.
Echocardiography.
In this procedure, ultrasonic waves could produce images of the heart called echocardiograms thereby outlining the size of the heart, the movement of the heart muscles, blood flow through the heart valves and the valvular functions during rest or related to exercise ischaemic conditions, Left ventricular functions is usually compromised by ischaemia
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Angina Pectoris and Acute Chest Pain of Cardiac Origin.
Coronary Angiography:
For this investigation to be achieved, x-rays of the coronary arteries are taken after a radiopaque dye is injected.
Coronary angiography is probably the most accurate procedure for diagnosing coronary artery disease.
It is usually indicated when the diagnosis of angina pectoris or coronary artery disease is uncertain.
Coronary angiography is commonly used to help evaluate whether Coronary by-pass Graft or percutaneous coronary intervention is appropriate.
It could also be employed in the diagnosis of a spontaneous or drug induced coronary artery spasm.
Multimodal Computer Assisted Tomographic Scans.
Electron beam computer assisted tomographic scans could detect the amount of calcium deposits in the coronary arteries.
The amount of calcium present could be computed as the calcium score is roughly proportional to the likelihood of the person having an angina or a heart attack. The limitation of this procedure is that occasionally, calcium deposits may be present in people whose coronary arteries are not terribly compromised so the scores derived may not very much predict the need for a percutaneous coronary intervention or coronary artery bypass grafting.
Given the significant radiation exposure with this procedure, EBCT will not usually be applied universally, but would be restricted to those cases with a relatively significant high risk of death or acute coronary syndrome especially those with risk factors such as metabolic syndromes, diabetes mellitus, hypertension, hypercholesterolaemia, hyperuraceamia, abnormal, unclear or ambiguous test results.
Multidetector series Computer Assisted Tomographic scan
This is a new technique that employs a high velocity CT scanner comprising of multiple small detectors that could accurately identify coronary artery narrowing.
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Angina Pectoris and Acute Chest Pain of Cardiac Origin
However the amount of radiation exposure is considerable.
It could also be used to ascertain the patency of a stent or a bypass graft, or to ascertain if atheromas have undergone dystrophic calcifications.
In addition it is very useful for displaying the cardiac and the coronary venous anatomy.
This procedure is absolutely contraindicated in pregnancy, or in patients who are unable to hold their breath for at least a quarter of a second or tachycardiac or cannot tolerate bradycardia or the drugs used to induce bradycardia.
If the test must necessarily be employed in patients with tachycardia, they may need to be allowed a cardio-selective beta blocker to regulate the heart rate below 70 beats per minute.
The technique is noninvasive and highly accurate in excluding coronary artery narrowing as a source of a person’s symptoms especially in those instances where the exercise stress test was unachievable or its results inconclusive.
Cardiac Magnetic Resonance Imaging
In cases of coronary artery disease, MRI could be employed to evaluate the narrowing of the coronary arteries, measure the blood flow in the coronary arteries and test how well the heart is being supplied with oxygen.
It could also be used to assess the abnormalities of the heart wall motion during exercise stress test which may signify a poor blood supply to that area.
MRI may also be used as a test of viability to assess whether areas of the heart muscles damaged following an acute coronary syndrome could recover.
It could be used to evaluate the heart and the large blood vessels arising from the heart such as the aorta and the pulmonary arteries.MRI is radiation free.
Management:
Treatment of angina pectoris depends partly on the stability and severity of the symptoms of the angina.
Management of Angina pectoris implies attempts to slow or reverse the progression of coronary artery disease by the rapid treatment and modifications of risk factors, such as hypertension, high cholesterol levels, cessation of cigarette smoking, obesity, recommendation of a low varied polyunsaturated fatty diet, which is low in highly refined sugar carbohydrates and exercise.
The patient will need to comprehend the pathological process to drive the motivation to modify coronary artery disease risk factors and to be able to know
where and when to seek medical assistance during aggravation of symptomatologies.
In stable angina with symptomatologies of mild to moderate severity, modifications of the above risk factors with occasional drug use may suffice.
If this approach is not enough to ameliorate the symptomatologies of angina considerably, a procedure to restore the myocardial perfusion by improving the coronary blood flow through a revascularization procedure should be recommended. When symptomatologies worsen rapidly, immediate hospitalization is usually required and the case should be evaluated for a acute coronary syndrome.
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Angina pectoris and Acute chest pain of cardiac origin.
Pharmacotherapy:
Pharmacotherapy of stable angina pectoris is aimed to ameliorate myocardial perfusion and decrease the work load on the heart.
The pharmacotherapy of angina pectoris revolves around six groups of drugs.
A-The organic nitrates
B-The beta-blockers
C-The calcium channel blockers
D-The angiotensin-converting enzyme inhibitors.
E- The antiplatelet drugs.
F-Non-opoid and opoid analgesics.
The Beta Blockers
These groups of drugs antagonize the effects of the catecholamine hormones epinephrine and norepinephrine on the heart and other organs.
These hormones have chronotropic (increases the heart rate) and inotropic effects (increases the strength of the heart contractions, and causes the arterioles to constrict (there by causing the blood vessels to constrict)
The resting and the activity related augmentation of the heart rates and blood pressures are usually ameliorated by these groups of drugs, thereby the myocardial oxygen demand is reduced. This group of drugs reduces the risk of heart attacks and sudden deaths.The long term outcome for people with coronary artery disease is therefore improved.
[2]-Organic Nitrates
[A]-Short Acting Organic Nitrates
Nitroglycerin is a very short acting organic nitrate drug that dilates the blood vessels.Following the administration of this drug .the anginal events are usually ameliorated in about two to four minutes. It is usually quite handy and could be taken shortly in anticipation of an anginal event, however the effects of nitroglycerin usually lasts about 30 minutes. The dose could be administered sublingually (i.e. as a tablet placed under the tongue) or bucally (as a tablet placed next to the gum) or intranasally (as a spray inhaled through the nostrils)
[B]-Long Acting Organic Nitrates
Isorsorbide dinitrate is an example of a long acting organic nitrate
This drug could be taken as tablets orally about one to four times daily.
It could also be administered as nitrate skin patches and paste allowing cutaneous absorption over several hours.
However if they are taken regularly they may loose their ability to provide relief.
Usually a period of nocturnal drug holiday is usually appropriate for its long term anginal relieving effect to be maintained. The use of their effect is mainly aimed towards the relieve of anginal symptomatologies rather than prophylaxis against acute coronary syndromes or sudden deaths.
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Angina Pectoris and Acute Chest pain of Cardiac origin.
[3]-Calcium Channel blockers
This group of drugs prevents the blood vessels from narrowing (constricting) and could attenuate coronary artery spasm and as a group have a hypotensive effect.
Calcium channel blockers are equally effective in Prinzemetal angina pectoris.
Verapamil and daltiezem has negative chronotropic and antiarrythmic effects.
This group of drugs on the average is used as third line drugs, if the above two groups of drugs are inadequate for symptomatic relief or are contraindicated.
[4]-Angiotensin Converting Enzymes Inhibitors
ACE inhibitors such as ramipril were often given to angina pectoris cases with the aim of attenuating the risk factors of acute coronary syndromes and sudden deaths rather than symptomatic relief of angina pectoris.
[5]-Antiplatelet Agents
Aspirin, disopyradimole, ticlopidine, and clopidegrel have antiplatelet effects and influence the function of platelets, so that they do not clump together and stick to the blood vessels walls.
Platelets which circulate in the blood, promote clot formation (thrombosis) when a blood vessel is injured.
If platelets collect on atheromas in an artery’s walls, the resulting clot could narrow or block the artery and result in an acute coronary syndrome.
Aspirin modifies the platelet function by reducing the tendency for its aggregation and thrombus formation thereby ameliorating the vascular occlusion associated with artheromatous thrombus formation.
Antiplatelet drugs are usually given to individuals with angina pectoris, unless there is a good reason not to, such as in individuals with bleeding diathesis or haematological dyscrasias of unclear significance or consequence.
The traditional teaching is for individuals with coronary artery syndrome to take cardio-aspirin 75mg daily in addition to clopidogrel daily to reduce the risk of an acute coronary syndrome. Another beneficial antiplatelet drug Ticlopidine, although with more frequent side effects could be employed in individuals who are allergic or intolerant to aspirin-clopidogrel combination.
[6]-Non-Opoid and Opoid Analgesics
Analgesia of varying strengths will invariably be a component of the anti-anginal pectorisarmamentarium.
Paracetamol, Aspirin, other non-steroidal anti-inflammatory agents and codeine or morphine could be employed in combination on individual cases as bridge therapy until appropriate beta blocker or calcium channel blocker is defined.
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Angina Pectoris and Acute Chest pain of cardiac origin
In summary the objectives of the pharmacotherapy of angina pectoris is aimed at the reduction of myocardial oxygen demand during exercise or stress by the use of organic nitrates, beta blockers or calcium channel blockers
Promotion of maximal dilatation of the coronary arteries by the use of nitrates, calcium channel blockers.
Revascularization Procedures:
Percutaneous Coronary Intervention (PCI) angioplasty and Coronary Artery By Pass Grafting [CABG]
These procedures are the two main revascularization procedures employed in the management of angina pectoris where pharmacotherapy was unsuccessful.
However, it is worthwhile to note that these procedures are invasive anatomical techniques which aim to alleviate the immediate pathology, but they are not usually effective for the cessation of the progression of the underlying disease without influencing the risk factors favourably.
[A]-PCI is non-invasive and hence it is often preferred to CABG in a selected group of patients. It is particularly indicated in cases in one or two coronary artery vessel disease, especially when the blocked coronary vascular segments are not lengthy or extensive. However, increasing experience and more sophisticated techniques is allowing this procedure to be used confidently and more frequently.
[B]-CABG, is particularly beneficial to individuals with severe angina and coronary artery disease which were not amenable to relevant pharmacotherapy, good cardiac reserve and a normally functioning heart, no previous acute coronary syndromes, no chronic obstructive pulmonary disease, thrombophilias, carcinomatosis or other conditions that could preclude surgery or make it hazardous About nine of ten of such people will have a rapid and absolute relief of symptoms after surgery.
CABG could improve symptoms, exercise tolerance, and could decrease the number and dose of drugs needed
An electively planned CABG has an infinitesimal mortality at about less than 1 in 100 and a risk of asymptomatic or symptomatic intraoperative acute coronary event of about less than one in twenty.
Guest Editors: CRS [Med] Consulting Academic clinical Editorial Experts.
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Osteogenesis Inperfecta fragilis with blue sclerotics in childhood.
Guest Editors: CRS [Med] Consulting Academic clinical Editorial Experts.
An unusual preponderance of observational reports on children with phenotypic features consistent with Osteogenesis Imperfecta fragilis presenting covertly with a history of multiple long bone fractures and /or easy bruisability of the skin espoused an expert guest editorial consultations and review article on this theme.
Introduction/Historical Perspective
Osteogenesis Imperfecta or Vroliks syndrome is a heterogeneous group of inherited conditions arising from a variety of biochemical and morphological collagen defects.Willam Vrolik professor of Anatomy ,Pathological Anatomy and zoology at the Athenaeum Illustre(University of Amsterdam) discussed in his handbook of Pathological Anatomy(1842-1844) and Tabulae and Illustradam embryogenesis hominis et mamalium,naturalem tam abnorman (1844-49) a new born infant with numerous fractures and hydrocephalus.The Tabulae, had both Latin and Dutch texts, in the Latin text ,Vrolik used , the term Osteogenesis Imperefecta (in Dutch, gebrekkige beenwording) Vrolik also mentioned that the infant lived for three days and that both parents were suffering from Lues Universalis at the time of the baby’s birth .
On re-examination of the Index case.
The whole skeleton of the baby appeared poorly mineralized.
The family had large skulls, exhibited a broad and high forehead, large fontanels, frontal and temporal bossing, shallow orbits, and a protruding occiput.
At autopsy, the calvaria of the baby consisted of many wormian bones.
The tubular bones, although of normal length and only slightly curved, were very thin as were the ribs. All the skeletal structures showed one or more fractures and many fractures showed callus formation. Following further investigations on this topic, this case was retrospectively diagnosed as a case of Osteogenesis Imperfecta type II..
Willan Vrolik was one of the first scientists to realize that many skeletal dysplasias were not the result of a post natally acquired disease such as rickets or osteomalacia as many of his contemporaries believed. He thought that it might be due to insufficient intrinsic generative energy. He substantiated this by stating that in his study specimen a primary impairment of ossification is present and not a secondary degeneration.
The Index Case Description.
The descriptions given by Willem Vroliks in some of the specimens ,generated the term Osteogenesis Imperfecta and the eponym Vroliks syndrome for this genetic disorder characterized by increased fragility.[1]Fractures are frequent and its onset depends on the type of Osteogenesis Imperfecta.[2]Osteogenesis Imperfecta is a heritable disease that may result in bone fragility, increased joint laxity, decreased muscle tone, thinning of the skin, a bluish appearance of the sclera, scoliosis, deafness, dentigenosis imperfecta and other multiple abnormalities.
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Osteogenesis Imperfecta Type 1
Osteogenesis Imperfecta type 1 is a disorder characterized by frequent bone fractures and deformities associated with fracture malunion and poor healing, blue sclera, normal or near normal stature and autosomal dominant inheritance pattern.
The incidence is thought to be one in about twenty thousand live births.
Males and females are affected equally Osteopaenia is associated with an increased rate of long bone fractures upon ambulation.
For some reasons not well understood, fracture frequency decreased dramatically at puberty and during young adult life but increases once again in late middle age.
Most observed patients presented with clinically overt symptomatologies prepubertally.
Progressive hearing loss, often beginning in the second and third decade, is a feature of this disease in about half the families.The proportion of Osteogenesis Imperfecta Type 1 patients with significant hearing loss rises steadily into middle age ,despite the general decline in fracture frequency .Conductive or mixed conductive and sensorineural hearing loss are more common in dominant pattern oseogenesis imperfecta than sensorineural hearing loss alone. Dentigenosis Imperfecta is observed in some subset of the patient population.
Mortality Figures.
Mortality could be significant even in hospitalized patients at about three percent..
Mortality figures are 3.7 times more in the negroid race.
OI related demises are more likely in women. Admissions at younger age connote more severe disease.
Children at a younger age are more severely affected and more difficult to manage.
They tend to fair better when referred to a dedicated paediatric unit [3]
A classification scheme and the inheritance pattern for OI
Inheritance pattern depends on the type. The classification of OI has been debatable.
The detailed aspects of its nomenclature have been reviewed.
Anecdotally there are four types of Osteogenesis Imperfecta, although with recent advances in molecular biological techniques, other sub-types have been deciphered with considerable over-lap between the subtypes. The mode of inheritance is autosomal dominant for Type1and VI and recessive for Type II and III. It has been reported in one of two twins. [4]Osteogenesis Imperfecta is a defect of Type 1 collagen which is present in many tissues including blood vessels.
The differentiation of child abuse and Osteogenesis Imperfecta is critical, because of medical and legal implications for the latter. [5, 6, 7]
Considerations for the Inheritance pattern in OI
The mode of inheritance of OI is variable and the clinical manifestations are heterogeneous. In addition to its bone involvement, osteogenesis imperfecta, may involve tendons, ligaments, skin scleras, dentin, fascia and blood vessels.
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A modified classification scheme for OI
The most commonly used classification schemes are those developed by Sillence and Colleagues. [8]Type 1 Osteogenesis Imperfecta, which was previously called Osteogenesis Imperfecta tarda, is the most common form and is a relatively mild to moderately severe disorder , inherited as an autosomal dominant trait with varying penetrance and expressivity.
It is associated with blue scleras and hearing loss in about fifty percent of the cases [9]
Type 2 Osteogenesis Imperfecta is lethal in the perinatal period and was previously known as Osteogenesis Imperfecta congenita, it is the most severe form and it is inherited as an autosomal recessive trait.
Types 3 and 4 Osteogenesis Imperfecta have clinical manifestations of intermediate severity between that of types 1 and 2 and are often associated with bone deformities and varying degrees of short stature and hearing loss.
Hearing loss commonly occurs in patients with Type 3 OI, it also occurs in some patients with Type 4 Osteogenesis Imperfecta. [8, 9]Loss of hearing is one of the least constant major features of Osteogenesis Imperfecta, with occurrence that varies between twenty six and sixty percent[10]The association of Osteogenesis Imperfecta, hearing loss and blue sclera’s is often referred to as de Van der Hoene Kleyn syndrome, which was originally well described in 1918 by two authors. [11]This syndrome usually occurs in the late second and early third decades of life, younger patients have a mixed or sensorineural hearing impairment.[12]
Histopathological Features and Molecular genetic pathological aspects of OI.
Findings at histopathologic examination of specimens extracted from patients with Osteogenesis Imperfecta provide some clues and values to the Type of hearing Impairment that could be expected in these patients.Osteogenesis Imperfecta.
OI is one of the inherited connective tissue disorders that result in brittle bones.
The molecular defects reside in various mutations that lead to defective or decreased Type 1 collagen synthesis [9,13, 14]In greater than ninety percent of cases , Osteogenesis Imperfecta results from mutations in one of two genes,COL1A1 gene on chromosome 17 and COL1A2 gene on chromosome 7 which encodes the chains of Type I collagen.[15,16]
Clinico-pathological correlations.
Findings at the histopathological examination of specimens from patients with Osteogenesis Imperfecta provide clues to the types of hearing impairments that could be expected in these patients.Temporal bone specimens extracted from patients with osteogenesis Imperfecta congenita(Type II) demonstrated markedly delayed and deficient ossification in the three layers of the otic capsule.[17]Conductive hearing loss is expected in these patients and is mainly due to fractures that commonly involve the crus of the stapes or the handle of the malleus.These fractures lead to discontinuity of the ossicular chain or to fixation by ankylosis of the head of the malleus to the medial attic wall, Therefore the hearing loss in Osteogenesis Imperfecta could be of a conductive type and may not always be progressive[18] Adult onset hearing loss with Osteogenesis Imperfecta therefore could be of a conductive type and is not always progressive.
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Reports of Osteogenesis Imperfecta tarda (Type 1) are somewhat scanty since the availability of specimens depends on surgical procedures or autopsies.
The whole question on the f temporal bones morphologies related to deafness in OI was answered elsewhere. [19, 20, 21, 22]
Radiological Features of OI
Conventional radiographs of the tibia and fibular in IO could demonstrate multiple healed or healing fractures of the tibia , as well as non-united fracture deformities of the tibia or the fibular shaft .The bones are usually gracile and osteoporotic in appearance, which are some characteristics radiological features of osteogenesis imperfecta.
Further discussions on the collagenous defects in OI
The evaluation of children with multiple unexplained fractures is a clinically and radiological challenging one, because of issues concerning non-accidental injuries.
An inconsistent, non-correlating historical, clinical and radiological profile and cessation of further events will point more to a non-accidental injury as the cause of the pathological fracture. Abnormalities in collagen in other tissues other than the bones will account for clinical findings in addition to fractures, such as dentinogenesis imperfecta (fragile discoloured teeth), blue or grey colour of the sclera, increased joints laxities and a tendency to bruising(thought to be due to defective collagen of small blood vessels)
The distinctive features of OI
Reports on most of the observed cases, suggest that they probably belong to the Type I Osteogenesis Imperfecta or Type III.OI subsets.
The exact classification scheme could be accomplished, clarified and distinguished with exact certainty at molecular biological studies to determine if the collagen structure is qualitatively normal, but quantitatively reduced (Type I) or defective in (TypeIII) where it is abnormal. On epidemiological basis however, Type 1 OI would likely to be the diagnosis in the most of the observed reports because it is the most common and complicating fractures may not necessarily be present at birth.
Fracture patterns in OI
All types of fractures could be seen in osteogenesis imperfecta such as transverse, oblique and spiral fractures. Greenstick fractures and torus also occur commonly in children with mild to moderately severe defects..
Localizations to the lower limbs are particularly more common in osteogenesis imperfecta in comparison to normal children. Fractures in osteogenesis imperfecta could be metaphyseal.[23, 24] Rib fractures are also relatively common.
Of all these fracture patterns, metaphyseal fractures are those with the most implication for a multidisciplinary intervention, because of its strong connotations for
non-accidental injuries. [25]
The associated osteopathies in OI with their diagnostic significance.
Given that the primary defect in Osteogenesis Imperfecta is principally with the collagen.
The anecdotal evidence that osteopaenia, osteoporosis and diminished bone density are features in Osteogenesis Imperfecta could be unfounded. This is buttressed by the fact that precise densitometry and native densitometry in unfractured bones in Osteogenesis Imperfecta is generally within the normal reference range.[26]
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Osteopaenia and other abnormalities occasionally inferred, were usually multiple previous fractures and prolonged immobilization related. This diagnostic enigma is worsened by the fact that the assessment of bone density from X–ray radiological features is unduly unreliable [27] and as such it is usually not very useful overall in achieving the diagnosis of osteogenesis imperfecta especially in older children.
The diagnostic accuracy, reontenographic-densitometric correlations and precision is more distinct in infants and children with temporary brittle bone disease [28, 29] where they could assist to distinguish these conditions from Osteogenesis Imperfecta.
However lack of a standard reference range has compromised the applicability of densitometry with robust precision.
Wormian bones in OI
Other features that could espouse the diagnosis of Osteogenesis Imperfecta includes the presence of Wormian bones which are additional small bones within the sutures of the skull as was originally described in 1643 by Olaus Worm, a Danish anatomist.
In osteogenesis Imperfecta the number of Wormian bones are usually excessive and their distribution is in a crazy-paving pattern rather than a linear profile.
They are particularly most pronounced in the majority of cases with Type III Osteogenesis Imperfecta.
However Wormian bones could also be seen in excess in the milder types I, IV and other pathological states. The x-ray radiological techniques for the demonstration of Wormian bones and its implication for the diagnosis of Osteogenesis Imperfecta and other simulating conditions is an interesting topic.[30]The lateral and especially the Townes views are the most informative views in the assessment and demonstration of wormian bones and are particularly recommended as most appropriate for the exact assessment of Wormian bones ,especially in children with fractures, where the exclusion of a non-accidental injury will be expedient. The absence of Wormian bones will not be adequate to achieve an exclusion of Osteogenesis Imperfecta, especially in early infancy when it is notoriously absent as was confirmed by interval projections latter on.
Wormian bones are not notable features in temporary brittle bone disease.
Isolated periosteal reaction as a phenomenon is not all that a common feature of classical Osteogenesis Imperfecta except in relationship to fractures.
Occasionally, symmetrical metaphyseal sclerosis could follow fractures in Osteogenesis Imperfecta.
Other collagenous defects in OI with haemodynamic and functional consequence.
Being a collagen based defect, other features such as excessive joint laxity and cerebral aneurysms[31] and cardiovascular abnormalities especially pulmonary and aortic dilatation and regurgitation, mitral valve prolapse and subtle microscopic changes in the pulmonary artery and aorta could be associated features .These associations might not necessarily be present at birth, but usually they appear with the passage of time and plays a dominant role in the natural history of the disease process, especially with regards to deleterious consequences such as aortic dissections.[32, 33, 34] OI could be associated with bleeding diathesis which could be spontaneous or follow mild trauma. [35]OI could be associated with spondylolisthesis caused by extreme pedicle elongation.
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The clinico-radiological distinction between OI and non-accidental injuries.
The Type 1 collagen is thought to make an important contribution to the structure and function of long bones.[36]From a Biomechanics perspective, bone is considered to be a non homogenous composite structure consisting at the very least of mineral and organic phases. In test of whole bone properties e.g. four point bending, the sum of the various phases of a composite structure usually contributes to the mechanical properties measured in other mechanical tests, one phase may predominate over the other.
Increased number of fractures with few or no bruises will point more to classical Osteogenesis Imperfecta rather than a non-accidental injury as the aetiological factor in cases of fractures, where as in non-accidental injury, bruising is much more common than fractures.[37, 38]
Although imaging is relevant to the diagnosis of Classical Osteogenesis Imperfecta it cannot be considered in isolation from the clinical findings, the historical events, the family history, the social assessment and the laboratory features.
Plausible pharmacological and physical therapeutic Interventions for OI
The beneficial therapeutic aspects of Osteogenesis imperfecta were contentiously discussed in the medical literature .Although no pharmacotherapeutic intervention has revolutionized the medical management of OI as such,however,the evidence for the beneficial effect of several pharmacotherapeutic interventions appears to be most convincing for the biophosphonates ,especially ,palmidronate or alendronate.
Also other reports have suggested the beneficial effect of the growth hormone in some selected group of children with OI, especially in aiding the affected children to attain an aesthetically acceptable linear growths, in other wise a subset that would have been disfiguringly stunted if the natural history of the pathology is uninterrupted with these pharmacological interventions.Orthopaedic and physical therapy in addition to modifications of functional, social professional and occupational ergonomics using assistive devices are other plausible interventional strategies.
Conclusion and Importance:
This editorial review article buttresses the need for clinicians to apply an in-depth evaluation, and scrutiny during patient’s encounter, to be able to decipher clinically covert pathological conditions and circumstances. Normally these evaluations would be most rewarding if a holistic multidisciplinary approach is introduced.
Guest Editors: CRS [Med] Consulting Academic Clinical Editorial Experts.
References:
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[3.] Vitality,MG,Matsumoto ,Hiroko MA,Kessler ,Weinstein M,Hoffmann ,William BA,Roye,David P.Osteogenesis Imperfecta.Determining the demographics and the predictors of death from an inpatient population .Journal of Paediatrics orthopaedics.March 2007,27(2).228-232.
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[4.] O.Seriki.Osteogenesis Imperfecta in one of twins.Acta Paediatrica 21Jan, 59(3).340-342. and as a familial aggregation (Braga S, Passage E.Congenital Osteogenesis Imperfecta in three sibs (Human genetics) 1Oct1981, 58(4).441-443.
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[11.] Van der Hoene J de Kleyn A.1918.Blaue Skleren, Knochen bruchigkeit Und Schwerhohrigkeit. Arch Ophthalmol 95.81-93. (German).
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[13.] Byers PH, Steiner RD, 1992, Osteogenesis Imperfecta .Annu Rev Med 1992, 43.269-282
[14.]Kuivaniem H,Tromp G,Prockop DJ 1997,Mutations in fibrillar collagens(Type I,II,II and XI) ,fibril associated collagen Type IX and network forming collagen Type X cause a spectrum of diseases of the bone, cartilage ,and blood vessels. Hum Mutat 9.300-315
[15.] Byers PH, Steiner RD 1992MOsteogenesis Imperfecta Annu.Rev.Med.43.269-282
[16.]Skyes B,Ogilvie D,Wordsworth P,et al 1990,Consistent linkage of dominantly inherited Osteogenesis Imperfecta to the Type I collagen Loci.COL 1A1 and COL1A2,Am J Hum Genet 1990,46.293-307.
[17.] Berger G, Hawke M, Johnson A, Proops D.Histopathology of the temporal bone in Osteogenesis Imperfecta Congenita. a report of 5 cases. Laryngoscope 1985, 95.193-199)
[18.] Zajtchuk JT, Lindsay JR.Osteogenesis Imperfecta Congenita and tarda. A temporal bone report .Ann Otol.1975, 84.350-358
[19.] Sando I, Myers D, Havada T, Hinojosa R, Myers E Osteogenesis Imperfecta tarda and Otosclerosis, a temporal bone histopathology report. Ann otol 1981, 90.199-203
[20]Marion MS, Hinojosa R. Osteogenesis Imperfecta Am J Otolaryngol 1993, 14.137-138.
[21.]Tabor EK ,Curtin HD,Hirsh BE, May M .Osteogenesis imperfecta tarda.appearance of the temporal bones at Computed Tomography .Radiology 1990,175.1810183
[22.]Mafee MF,Valvasson GE,Ditch RL,et al .Use of the CT scan in the evaluation of Cochlear Otosclerosis.Radiology 1985,156.703-708
[23.] Hoddsmorth CE, Endahl GL, Soifer N, et al Comparative biochemical study of Otosclerosis and Osteogenesis Imperfecta .Arch oto-laryngol 1973, 98.336-339
April 2014 Supplement 1 Classics and Revisits in Scientific Rheumatology
[24.] Dent JA, Paterson CR.Fractures in early childhood.Osteogenesis imperfecta or child abuse? J Paediatr Orthop 1991; 11:184-186.
[25.] Ablin DS, Greenspan A, Reinhart M, Grix A, Differentiation of child abuse from Osteogenesis Imperfecta.Am J Roentgenol 1990.154:1035-1046
[26.] Paterson CR, Mole PA.Bone density in Osteogenesis Imperfecta may well be normal Postgrad Med J 1994:70:104-107.
[27.] Williamson MR, Boyd CM, Williamson SL Osteoporosis: Diagnosis by Plain chest film versus dual photon absorptiometry.Skeletal Radiol 1999; 19:27-30.
[28.] Miller ME. Temporary brittle bone disease: a true entity? Semin Perinatal 1992; 23:174-182.
[29.] Koo WWK.Sherman R, Succop P et al.Fractures and rickets in very low birth weight infants: Conservative management and outcome.J Paediatr Orthop 1989; 9:326-330.
[30.]Cremin B, Goodman H, Spranger J, Brighton P, Wormian bones in Osteogenesis Imperfecta and other disorders. Skeletal Radiol 1982; 8:35-38
[31.]Hawlik DM ,Nashesky MB,Ruptured cerebral artery aneurysm and Bacterial meningitis in a man with Osteogenesis Imperfecta.American Journal of Forensic Medicine and Pathology June 2006,27(2).117-120
[32.]Modestino G,Criscitiello M ,James A ,et al Cardiovascular abnormalities in Osteogenesis Imperfecta .Circulation 1965,31.255.
[33.]Wong R.S Follis.F, M Shively.B.K, Wernly .J.A.Osteogenesis Imperfecta and Cardiovascular diseases Ann.Thorac.Surg.November 1.1995, 60(5).1395-1397.
[34.] Moore.J.B, E.G.Wook and Kinkead.L.R.Ulnar artery aneurysm in Osteogenesis Imperfecta.J.Hand Surg Eur vol, February 1983.Original series, volume 15(1).91-95.
[35.]Ganesh.A, Jenny.C,Geyer.J.Shouldice M,Leven A, Retinal haemorrhages in type 1 ostegenesis imperfecta after minor trauma.Opthalmology.Volume 111,Issue 7.Pages 1428-1431.
[36.] Martin, R.B, Burr .D.B. (1989) The structure, function and adaptation of compact bone (Raven, New York), pp.57-84.
[37] McMahon P,Grossman W,Gaffney M.Stanitski C.Soft tissue injury as an indication of child abuse.J Bone Joint Surg 1995;77A:1179-1183.
[38.]Paterson CR, Monk EA.Long term follow up of children thought to have temporary brittle bone disease.Osteoporos Int 2000;11(Suppl 4):S47-S48
April 2014 Supplement 1 Classics and Revisits in Scientific Interdisciplinary Medical Themes CRS [Int.Disc.Med.Them.]
Proteus syndrome in childhood with a selective literature review.
Guest Editors: CRS [Med] Consulting Academic Clinical Editorial Experts.
The need for an Editorial review article on Proteus syndrome PS.
An editorial review article following academic clinical experts consultations on PS is undertaken on the basis of concerns evoked following observations on few compatible cases encountered over a considerable time. Given the progressive compressive, the vascular and malignant neoplastic implications of PS,a high index of suspicion should be expedient for an early and timely exclusion or diagnosis for PS,especially those cases presently covertly, or with minimal phenotypic expressivities or attenuations.
Case Definition
Proteus syndrome (PS) is a rare sporadically occurring hamartomatous disorder
associated with mosaic multifocal irregular asymmetric overgrowth of multiple body
tissues and cell lineages. The aetiology of Proteus syndrome is still idiopathic.
However, the predominant hypothesis is that PS is caused by a postzygotic mosaic
alteration in a gene that is lethal in its nonmosaic state.
Most malformations in Proteus syndrome have a predominantly ectodermal or
mesodermal origin.
Characteristic multifocal, multicentric, metachronous or synchronous overgrowths
(partial or regional gigantism, or hemi hypertrophy) in Proteus syndrome could involve
any body structure, especially the skin, bone, connective tissues, and fat.
Globally, published or unpublished reports of PS , are still very few.
A cluster of phenotypic features evoking the possibility of PS ..
On closer scrutiny,observations and follow ups, a couple of children presented with some of the spectral phenotypic dysmorphological features of PS such as lower limb hemi-hypertrophies, cerebriform cutaneous hyperplastic unilateral sole overgrowths, widespread hyperpigmented capillary haemangiomatosis on the limbs and extremities, saccrococcygeal, gluteal, perineal protuberant lipomatous masses,congenital absence of the gluteal cleft and progressive abdominal tumefactions.
Establishing PS as the most plausible diagnosis.
Following the observations of these features consistent with the recognized clinical diagnostic criteria for Proteus syndrome, the possibility of .this diagnosis was evoked and suggested, on follow ups ,with progressive enlargement of these structures the probability of Proteus syndrome was considered and discussed.
Rare embryonic neoplasms in PS
Following focused radiological imaging procedures the diagnosis of Proteus syndrome complicated by nephroblastoma, multifocal neuroblastomas and embryonic tumours was established in these cases.Although a range of malignancies have been previously reported in PS, embryomas were relatively uncommon especially Wilms tumour and Nephroblastom as they were demonstrated in the editorial observations
April 2014 Supplement 1 Classics and Revisits in Scientific Interdisciplinary Medical Themes.
The diagnostic and therapeutic difficulties in PS
The diagnosis and management of Proteus syndrome is challenging, enigmatic and
difficult and were controversially discussed in the medical literature.
Proteus syndrome could be associated with synchronous, metachronous,
multicentric, multifocal, phenotypic expressivities and intrabdominal embryomas
Literature search to aid the diagnosis. PS.
In establishing this diagnosis,the extant,and current medical literature were briefly reviewed to examine the rarity and strengths of these associated structural features in achieving the diagnosis of this syndrome against its other differentials. Other methodological issues concerning its epidemiology, presentations, diagnosis and management were also proffered and discussed.
Case definition.
Proteus syndrome (PS) is an extremely rare disorder of patchy or mosaic postnatal
growth dysregulation, primarily involving overgrowth. The disorder is thought to be
related to a somatic genetic alteration ,but its exact etiology is still largely unknown.
Proteus syndrome was named after the Greek god Proteus who could change his shape at will.This rare disorder is characterized by multiple lesions of the mesodermal tissues, fat cells,lymphatic and blood vessels (lipolymphohaemangiomas), overgrowth of one side of the body(hemihypertrophy), an abnormally large head (macrocephaly), partial gigantism of the feet, darkened spots or moles (nevi on the skin).
Historical Perspective and Nomenclature of interest.
Studies on Proteus syndrome commenced in earnest in 1978, when Bennett proposed a putative experimental model for the mode of inheritance in PS and other related syndromes associated by a dominantly determined genomically patterned inheritance with mosaicism sustaining the survival of a naturally lethal gene.[1]
Although some evidence of this syndrome was originally published in the medical
literature as early as in 1907 or before, the more modern medical description of the
disease was attributed to Cohen and Hayden, who identified the syndrome in 1979.[2]
To stress the polymorphic nature of the clinical manifestations of this new
syndrome, in 1983,Wiedermann coined the term Proteus syndrome after the Greek mythological god Proteus (meaning polymorphous), the Old man of the Sea who could change his shape at will to avoid capture. [3]
The Elephant Man had PS on retrospective analysis.
Anecdotally, Joseph Merrick, the Elephant Man described by Sir Fredrick Treves in 1884
celebrated by stage plays and movies with the same name, was initially thought to have
suffered from elephantiasis and latter on a diagnosis of neurofibromatosis was suggested
, but now believed retrospectively to have had a Proteus syndrome after a study of the preserved castings of his soles revealed a cerebriform cutaneous hyperplasia, a characteristic finding in Proteus syndrome. [4][5]It was in 1996 that the answer to what affected Merrick was determined with exact certainty . A radiologist, Amita Sharma, of the National Institutes of Health (U.S.A), examined very meticulously the x-rays and CT scans of Merrick’s skeleton preserved at the Royal London Hospital after his death.
April 2014 Supplement 1 Classics and Revisits in Scientific Interdisciplinary Medical Themes
Dr.Sharma in 1979. determined that Merrick had Proteus syndrome, an extremely rare disorder, she asserts that Merrick’s appearance, and especially his skeleton, portrays all the identified stigmata of the defect in their extreme.
Folk-lores on the Elephant man
Historical legends had it that the head of the elephant man was so large that his hat measured about three feet in diameter.
Undoubtedly, Joseph Carey Merrick the elephant man (1862-1890) wanted to be like other people. He often wished he could lie down and sleep, but because of the size and weight of his head he had to sleep sitting up. One morning in 1890 he was found lying down in bed on his back, dead. The weight of his head had compressed his airway, and he suffocated. He was aged 28 years old at the time of his death.
Retrospectively, in 1983, Weidemann suggested that the patient reported by Graetz in
1928 could have had Proteus syndrome. [6]and that another case reported by Temtamy and Rogers in 1976 had features consistent with PS [7]
An earnest Search for the phenotypic features compatible with PS
The same year, Weidemann et al described a new syndrome in four unrelated boys with a
constellation of clinical features of partial gigantism of the hands and or the feet, nevi,
hemihypertrophy, subcutaneous tumors, macrocephaly or other skull anomalies, possibly
accelerated growth and visceral affectations which were in keeping with Proteus syndrome. [3]Additional advancements in the comprehension of the
pathomorphology in Proteus syndrome led to relevant updates in 1984.[8]
That same year, Wiedemann and Burgio studied the extent and the nature of the dermal
changes in Proteus syndrome and asserts that they were papillomatous epidermal nevi piquantly aligned along the lines of Baloshka.[9]
Time lines of the Investigations on PS
Several Investigations were undertaken on PS from 1984 to 1990
Further investigations into the pathogenesis and phenotypic expressivity in
Proteus syndrome was undertaken by Lezama and Buyse et al still in 1984[10]
In the same year, Gorlin described another case with features consistent with Proteus
syndrome. [11]
Lipomatosis as an association of PS was highlighted In 1985
Mucke et al et al highlighted the dynamism of the clinical features of the Proteus syndrome by illuminating on the natural history of lipomatosis in I985 [12]The same year, further reports on the multi-focality of Proteus syndrome were made by Costa et al.Two cases were reported, with both having abdomino-pelvic lipomatosis.This was noted in one of the cases aged seven who retrospectively had a conjuctival dermoid at the age of thirty six months.Laparatomy at the age of seventy two months for an acute abdominal pain revealed a right iliac fossa lipomatosis and a twisted necrotic mesenteric fat as the implicated aetiopathogenic factor for the pain.Some morphological similarities of PS to the Bannayan-Zonana syndrome and linear sebaceous nevus syndromes were demonstrated. [13]With further case enrollments into these series in the same year by Costa et al, they asserted the paternal age independence of these cases in a study of ten cases, with a mean paternal age of 30(range 23 to 40) which were not unusually extreme.
April 2014 Supplement 1 Classics and Revisits in Interdisciplinay, Medical Themes.
The alignment of dermopathy of PS along the lines of Baloscka was noted in 1986
Happle pointed out that the lesions follow the dermal lesions along the lines
Baloschka.Happle had proposed that the likely putative aetiopathogenic pathway in PS is
an autosomal dominantly determined genomically transmitted lethal gene persisting by
mosaicism akin to hypothetically proven aetiopathogenic mechanisms propagating the
MCCune-Albright syndrome and probably the Schimmelpenning-Feuerstein-Mims
syndrome [14,15]
Association of PS with adherent lipomatosis and haemangiomas reiterated in 1987
Clark et al reported eleven cases of PS syndrome all with the remarkable
association of lipomatosis. [16] The same year in 1987, a case series was reported in Southern Africa by Viljoen et al [17], in the same year they mentioned that the surgical removal of lymphatic, fatty or hemangiomatous elements could be difficult ,usually resulting in unsightly scars and keloids,at about the same period a further case of a child aged fifty four months who was severely affected by Proteus syndrome was reported by Malamitsi-Puchner et al. [18] Between 1987 and 1988, further works were undertaken retrospectively by Cohen with regards to the Elephant man. [4, 19, 20]
Further aspects of the dermopathies of PS were highlighted in 1988,
Viljoen et al described the skin manifestations of the Proteus syndrome in all of their six patients, with marked hypertrophy of the skin of the soles, being a consistent and universal feature of this syndrome that the authors inferred that it was almost pathognomonic for the syndrome. In three cases, additional features such as large epidermal nevi and linear macular lesions with areas of depigmentations were also demonstrated.
On light microscopy, the affected skins from the soles were noted to have an elongation
of the cytoplasm of the basal cells.
Bannayan-Zonana syndromes suggested as a possible differentials of PS
The differential diagnosis of the Proteus and Bannayan-Zonana syndromes were discussed by Bailer et al in 1988In their examination of the relevant literature they noted a history of consanguinity in 5.56% of their cases with compatible Proteus syndrome.
The overlap within syndromes that include hamartoma as eloquent features intimates that
these syndromes may be aetio-pathologically related, probably with regards to
perturbations in the secretion of a hypothetical humoural growth factor or an irregular tissue or receptor responsiveness to this growth factor.[21]
Malignant transformation of PS lesions exemplified by progressions to testicular malignancy.
Samlaska et al. in 1989 reported an archetypical case and reviewed thirty four others reported sporadically. [22], Malamitsi-Puchner et al in 1990 gave an account of the
follow up of the cases that they studied and reported earlier in 1987.
A striking tissue overgrowth occurred after surgical interventions in this child who latter
developed testicular malignancy. [23]Still in 1990, Beluffi et al reported a case of Proteus syndrome with pelvic lipomatosis and illustrated the use of a computer assisted tomographic scan for demonstrating pelvic lipomatosis.
April 2014 Supplement 1 Classics and Revisits in Scientific InterdisciplinaryThemes The musculoskeletal associations of PS highlighted.
Further investigations in 1990 by Hotamisligil and Ertogan described the case in
a nine month old girl who in addition to other features, had soft tissue masses in the
paravertebral and gluteal areas with aggressive involvement of the spinal canal and a
hyperpigmented epidermal nevus with hyperkeratosis on one side of his body.
He had a bipedal and unilateral left palmar and dorsal macrodactyly with syndactyly of
the third and fourth left digits. In this instance, nephrogenic diabetes insipidus was
described in Proteus syndrome for the first time.[24 ]
Familial transmission of PS demonstrated.
Although traditionally considered a sporadic congenital disorder, some anecdotal reports
have proposed the possibility of a familial transmission in Proteus syndrome.
Goodship, et al in 1991 presented a possible case of father to son transmission of Proteus
syndrome. The son had a cranial hemihypertrophy, a lymphangioma, a lipomata, and
epidermal nevi, where as the father had a large lymphangioma resected from the right
side of his face as a child, given this association, the possibility that his father was a mosaic was proposed. [25]
The possibility of PS presenting with minimal phenotypic expressivity or attenuation highlighted.
Kruger et al in 1993, observed mild Proteus syndrome in a boy whose mother had very
attenuated manifestations of PS. She had facial asymmetry with hypertrophy of her right lower cheek and impaired facial expression in that region. He was not cognitively impaired, but he a had minimally expressed left unilateral superior labial hypertrophy and cheek, causing a perilesionally lateralized impaired focal facial expressivity, hypertrophy of the left arm, partial gigantism of the left middle finger, and a large subcutaneous swelling in the upper left abdomen thought clinically and sonographically to be a lipomata.Both this mother and her son had distinctive superiorly localized venous markings. [26]
The probability of spinal cord compression from related structural defects suggested in PS.
Also Skovby et al in 1993 reported two illustrative cases of the two plausible
pathways by which spinal cord compromise may develop in Proteus syndrome:
In the first instance, spinal stenosis resulted from an anomalous vertebral architectonics
such as angular kyphoscoliosis.In the second case, cord compression resulted from tumoural infiltration most likely froma paraspinal, intrathoracic angiolipomata.[27]
Somatic Mosaism and further associated organic structural defects in PS remarked.
Two unusual cases of PS were further reported by Cohen which supported the concept of
somatic mosiacism.In one case, a huge connective tissue nevus covered the anterior
thoraco-abdominal walls, in addition to multifocal calvaria hyperostoses.
In the other subject, linear verrucous epidermal nevi, epibulbar dermoids, and
hyperostoses were demonstrated. However no appendageal, digital or superficial plantar
enlargements were demonstrated in these instances.In the same year further review of selective aspects of Proteus syndrome, including uncommon neoplasms, pulmonary and renal abnormalities, brain malformations,and patterns of abnormal growth in the craniofacial skeleton were undertaken by Cohen.
April 2014 Supplement 1Classics and Revisits in Scientific Interdisciplinary Medical Themes,
At this point, the presence of external auditory meatal hyperostosis was taken as a very specific feature of Proteus syndrome. [28]
The multifocality of PS lesions demonstrated.
Smeets et al in 1994 reported another patient with a cranio-facial manifestation of
Proteus syndrome. In this case, multi-focal calvarial, facial bony, external auditory
meatal and mandibular hyperostoses were the principal regional features demonstrated.
Also a scleral tumor was demonstrated in this patient. These observations were interpreted as lending a further support to the hypothetical concept of somatic mosaicism. [29]
Further aspects of malignant transformations in PS were reiterated.further
Gordon et al in 1995, observed two patients with Proteus syndrome who progressed to
malignant transformations. One case had a probable mesothelioma, in addition to features
suggestive of thyroid papillary carcinoma, which was not ruled out with
exact certainty pre-mortem, this case latter died at the age of five whilst asleep, at
autopsy, a papillary neoplasm, most likely of mesothelial origin, involving the inferior
surface of the diaphragmatic musculature and infiltrating into the omentum, the pelvic
area, the scrotum and some of the mesenteric lymph nodes were demonstrated.
The other case had bilateral ovarian serous cystadenomas with nuclear atypia identified at six years three months of age, warranting.a right ovarian oophorectomy, invasion of the right fallopian tube was also noted. During this period a catalogue of uncommon neoplasms in Proteus syndrome was compiled and proffered. [30]
Multiple organomegalies and Visceromegalies as an association of PS.
Lacombe and Battin, in 1996 described two unrelated children diagnosed at birth with
isolated macrodactyly .At follow ups they were noted to have developed hemihypertrophy. Three dorsally located angiomas were seen in one of these cases,
a female child, when she was aged four years. The clinical features encountered in both cases were consistent with the specific diagnostic criteria for Proteus syndrome. [31]
Ceelen et al in 1997described a man with Proteus syndrome who sustained an enlarged
splenic rupture while horse riding. [32]
Splenomegaly as a common feature of PS
Biesecker et al in 1998 described their experience with eighteen cases with a
presumptive diagnosis of Proteus syndrome.Splenic hyperplasia was noted to be a recognized feature of PS. The spleen was enlarged in two of the eighteen cases, and another case with asymptomatic splenomegaly and thymic hyperplasia was known to them earlier on. However, they annotated that it was likely that hemi-hyperplasia commonly over simulates Proteus syndrome [33]
In the same year Biesecker et al described a distinctive subset characterized by static or
mildly progressive hemi-hyperplasia and multiple lipomata.
April 2014 Supplement 1 Classics and Revisits in Scientific Interdisciplinary Medical Themes.
A review of the diagnostic criteria for PS
The recommendations for the diagnostic criteria, differential diagnosis, and
guidelines for evaluation of patients with Proteus syndromes which were developed at a
workshop held at the National Institutes of Health in 1998 were reviewed by
Biesecker et al.The general criteria suggested as mandatory for the diagnosis of PS were mosaic distribution of lesions, a progressive course, and a sporadic occurrence.
Pathognomonic Features for the diagnosis of PS suggested.
Specific clinical manifestations also were suggested as necessary to meet the diagnostic
Criteria for PS. Connective tissue nevi, which were common manifestations in Proteus
syndrome, were considered almost pathognomonic for the syndrome, although they were
not always present in all cases, where the diagnosis of PS was made.
Other combinations of manifestations (e.g., epidermal nevus, disproportionate overgrowth, specific tumours were suggested to meet the diagnostic criteria. [34]
Describing other mutational variants of PS
Happle in 1999 suggested the designation elattoproteus syndrome for a disorder that he considered to be an inverse form of Proteus syndrome.
He described a seven year old boy with partial lipohypoplasia and patchy dermal hypoplasia involving large body surface areas. These areas of attenuated growth were similar to those described in many cases of Proteus syndrome. Paradoxically; however, he had a minimally expressed inordinate overgrowth defects.Happle proposed to explain this unusual phenotype by suggesting that there may be various allelic mutations in the putative gene locus responsible for Proteus syndrome, which gives rise to overgrowth of somatic tissues.
Pleioproteus and Ellattoproteus as mutational variants extremes of PS.
Such mutations could be called Pleioproteus alleles, a term derived from the Greek word pleion meaning plus. Conversely, the same gene locus may harbor alleles responsible for deficient growth of somatic tissues. Mutations in these points were referred to as Ellattoproteus alleles after the Greek word elatton, meaning minus.
Patients affected with Proteus syndrome may show classic overgrowth or a composite picture with Pleioproteus and Elattoproteus lesions or even an isolated putative previously uncharacterized elattoproteus phenotype [35]
Ophthalmopathy of PS highlighted.
De Becker in 2000 described the ocular manifestations in a case of Proteus
syndrome and reviewed the ocular features demonstrated in published cases. [36]
Also the same year ,Hodge et al described a ten year old boy with Proteus syndrome
who presented with an effusive pericardial disease, a panhypogammaglobinaemia, with a specific deficiency in IgG and IgA subsets accompanied by low levels of specific antibodies to pneumococcal and hemophilus type B polysaccarides, and global lymphopenia.Given that no other cause was found for this immune deficiency in this case of PS, the authors suggested that it may represent a previously unrecognized feature of Proteus syndrome. [37]
April 2014 Supplement 1 Classics and Revisits in Scientific Interdisciplinary Medical Themes
Cognitive impairments associated with neuronal migration disorders and ophthalmopathies demonstrated in PS
Further work on PS was undertaken by Gilbert-Barnes et al, in 2000, who reported an exceptional patient with Proteus syndrome in whom manifestations included multifocal meningiomas, polymicrogyria, and periventricular heterotopias, with both eyes having epibulbar cystic lesions. The retina showed diffuse disorganization with nodular gliosis, retinal pigmentary abnormalities, chronic papilledema, and optic atrophy. Other abnormalities noted included progressive cranial, mandibular, maxillary, and auditory canal hyperostosis, epidermal nevi, and mental sub-normality. His limbs, hands and feet were noted to be proportionate and otherwise normal. [38]
Deleterious Pulmonary embolism observed in PS
The same year, Slavotinek et al reported three patients with Proteus syndrome who died suddenly from pulmonary embolism related to different clinical circumstances.
The first patient, who was diagnosed with Proteus syndrome at the age of twelve years, had varicose veins, portal vein thrombosis, right iliac fossa vein occlusion and recurrent pulmonary embolism. At the age of twenty five years old he died from pulmonary embolism. The second patient was a nine year old male who collapsed and died at home. Autopsy asserted the cause of death to be pulmonary embolus associated with deep vein thrombosis. The third patient was a seventeen year-old female undergoing inpatient treatment for sinusitis when she suddenly died. Autopsy showed a large pulmonary embolus with no identified deep vein thrombosis.
The need for relevant historical enquiry for PS and evaluation for thrombogenicity to direct anti-thrombogenic prophylactic measures in PS reiterated.
Given the fatality related to these complications, Slavotinek et al promptly suggested that patients undergoing surgical procedures should be evaluated for coagulopathic potential to determine whether antithrombotic prophylaxis will be appropriate for them [39]Cohen in 2001 reviewed at least seventeen reported cases of premature deaths in Proteus syndrome and suggested that patients with this disorder and/or their families should make their health care providers aware of the risk of deep venous thrombosis and pulmonary embolism. [40 ]
Biesecker in the same year reported Proteus syndrome in a five year old case and made a selective literature review.[41 ]
Controversies on the role of PET Gene mutations in PS.
Further work on the molecular genetic aspect of PS was undertaken by Barker et
al in 2001, however they did not identify mutations in the PTEN gene in eight unrelated patients with classic Proteus syndrome, hence suggesting that the mutation exclusion hypothesis may not be unfounded.[42]
April 2014 Supplement 1 Classics and Revisits in Scientific Interdisciplinary Medical Themes
Obstipation demonstrated in PS, a consequence of infiltrative colonic lipomata.
Mackay et al in 2002 reported a twelve year old boy with Proteus syndrome who had presented with gross abdominal distension and severe intractable obstipation.
At an Axial T1- weighted MRI, a diffuse hyperintense tissue, signal typical of fat surrounding and separating bowel loops, with a lesional aspect extending posteriorly on the left into the paraspinal musculature, displacing the psoas muscle anteriorly was
demonstrated. At laparatomy a huge infiltrating lipomatous mass was identified encasing the left colon, including the rectum. [43]
Induced abortion with Misoprostol by a mother related to birth of a baby with PS
Mohameddbhai et al. also in 2002 reported the case of a newborn male Proteus
syndrome case whose mother had ingested misoprostol, an orally active prostaglandins,
at six weeks of gestation in an attempt to abort that conception. [44 ]
Further definitions of diagnostic criteria for PS
Turner et al in 2004 reviewed two hundred and five reported cases of Proteus syndrome.
Interestingly, of all these lot, only ninety seven were thought to have strictly met the diagnostic criteria of PS, eighty cases clearly did not meet the criteria; and although twenty eight cases had features suggestive of Proteus syndrome, there were insufficient clinical data to substantiate this diagnosis.
Follow up adverse events on putative PS cases using a stringent criterion.
On follow up, the reported cases that met the strict criteria for Proteus syndrome had a higher incidence of premature deaths and other complications (Scoliosis, megaspondyly, central nervous system abnormalities, tumors, otolaryngologic complications, pulmonary cystic malformations, dental and ophthalmic complications) compared to those in the non-Proteus group. Cases that met the criteria were more often males, which had implications regarding the hypothesis for the etiology and pathophysiology of Proteus syndrome.Cerebriform connective tissue nevi (skin lesions characterized by deep grooves and gyrations as seen on the surface of the brain, which are striking features when seen on the hands and feet) were considered characteristic.
Specific later onset tumours in PS
Specific tumors occurring before the second decade include ovarian cystadenomas
and parotid monomorphic adenomas. Lung cysts were also added as a criterion.[45]
The Controversies surrounding PTEN in PS clarified.
Thiffault et al in 2004 reiterated that the most plausible suggestion for the genetic
basis of Proteus syndrome is the Happle somatic mosaic hypothesis, although no somatic
mutations in candidate genes had been reported, because germ line mutations in the
PTEN gene had been identified in patients diagnosed with Proteus syndrome.
In the same year, Thiffault et al screened affected and unaffected tissue from six patients
with Proteus syndrome by direct sequencing of genomic DNA for germ line or somatic
mutations in the PTEN or GPC3genes. No intraexonic mutations were identified,
indicating that neither PTEN nor GPC3 was likely to have a major role in the etiology of
Proteus syndrome in this series of cases. [46 ]
April 2014 Classics and Revisits in Scientific Interdisplinary Medical Themes
Post zygotic mutations associated Mosaicism suggested as a causality factor in PS
Brockmann et al in 2008 reported a pair of monozygotic nine year old male twins
discordant for Proteus syndrome. The affected boy showed progressive postnatal
overgrowth of his right lower limb and foot with asymmetric progressive overgrowth of
single toes. There was a small cerebriform connective tissue nevus on his right fourth toe.
The phenotype was mild but still fulfilled the diagnostic criteria for Proteus syndrome.
The findings supported the hypothesis that this condition is caused by a post zygotic
mutation event resulting in mosaicism. [47]
Further discussions on establishing the diagnosis of PS:
Although the exact aetiology of Proteus syndrome is still idiopathic; however the
predominant hypothesis is that Proteus syndrome is a consequence of a post zygotic
mosaic alteration in a gene that is lethal in its non mosaic form, however, Proteus like syndromes were associated with PTEN mutations.
Somatic mutations and defective production or regulation of tissue growth factors or receptors as a causality factor in PS
The concept of a somatic mutation that affects the production or regulation of tissue
growth factors or their receptors could explain the sporadic occurrence, the random
distribution of overgrowth, and the wide range of findings within the phenotype.
Many features of Proteus syndrome overlap with other overgrowth syndromes.
A Proteus like syndrome was previously reported in association with a germ line and
tissue-specific somatic mutations in the PTEN [48, 49, 50] These PTENS genes are usually mutated in Cowden syndrome and in Bannayan-Riley-Ruvalcaba syndrome (BRRS)
Confounding diagnostic difficulties with PS due to other overlapping syndromes.
Some of the phenotypic features of this syndrome overlap with those of the other
overgrowth syndromes such as the Klippel-Trenaunay-Weber syndrome, the
Ollier disease and the Maffucci syndrome occasionally posing some diagnostic
challenges. [51]Although chromosomal alterations have not been demonstrated with routine cytogenetic studies as such in Proteus syndrome, however the karyotype in two patients with Proteus syndrome showed structural abnormalities of chromosome 16 and chromosome 1 in a mosaic distribution, further supporting the hypothesis of somatic mutation. This concept of a somatic mutation that involves tissue growth factors or their receptors could explain several aspects of Proteus syndrome, such as the mosaic distribution of lesions, its sporadic occurrence, the unaffected offspring from the affected individuals, and the existence of discordant twins. Despite the evidence implicating a somatic mutation, no causal gene mutations were known for Proteus syndrome.
However, ongoing advances in the comprehension of the aetiologic-phenotypic
correlations in the context of supposedly genetic defects appear to make the future more
certain. [52]
Striking a balance in the diagnostic criteria for PS.
The most appropriate criteria for the exact diagnosis of Proteus syndrome with much certainty were controversially discussed. Although under diagnosis could be more common, most stringent diagnostic criteria is commonly advocated over simplistic
overlapping generic ones to facilitate appropriate research investigations and utmost
clinical care.The strengths of most stringent criteria are that they define an entity with an
apposite level of congruity in clinical features and outcome which could lead to an
enhanced comprehension of the inherent putative pathogenic mechanisms in these cases [41,33,34 ]
Plausible diagnostic, prophylactic and therapeutic interventions for PS
The aggressive and disproportionate overgrowth in PS is progressive involving mostly the musculo-skeletal systems and these orthopaedic complications could be difficult and challenging to manage. An optimal intervention will imply an adequate knowledge of a spectrum of manifestations and complications of the disorder and a multidisciplinary approach involving the geneticist, surgeons and other specialists.
The diagnosis of Proteus syndrome could be achieved without routine cytogenetic
studies, which have demonstrated inconsistent chromosomal aberrations.
The determination of the molecular aetiology of this syndrome will be difficult, given its
extreme rarity, the abbreviated life span in affected subjects, and its sporadic occurrence,
making the conventional approach of traditional cloning less useful.
Novel molecular genetic approach for the diagnosis of PS.
The aetiology of this disorder could however be studied using various comparative molecular biological techniques such as c DNA arrays, genomic arrays, subtractive techniques, testing of candidate genes and other appropriate techniques.
In addition to testing for dysregulations of growth hormones and binding proteins in vivo.
A novel systematic diagnostic classification scheme for PS
An updated revised criteria was adopted for PS in 2014 [45], differing from that undertaken in 1999. [33] and other more generic less stringent ones which allowed the diagnosis of PS to be made in patients presenting ordinarily with hemi-hyperplasia. [34,53, 54].Its diagnosis requires the presence of all general criteria and various specific criteria, including the presence of the category A criterion or two category B criteria or three category C criteria. The affected subjects should have the following general criteria, mosaic distribution of the lesions, progressive course, and a sporadic occurrence.
The specific PS diagnostic criteria are as follows:
[I] +Category A-Cerebriform connective-tissue nevus.
[II] +Category B-Epidermal nevus; asymmetric, disproportionate overgrowth (limbs,
skull, external auditory meatus, vertebrae, and/or viscera); and specific tumours that
occur most commonly in the second decade (i.e., ovarian cystadenoma, parotid
Monomorphic adenoma)
[III]+Category C-Dysregulated adipose tissue (lipomas, regional lipohypoplasia)
, vascular malformations (capillary, venous, and/or lymphatic), lung cysts, and facial
phenotype..
The argument for a high index of suspicion for the diagnosis of PS
The variably mild form of this syndrome with a less eloquent phenotypic expressivity could be missed initially or entirely until catastrophic complication of DVT or PE renders it suspicious and the diagnosis now made in retrospect. Neuroimaging with Skull X-ray, CT or MRI will be most informative for those with significant facial dysmorphism, mental subnormality or epileptogenesis.Compressive neuropathies could follow tumourigenesis or bony exostosis.
April 2014 Supplement 1Classics and Revisits in Scientific Interdisciplinary Medical Themes
PS is a progressive and dynamic overgrowth condition.
Occasionally, the mild or moderate effects of Proteus syndrome, such as bening tumours, are present at birth. As the child grows and develops, the tissue overgrowth progresses and changes. This progression is often irregular, it is characterized by periods of major overgrowths and other periods of absent overgrowth.The effects therefore change over the course of a time.
PS is associated with ambulatory difficulties and compressive neuropathies.
However, most changes occur before adolescence, since tissue over-growths tend to plateau at that time.Patients with Proteus syndrome have difficulty ambulating because of toe macrodactyly, scoliosis, and joint instability, with frequent dislocations, expansive subcutaneous tumours and compressive neuropathies due to intraneural hamartomas.
Some patients may have persistent atelectasis, pneumonia, or symptoms of pulmonary
Strict diagnostic criteria, guidelines and algorithms has been proposed and accepted. Those features which are considered to have diagnostic values are considered because of the tremendous variability in the manifestations of Proteus syndrome.
These features could be classified as cutaneous or noncutaneous.
Cutaneous manifestations
Almost always, all patients with Proteus syndrome have at least one type of cutaneous
lesion, of which several different types have been reported. These skin lesions could
generally be classified into two groups, those that are congenital or neonatal in onset and are stable in the first group and those that are post neonatal and progressive in the second
group. In some patients, the presenting symptom may be one or more of the many types
of heterogeneous skin lesions that characterize Proteus syndrome.
These include cerebriform connective –tissue nevi, epidermal nevi, vascular hamartomas, lipomatas and hyperpigmented or hypopigmented areas. Epidermal nevi and vascular malformations are considered group one lesions, while lipomatas and cerebriform connective tissue nevi generally occur latter and are considered group two lesions.
Group 1 cutaneous lesions are characterized as follows:
Epidermal nevi are a common finding in patients with Proteus syndrome.
These manifest at birth as tan-to-brown, flat topped, hyperkeratotic or verrucous papules,
which run in a linear or whorled pattern along the lines of Blaschko.
Epidermal nevi are found asymmetrically, scattered around the body.
Histologic features are acantholysis and hyperkeratosis in a clinical lesion consistent with an epidermal nevus.
Congenital Vascular malformations are other common cutaneous features in PS.
These anomalies can be of venous, capillary, or lymphatic origin and include nevus
flammeus, angiokeratomas, cavernous hemangiomas, superficial and deep lymphangiomas, and varicosity of the superficial veins. [55, 56]
These lesions are developmental abnormalities and they grow proportionately through a
patient’s lifetime without regression .In an occasional patient these vascular malformations have been noted to expand beyond proportionate growth.
Prominence of veins may be enhanced in areas of regional lipodystrophy.
April 2014 Supplement 1 Classics and Revisits in Scientific Interdisciplinary Medical Themes.
Group Two cutaneous lesions are characterized by the presence of cerebriform nevi
Cerebriform connective-tissue nevus is one of the most common and characteristic
features of Proteus syndrome, although its presence is not required for diagnosis and its
presence alone is not pathognomonic for Proteus syndrome, although its presence is not
required for diagnosis and its presence alone is not pathognomonic for Proteus syndrome.
An occasional patient developed an isolated plantar cerebriform collegenoma who lacked the general diagnostic criteria for Proteus syndrome. These nevi have a relatively delayed onset, making the diagnosis of PS difficult in neonates and infants.
Cerebriform nevi occur as well-demarcated, skin coloured plaques with a cerebriform or
rugated appearance. Most often they occur in the cutaneous and subcutaneous tissues on
the palms or soles but have also been noted on the forearm, on the trunk, and inferior to the ala nasi.Lesions on the sole typically cause the most morbidity because they cause ambulation difficulties, are usually prone to ulceration and infection, and are malodorous.Histopathologically, the lesions consist of an irregular whorly non-lipomatous proliferation of highly collagenised cellular fibrous tissue.
Lipolymphohaemangiomas are characteristic features in PS
In PS adipose tissue abnormalities are common, resulting in lipomatas and areas of lipohypoplasia.Lipomatas occur as hamartomatous masses consisting of subcutaneous
tissue or a variable combination of adipose, lymphatic, and hemangiomatous components
(eg, lipolymphohaemagiomas) and are the second most frequent type of skin findings that
characterize Proteus syndrome. The soft subcutaneous tumours can affect any area of the
body but are most common on the head, abdomen, groin, or legs. They appear as soft,
skin coloured nodules or tumours.Patchy areas of lipohypoplasia or dermal hypoplasia
are also observed in some patients.Lipohypoplasia occurs as regions of skin with minimal
fat, while dermal hypoplasia appears depressed, red plaques in areas with prominent
Other non-lipomatous growths in PS
Cutaneous manifestations also include striking overgrowth anomalies, including
asymmetric hypertrophy of the face, part or all of one or both limbs (particularly
digits), the trunk, or any combination of these; often, hemihypertrophy of one side of the
body results. These abnormalities could also be the first to attract the parent’s attention
and cause considerable anxiety.Cafe au lait spots and areas of hypopigmentation or
hyperpigmentation with a linear or whorled arrangement are also hallmark skin findings
in persons with Proteus syndrome.Hypertrichosis and nail abnormalities are also seen.
Non-cutaneous manifestations in Proteus syndrome:
The most characteristic non-cutaneous findings in patients with Proteus syndrome involve skeletal overgrowth and include corporal hemihypertrophy, partial gigantism of the hands and or the feet, and skeletal anomalies such as long-bone overgrowth and scoliosis. Overgrowths of the hands, feet, or both were universal in one series of twenty four patients with Proteus syndrome. Arm or leg over growth is nearly always a constant feature of PS.
April 2014 Supplement 1 Classics and Revisits in Scientific Interdisplinary Medical Themes,
Cranio-facial abnormalities
These are fairly common and progressive and include cranial hemihyperplasia, hyperostosis of the skull or external auditory canal, craniosynostosis,
and unilateral condylar hyperplasia. Hyperostosis and unilateral condylar hyperplasia
were common, while cranial hemihyperplasia and craniosynostosis were rare.
A facial phenotype with dolichocephaly, long face, down-slanting palpebral fissures,
ptosis, low nasal bridge, wide or anteverted nares, and open mouth at rest has been
associated with mental retardation and seizures.
The other commonly associated neoplasms in Proteus syndrome
Specific tumours have been noted to occur with an increased frequency in patients with Proteus syndrome. In addition to being found subcutaneously lipomata could also infiltrate muscles and internal organs, including the heart, pancreas, spinal cord, and the pharynx. Other tumours noted to occur in several patients include ovarian cystadenomas, testicular tumours, and parotid adenomas. Other benign neoplasms, such as bronchial hamartomas and multiple meningiomas have been reported. Cystic lung disease is found in approximately 1:10 of patients with Proteus syndrome and could lead to a significant morbidity, with rapid progression, fatality could result. Renal cysts in PS have been previously described. Visceral overgrowth was reported previously, especially involving the spleen or the thymus. Internal vascular malformations of the gastrointestinal tracts, spleen, kidneys, and the testicles have been reported previously.Other occasional reports of intravesicular venous lesions associated with life threatening haematuria was previously proffered.
Other ophthalmopathies in PS
Many ocular manifestations were previously reported in patients with Proteus syndrome. The most commonly recorded of these ocular involvements included strabismus, nystagmus, and epibulbar tumours.Other ocular involvements include an over representation of a high degree of myopia, retinal pigmentary abnormalities, retinal detachment, cataracts, posterior segment hamartomas, retinal colobomas, heterochromia irides, and glaucoma.
Associated myopathies, cardiopathies and Immuno-deficiency states in PS
Other additional features in PS include asymmetric myopathies, congenital cardiac defects, malocclusions, hypodontias, and hypoplastic enamels. A congenital immunodeficiency of some sort was previously reported in a case of Proteus syndrome.
HHML is a differential of PS with much clinical consequence.
A very important differential diagnosis to be considered in these is hemihyperplasia
and multiple lipomatosis syndrome (HHML), because of the higher association with
nephroblastoma (Wilms tumours and possibly hepatoblastomas) in HHML.
The diagnosis of HHML with certainty will have implications for apposite screening of these patients for malignancies using standard screening protocols. [57]
April 2014 Supplement 1 Classics and Revisits in Scientific Interdisciplinary Medical Themes.
Early prophylactic surgical management are more appropriate interventions.in PS
The management of PS has been an inescapable but crucial medical dilemma,
especially with regards to the prolonged multiple staged orthopaedic interventions.
The rapidly progressive overgrowths are quite cosmetically disfiguring, unsightly and
probably mechanically and functionally incapacitating, seldomly or occasionally causing
compressive aerodynamically significant airway defects or neuropathies and rarely
malignant transformations. Despite several previous descriptions of early, intermediate or
delayed modified surgical interventions for these defects, overall short, medium or long
terms outcomes for these defects appear to be discouraging and unrewarding.
However, the evidence for a primordial intervention before the onset of catastrophic or
debilitating complications appears more convincing and satisfactory.
Interventions for PE in PS
The other factors of interest in the management of PS are the continual concerns with
deep vein thrombosis related thrombo-embolic phenomenology especially with regards to
its frequent occurrence peri or post operatively. The importance, strengths and the morbidity and mortality implications of these associations raised and buttressed the
argument for routine pre or perioperative anticoagulant prophylaxis and monitoring
of PS cases. However, this practice is more or less anecdotal and could occasionally be a
complex decision in the context of a sequence with a supposedly universal vascular
anomalies, real risk of bleeding especially in the association of Marriet Kaselbach
syndrome which is a potential complication of platelet consumption by the meshwork of
the vascular malformations.
Screening for Neoplastic conditions as plausible prophylactic strategy for PS
The other area worthy of adequate attention and consideration in the overall management of PS patients which were contentiously reviewed and addressed in the literature are those dealing with the most safe, cost effective and clinically useful way of detecting the tumourous events in PS early enough for appropriate interventions and outcomes to be most therauptically and prognostically rewarding. However, this decision is compounded by the fact that the list of plausible neoplasms which could complicate a case of PS are quite diverse and there appears to be no robust figures to suggest that earlier detection of these tumours or interventions by any means is actually rewarding.As such the time for active surveillance of these tumours should be weighed in favour of clinically and symptomatically suspected cases as against a routine procedure.
Genetic Counseling
Also issues concerning the genetic counseling of PS patients seem anecdotally based on
the mutational, nonmosaic lethality principle which infers a low recurrence risk for PS.
Finally, the physician investigators concerns of the most appropriate way of PS gene
mapping, identification and elaboration appears to be future challenges given its sporadic
Conclusions:
For a long time in the medical literature, issues concerning the exact diagnostic criteria of
PS in suspected cases have been topics of much debate, were controversially discussed,
inconclusive and not uniformly accepted. Although recently, these criteria were consensusly fairly well elaborated on, with some convincing recommendations derived. However, it is likely that these criteria will need to be modified from time to time as it has always been, to circumvent underdiagnosis or over diagnosis, the extensiveness and expressivity of the increasing number of overlapping syndromes make these diagnostic challenges likely, especially with further work in these areas or longer term follow up of putative PS probands and their families.
Relevance and Importance.
These editorial review discusses an unusual hitherto previously unaddressed co-existence and associations of embroyomas in PS.The diversity in its phenotypic expressivities and the variabilities in the chronology and temporality of their manifestations were reiterated.The rarity and strengths of its associated features as against its differentials especially hemihyperplasia and multiple lipomatosis syndrome (HHML) which is associated with embryomas were examined.[58] The other available literature on the other putative tentatively peculiar overlapping syndromes could be reviewed as a topic for debate in the near future.
Guest Editors: CRS [Med] Consulting Academic Clinical Editorial Experts.
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[47]-Brockmann, L.G.; Happle,R.;Oeffner,F;,Konig,A.: Monozygotic twins discordant for Proteus syndrome.Am.J.Med.Genet.146A:2122-2125,2008.
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Brereton,J.J; Marsh, D.J.Germline mutation of the tumour suppressor PTEN in Proteus syndrome. (Letter)J.Med.Genet.39:937-940,2002.
[49]-Zhou,X;Hampel H;Thiele,H;Gorlin,R.J;Hennekam,R.C;Parisi,M;Winter R.M;Eng,C.:Association of germline mutation in the PTEN tumour suppressor gene and Proteus and Proteus-like syndromes. Lancet 358:210-211, 2001
[50]-Zhou, X.-P.;Marsh,D.J.;Hampel,H.;Mulliken,J.B,;Gimm,O.;Eng,C,:Germline and germline mosaic PTEN mutations associated with a Proteus-like syndrome of hemihypertrophy,lower limb asymmetry,arteriovenous malformations and lipomatosis.Hum.Molec.Gent.9:765-768,2000
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April 2014 Supplement 1 Classics and Revisits in Scientific Interdisciplinary Medical Themes.
Cervical haemangiomas with intracerebral extensions in childhood.
Guest Editors: CRS [Med] Consulting Academic Clinical Editorial Experts.
Background and Purpose
The need for a multidisciplinary intervention in most cases in clinical medicine is further reiterated by another editorial annotation and perspective from the CRSM consulting guest editorial experts by cases of exophytic cervical haemangiomas with intracerebral extensions observed in some young children,
Editorial Annotation and perspective.
In this correspondence, unusual presentations of congenital Cervical Haemangioma with intracerebral corticomedullary extension, with acute respiratory distress, hypochromia and thrombocytopaenia in children is discussed. In such instances, early and timely interventions will have positive implications for guarding against occult Marriet-Kaselbach syndrome, high output cardio-respiratory decompensation, neurologically significant and multiple vital organic compressive-obstructive effects. In these circumstances, application of intralesional corticosteroids appears to have arrested the progressive course of these lesions, until interval extramural instrumentations were achievable.
Introduction
The descriptive epidemiology of Haemangiomas intimates that they are relatively common childhood tumours occurring in 1:10 to 1:20 infant’s .Female gender and extreme prematurity appears to be significant features of its epidemiologic associatiions.They are usually localized to the cutaneous or subcutaneous tissues. Although almost universally congenital, they are rarely fully developed at the time of birth. About 2 in 3 cases of haemangiomas are localized to the head and neck region. Most haemangiomas are present or appear during the neonatal period as soft rapidly growing solitary unifocal lesions or occasionally multifocal lesions.
Complications of Haemangiomas.
In haemangiomas rapid growth over six to nine months may occur to produce severe cosmetic disability, orificial obstructions, threatened viability of vital structures such as the eyes and multiple ulcerations. In less than 1: 10 cases, rapidly growing hemangiomas of the head and neck region could produce catastrophic complications, with aero-digestive tract obstructions especially following a sudden increase in size related to haemorrhages, causing visual, auditory, deglutinatory and vocal difficulties. Also the tumefactions could get secondarily infected through the ulceration of the overlying skins .If an arteriovenous malformative communications of a considerable capacity with a haemodynamic significance or consequence develops an intractable high output congestive cardiac failure could follow.
April 2014 Supplement 1 Classics and Revisits in Scientific Interdisciplinary Medical Themes.
Other deleterious consequences of Haemangiomas.
There are other several complications of great concern such as the Kasabach-Merrit syndrome. In this complication, an inordinate bleeding diathesis and purpura may result from platelet consumption, DIC, microangiopathic haemolytic anaemia.
Previously, internal organs have been reported to be occasionally involved, in
haemangiomas, however, intracerebral involvement in haemangiomas are rather an
unusual and rare phenomenon.
Thrombocytopenia, thrombocytosis, respiratory distress and occult bleeding diathesis from coagulapathy and thrombocytopaenia and severe recurrent iron deficiency anaemia appears to be real major concerns with significant lesions.
Associated naevus may initially be considered to be inconsequential but could occasionally pose cosmetic and aesthetic difficulties with the passage of time.
The natural history of haemangiomas and its simulating lymphogiomas
The natural history of haemangiomas peculiarly involves an initial proliferative growth
phase that lasts about half to one year and an involutional phase which is associated with
a slow regression of the haemagiomas, with about one half of the lesions resolving by sixty months and about 9/10th resolving by the age of ten years. A very important differential to consider in this case is a lymphangiomatous lymphatic malformations which could be unifocal or multifocal and commonly occurs in the facio-cervical region, axilla or thoracic, they are similar to haemangiomas in that they appear in infancy and childhood usually before thirty six months. However, they differ from them by the lack of spontaneous involution. They are equally obstructive and haemmorragic tendencies.
These lesions are quite extensive and diffuse making a stage evaluation for the exact
determination of the extent of the lesion and appropriate stage dissections imperative for
an utmost outcome.
Management
Intralesional steroids appeared to have arrested the progression of these lesions in some instances.In occasional circumstances however, management could be expectant, given that spontaneous resolutions has been known to occur. By the age of ten years 9 out of 10 cases would have resolved completely.
Systemic steroid applications would have been indicated otherwise if there were concerns with ocular integrity or ongoing concerns with airway obstructions, difficulties with feeding or defecation or in the Kasabach-Merrit syndrome
Although some recalcitrant cases may not be very steroid or radio therapeutically responsive. Administration of interferons appears to be very useful in catastrophic situations. Also Laser coagulations or sclerotherapy could be useful in haemangiomas but not usually in Lymphangiomas..Other agents like OTK 145 have been employed in selected cases with significant beneficial effects. In Kasabach-Merritt syndrome, treatment is supportive, platelets, cryoprecipitate and fresh frozen plasma, heparin and antifibrinolytic agents such as aminocaproic acid and traxenamic acid may be used.
In other instances, life threatening compressive effects such as severe respiratory distress or rising intracranial pressure with cardiopulmonary instability and intrabdominal hypertensive events has made urgent extramural instrumentations imperative.
Guest Editors: CRS [Med] Consulting Academic Clinical Editorial Experts.
April 2014 Supplement 1 Classics and Revisits in Scientific Haematology.
Fanconi anaemias in Childhood.
Guest Editors: CRS [Med] Consulting Academic Clinical Editorial Experts.
Background and Context:
Fanconi Anaemia-FA is a severe debilitating disease associated with a compromised life expectancy. Specific review on FA is crucial. This expert guest editorial review article by the CRSM academic clinical consulting editors is based on observational reports on a spectrum of compatible childhood symptomatic characteristic Fanconi akylating aplastic anaemias.
In the various subsets, overall aggregate annotations suggest that growth failures were universal, whereas dysmorphologies such as microcephaly, polydactyly, hypopigmented dermatoses were common associations.Rhizomelic short limbed dwarfism, hypodontias and caput quadratum were occasionally encounted defects. Bleeding diathesis, febrile neutropaenias, and pallor were predominantly featured phenomena.
Another notable feature of interest is that in individual cases, recurrent symptomatologies were invariable and somewhat predictable.
Multiple blood transfusions, antimicrobials, steroids. , testosterone, acyclovir and fluconazole were the most commonly applied interventions which lead to a partial remission in some cases.. Doubtful malignant transformations and acute catstrophic bone marrow failures were mutually exclusive events. Unprecedented demise was a notable phenomenon. Occasionally, recovery from FA could be spontaneous.
Case Definitions..
Fanconi aplastic anaemia is an inherited autosomal recessively transmitted, genomically determined instability syndrome, associated with multiple developmental structural and functional abnormalities, progressive bone marrow failure, leading to pancytopaenia and the requirement for haematopoietic bone marrow stem cell transplantation or increased morbidity or mortality as a result of defective haemopoesis and malignant predisposition to earlier onset acute myeloid leukaemias and latter onset solid stromal tumours. [1, 2]In Fanconi anaemia, cellular chromosomal instability, breakage and hypersensitivity to bifunctional DNA cross-linking agents especially mitomycin C is usually demonstrated, which usually forms one of the basis for its diagnostic criteria. [3]
Complementation somatic-cell hybridization genomic analysis in FA
For a long time, the relationship between congenital malformations, bone marrow
failures, cancers, chromosomal breakages and their derived complementation groups in
April 2014 Supplement 1 Classics and Revisits in Scientific Haematology
Fanconi anaemia syndromes were poorly understood. [4 ]
The principal pathophysiology in Fanconi anaemias, the most common of the familial congenital constitutional aplastic anaemias have been fairly well elucidated through major advances in complementation somatic-cell hybridization genomic analysis of FA cell lines which demonstrated and provided evidence for alphabetically designated complementation groups (FA-A, FA- B, FA-C, FA-D1, FA-D2, FA-E, FA-F, FA-G, FA-I, FA-J, FA-L) with implications for abnormal haemopoiesis and apoptosis in their mutant states especially for the FA-C [2] in addition to linkage to a specific pathognomonically genomic disease entity.
The distribution of the complementation subgroup subtypes.
The distribution of the complementation subgroup subtypes of FA is geographically distinct, in some regions the FA-A was the most frequently represented at 59%, where as in the other, FA-C was the predominantly featured subtype at 67%.
This distributional in homogeneity could have implications for mutation-screening
strategies in continental FA patients. [5]
The extensive genetic heterogeneity in FA and its influence on linkage analysis.
The implications of this extensive genetic heterogeneity for classical linkage analysis were further discussed in previous investigations. [6, 7, 8] however, a good part of its pathophysiology remains elusive.
Although it is a genetically inherited form of bone marrow failure syndrome, its presentation may not necessarily be congenital [9, 10], until the abnormal immune
responses implicated in its pathogenesis is triggered by genomic instability or by
environmental exposures to chemicals, drugs or viral infections or perhaps by
endogenously generated altered bone marrow cells, which then heralds its more florid
The molecular and geographic epidemiology of FA.
The pathobiology, molecular and geographical epidemiology with the relevant therapeutic options in Fanconi aplastic anaemia syndromes have been previously investigated, described, replicated and revisited in several settings [11,12,13,14] Previous occasional case studies and series suggested hospital and community based high incidence rates, however overall the disease prevalence was relatively low, such that longitudinal single city epidemiological studies were not logistically feasible, however more recent investigations achieved higher sample sizes but most of those were in adults.[15]Overall, FA is a relatively uncommon disease with an estimated prevalence of 1 to 5 per million and an approximated carrier frequency of 1 in 200 to 1 in 300 in most population studies [16] Although its diagnosis is probably underestimated, because its haematological features may be moderately expressed or they could present latter.
The influence of the Founder effect on the incidence of FA
Of growing interest in the elucidation of the drive behind the unusual preponderance of FA in some ethnic groups compared to others is the concept of founder effect associated with genetic and reproductive isolation. This phenomenon implies an increased risk of inheriting rare genetic disorders such as FA following the development of a high frequency of a mutant allele introduced into communities which are genetically and reproductively isolated and therefore consanguineously and endogamously married .
The carrier frequency in this subset was estimated to be 1 in 90 to 1 in 92[17,18]
Other previously described epitomes of the phenomenon of founder effect were demonstrated for a subset , where the carrier frequency was estimated to be about 1 in 77 [19,20]
Further recent investigations demonstrated the inordinately clustered overrepresentation
of the incidence of FA in the subjects of particular ancestry from heterogeneously
distinct descent, abode and ethnicity. However, the figures from those of subsets
with homogenous ancestries mostly of the complementation group A subset were
astonishingly extreme, probably the most globally at 1 in 64 to 1 in 70[21, 22]
Haplotype linkage disequilibrium as a causality factor in FA.
Other prominent clusters were demonstrated elsewhere, where the populace in
genetic isolation revealed clinical characteristics of the patients showing both intra-and
interfamilial heterogeneity suggestive of haplotype linkage disequilibrium for markers
flanking the FAA gene. This hypothesis may have implications for positional
cloning of this gene through haplotype disequilibrium mapping. [23]
The case for further investigations in FA
In most circumstances however, accurate estimates of childhood characteristic
Aplastic anaemia (Fanconi subset) Fanconi anaemia, its clinical profile, peculiarities, heterogeneity, complexities and the intricacies in its diagnosis and the feasible therapeutic options have not been previously analyzed, revisited or completely understood.
Reliable inference or reference review on Characteristic Fanconi akylating aplastic anaemia syndromes are crucial for interventional planning, hypothesis generation, research directions and focused hospital or population based interventions.
It could also offer some contribution to a better definition of its natural history. Furthermore, this annotation offers the opportunity to highlight and briefly discuss some of the commonly associated structural and functional anomalies in FA as was previously proposed [24] , given the relevance of a more lucid definition of these commonly associated anomalies in expediently advancing a rational prognostic scoring index which predicts the likelihood of progression to catastrophic bone marrow failures or malignant transformations as was determined previously[2,25,26] This editorial review article is a step to describe and characterize some of these aspects.
Case definitions, Observations and follow up of compatible FA cases.
On observation and follow ups, suspected cases with the diagnosis of compatible Characteristic Fanconi akylating aplastic anaemia syndromes would have achieved the clinical, radiological and pathological inclusion criteria of FA for descriptors, clinical features, predominant symptomatologies, laboratory features as was previously described by Camitta: [27]
With severe disease defined as the presence of two of three blood component counts
criteria of an absolute leukocyte count of<0.5×10 (9), platelet count of <20×10 (9) and a
reticulocyte count of < 1%.Extreme neutropenia (<0.2×10 9/L) defined very severe
aplastic anaemia, the other cases could be described as moderately severe aplastic
anaemia.The bone marrow biopsy had to be compatible with the diagnosis of aplastic
anaemia.Cases had to be analyzed for demographics, historical and medical information, especially with relevance to clinical features, recent and previous drug use and related infective episodes.
Clinico-Epidemiological diagnosis in FA
Further basic epidemiologic features of Fanconi aplastic anaemia bone marrow failure syndromes could be achieved by characterizing cases in terms of demographic variables, age, sex, medical history, clinically significant manifestation, proof of diagnosis, and prognostic criteria. Associated dysmorphologies were evaluated, studied, validated, analyzed and reported in a standardized manner using standard normograms from the dysmorphology databases.
Clinical features and associations of FA
Overall, presentations in FA were associated with the male factor, consanguinity, latter presentation,non-familial incidence,infective episodes, unusual cranio-facial features or inconsistent physical characteristics Bleeding diathesis especially with epistaxis was a common but less florid mode of presentation, where as massive haemoptysis.frank pulmonary haemorrhages and haematemesis,where less frequent, but clinically more alarming and deleterious.Lost cases were mostly related to massive haemorrhages or overwhelming infective events related to febrile neutropaenias.
Outcome of observed FA cases..
The presence of at least one associated anomalous dysmorphological defect is quite usual.Rhizomelic short limbed dwarfisms with delicate statute, microcephaly
pre-axial and post axial polydactyl, caput quadratum, absent radii,
hypodontias and abnormal gingival morphology were the most predominant dysmorphological phenomena. Abnormal pigmentation of the hair, the skin and the mucous membranes especially the lips were the most common intergumentary defect noted.
April 2014 Supplement 1 Classics and Revisits in Scientific Haematology
The diagnostic and the therapeutic aspects of FA.
For the suspicion of Characteristic Fanconi anaemia to be evoked, the blood film and bone marrow morphologies has to be compatible with that of bone marrow failure syndromes.
Undertaking radiological evaluations would be expedient to exclude respiratory tract infections or complicated organizing pneumonias or features suggestive of pulmonary infarctions or haemorrhage, it may equally exclude or suggest erythrophagocytic lympho-histiocytosis-X rather than FA as the plausible diagnosis to be pursued.
X-ray radiological surveys could be worthwhile from the context of suggesting absent radii. Or other more covert musculoskeletal defects.
Transthoracic and abdominal ultrasonic echographies or tomograms could be useful to exclude the possibility of other linked congenital visceral abnormalities.
Routine cultures and biochemical studies would have positive prophylactic, therapeutic and prognostic implications for those with persistent febrile episodes despite applications of broad spectrum and specific antimicrobial. Most patients would be hospitalized at a point, and most of the hospitalizations will be associated with whole blood transfusions, and most cases could be managed conservatively at the first instance.
The historical perspective of FA.
Fanconi aplastic anaemia syndrome, a form of Constitutional aplastic anaemia was
earlier described by Erlich towards the end of the 19th century. [28]
Constitutional aplastic anaemia could be associated with congenital dysmorphology
(Fanconi subset) or it may not be associated with it. In 1919 Smith reported aplastic
anaemia in a six year old boy associated with marked nuchal, genital, umbilical and areolar skin pigmentation. [29] Most cases of constitutional aplastic anaemias were usually autosomal-recessively transmitted, although an occasional case report suggested the possibility of an autosomal familial dominant inheritance pattern [30]
Fanconi in 1927 described three brothers aged five, six and seven with hyperchromic
anaemia, leucopaenia, thrombocytopaenia, haemmorragic diathesis, microcephaly, testicular hypoplasia, convergent strabismus, exaggerated deep tendon reflexes and generalized brown, melanin like pigmentation of the skin but not of the buccal mucosa. [31]
The Haemopoetic stem cell line defect in FA and its therapeutic implications.
Fanconi anaemia bone marrow syndromes usually arise as a result of damage to precursor cells so that they cannot supply an adequate quantity of haemopoetic cells .This damage could also be at the bone marrow stromal level (i.e.the bone marrow microenvironment) which is now regarded to be equally important in the pathogenesis of aplastic anaemia.The spectrum of clinical and laboratory features are implicit on the stage of the haemopoiesis where the maturational sequence was disrupted. Earlier damage will result in uniform pancytopaenia, whereas latter disruptions after haemopoetic progenitor cells differentiation will result in predominantly red blood cell aplasia, agranulocytosis or thrombocytopenia. These differential interruptions may have implications for variabilties in its symptomatologies, which if predictably consistent could be exploited for a
more focused and targeted blood component and adjunctive supportive therapeutic interventions with overall positive outcome, toxicity and costs implications.
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Irradiation and toxicity induced perturbations implicated as causality factors in FA
The association of the onset of the symptomatology of Fanconi aplastic anaemia with several putative inciting agents such as viral infections, drugs, toxic agents, irradiation,
autoimmune diseases and idiopathic- cryptogenic factors, intimated the probability of
several mechanisms for the non-specific triggers and amplifications of the disease
processes. [32] The effect of irradiation on the cells of characteristic Fanconi akylating aplastic anaemias were controversially discussed, although the results suggested that
there were no increases in radio-sensitivity to all cell types from characteristic Fanconis
akylating aplastic anaemia patients, however an apparent increase in chromosomal
radiosensitivity may be seen in the lymphocytes from an occasional case. [33]
Defects of Haemopoetic stem cell line and immonopathology suggested as FA causality factors.
Previous investigations proposed several hypotheses for the pathophysiology of Fanconi aplastic anaemias, which were derived from the results obtained after several systematic laboratory investigations. These results suggested the convincing evidence of the efficacies of several therapeutic options which linked it to some plausible aetiological mechanisms. The successful use of bone marrow transplantation to cure Fanconi aplastic anaemia implicated a stem cell deficiency, where as the significant response to immunomodulant therapies pointed more to an immune mechanism of haematopoietic
Clonality inconsistencies suggested as a possible causality factor for
FA and other associated diseases haematological dyscrasias.
The relationship of the Fanconi aplastic anaemia syndrome to other haematological dyscrasias associated with clonality has been suggested, such haemato-pathologies include large granular lymphocytosis, and single haematopoietic lineage deficiency states such as agranulocytosis, pure red cell aplasia and amegakaryocytic thrombocytopenia. [34] Other closely related entities to FA are Dyskeratosis congenita and other forms of Idiopathic cryptogenic unclassifiable forms of constitutional aplastic anaemias, like FA they are associated with a high incidence of haemopoetic clonal disorders, single-or multiple lineage cytopaenias, severe aplastic anaemia, myelodysplasia, and malignant transformation, identification of these associated mutant genes in such pathologies will have implications for elucidating the exact pathophysiology involved, and proffer relevance for deducing apposite novel therapeutic options. [35, 36, 37]
Chromosomal breakage points on peripheral lymphocytes may suggest the diagnosis of FA.
The diagnosis of Fanconi aplastic anaemias could be inferred from chromosomal breaks observed in peripheral blood lymphocytes or dermal fibroblasts exposed to mitomycin in the appropriate clinical and haemato-pathological setting.Fanconi anaemia cells will normally show excessive chromosomal breaks. [38, 39] In this way some young adults have been diagnosed to have Fanconi anaemia.
Myodysplastic syndromes and paroxysmal nocturnal haemoglobinuria as FA differentials
Some less common congenital marrow failures syndromes, without specific markers,
could be more difficult to exclude.Myelodysplastic syndromes could be ruled out by
appropriate marrow haemato-pathologic and cytogenetic analysis.The distinction
between Fanconi anaemia, Myelodysplasia and other forms of bone marrow aplastic
anaemia is critical, because therapeutic options differ. [40, 41] Also, there are considerable overlaps between the pathogenesis, laboratory features and therapeutic options for the syndromes of paroxysmal nocturnal haemoglobinuria and Fanconi aplastic anaemia because many patients with FA bone marrow failure syndromes
have an increased population of abnormal cells [42]
Although FA is congenitally acquired, its onset is delayed till early childhood.
Interestingly, the onset of the aplastic anaemia is usually between the ages of four and seven years in boys and six and ten years in girls, however it may occur at an earlier age.
Results proffered by one investigation noted a male preponderance and a younger age at onset [43]
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The cryptic onset of the clinical features of FA in some instances and its diagnostic implications.
The onset of FA could be cryptic with pallor, skin bruises and petechiae.Easy fatigability
and anorexia then follows, with progression of the disease, bleeding could occur from
almost any site and a sore throat with ulceration of the mucosa due to leucopenia ensues.
Physical examination is more informative for the associated defects, the usual
pallor and occasionally for the related cutaneous bleeding diathesis. Also FA could
present in a more occult way as bone marrow hypoplasia or aplasia following further bone marrow studies of an incidentally or co-incidentally discovered abnormal blood film
morphology. Multiple sites aspirates and a trephine will be relevant for making a more
substantive diagnosis in indeterminate cases.
The therapeutic aspects of FA.
Whole blood, blood component therapy with platelets and chemoprophylaxis will be
required until there is a compatible bone marrow donor.Allogeneic bone marrow stem
cell transplant in children is a very rewarding intervention. However, this may not always
be achievable in all cases due to lack of a suitable donor. In such cases
immunomodulation and antithymocyte globulin therapy could be considered in the interim. [44]However, there are real concerns of longer term complications of hypocellular myelodysplasia compounding its diagnosis and transformation to acute leukaemias in longer term survivors even after bone marrow transplantation. [45]
The variations in the clinical course of Fanconi anaemia and the response to
immunosuppressive treatment could be explained by variations in the balance between
its primary defect and the secondary immune responsiveness, the co-involvement of other
accessory cells in the primary disease, the relative time course of the two components and
the efficiency of the repair mechanisms. [32]
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A rational and conservative approach to circumvent some complexities associated with some optimal interventions for FA
The inherent complexities of these interventions for FA imply that they will not always be rapidly achievable, in most instances or circumstances.
However, since previous investigations intimated that symptomatologies appear when the stem cell and progenitor cell populations are less than one percent, given the inherent high marrow reserve potential for haemopoiesis.There fore, this positive feature of an immense marrow reserve capacity for haemopoiesis could be exploited in more selective , specific and focused haematopoietic cell line conservative supportive interventions with colony stimulating factors and/or defined blood component therapies, which offers initial interim tentative options for well selected cases in restricted circumstances, while arrangements for an appropriate compatible umbilical or bone marrow stem cell donor are being made, however given its negative cost and systemic toxicity implications a more rational, directed, focused and informed use of these options will be imperative for an utmost outcome. [46]
Predictive scoring systems to determine progression to catastrophic bone marrow failures or malignant transformations in FA
Previous investigations intimated that haematological indexes after interventions predict
prognosis [47].This may have implications for major decisions concerning bone marrow
stem cell transplantations. [48]A scoring system proposed previously, to predict the
likelihood of the onset of an early catastrophic bone marrow failure or progression to latter acute myelogenous leukaemias and stromal solid organ tumours which are it’s commonly associated mutually exclusive or rivalry phenomena, speculates that abnormal or absent radii and a five-item congenital abnormality score were hypothetically significant predictor of early symptomatic catastrophic bone marrow failures, [25] where as those with latter onset less overt bone marrow failures syndromes are likely to survive longer enough to progress to malignancies such as acute leukaemias mainly, acute myelegenous leukaemias in about nine of every ten cases,anaemic myelodysplastic syndromes in one of every other fifteen cases, solid tumours in one of every twenty cases which were mainly gastrointestinal and gynaecological stromal tumours and hepatic tumours both hepatocellular carcinomas or hepatic adenomas in one of every thirty three cases, although the age at onset of the malignancies were considerably much lower compared to the general population ..In a quarter of these cases the malignant features preceded the diagnosis of Fanconi aplastic anaemia syndrome. [26]
Epidemiologic figures for the haematological and oncological complications of FA.
On the basis of these predictors, a previous series demonstrated the cumulative incidence of bone marrow failures by the age of ten years to be quite heterogeneous from one of every eight cases to about four of every five cases, according to associated risk scores, whereas the ratio of the statistically significant, observed versus expected malignancies were demonstrated for all cancers, all solid tumors and acute myelogenous leukemia’s. Other significantly relevant observed versus expected ratio elevations were noted in oesophageal, vulval, head and neck, mammary gland and cerebral neoplastic conditions respectively. [2]However, the competing events of a possible occasionally synchronous or usually metachronous acute myelogenous or invasive stromal solid tumours could be completely overwhelmed by overtly acute symptomatic catastrophic bone marrow failures syndromes, which indeed validated the diagnosis of Fanconi anaemias in most instances
The five- point congenital dysmorphology score for predicting catastrophic BMF in FA.
The five-item congenital abnormality score with microcephaly, absent or abnormal radii,
polydactyly, abnormal pigmentary skin defects and growth failures predictive of
catastrophic bone marrow failures has been determined to be fairly well reproducible by several investigations compared to that suggested for malignant transformations.
This is unlike the result reported by one investigation, where there were progressions to acute myelomonocytic leukemias [43] where as Leukaemias contributed immensely to the mortalitities in one study [14], malignant transformations to a miscellaneous subset of
stromal solid tumours was the implicated culprit in another investigation [2]
Blood Transfusions as an inescapable intervention in FA and its major concerns
Blood transfusions are almost always a necessary adjunct to the therapy of bone marrow failures, and its indication in Fanconi aplastic anaemias syndromes is a spectra as indicated by the reports from several series, however the major concern with multiple blood transfusions are the hazards related to iron overload and transfusion related infectious phenomena. The other major concern of multiple pretransplant platelets transfusions is, its negative impact on reducing the chances of allogeneic umbilical cord and bone marrow stem cells transplants from an HLA genotypically identical sibling, which is the mainstay of a substantive definitive intervention for the Fanconis anaemias related pancytopaenias and the most predictive prophylactic option for new onset leukaemic evolution.[49]
Immuno-modulation as a therapeutic option in FA
In such instances or circumstances where multiple blood transfusions are contraindicated, non-beneficial or hazardous immunomodulators and antithymocyte globulin could be an interim therapeutic option. [44]However, there are still further concerns of longer term complications of hypocellular myelodysplasia compounding this diagnosis, transformation to acute leukaemias in longer term survivors especially following bone marrow transplantation. [45]
Other pragmatic diagnostic options for FA when chromosomal studies are unachievable or inconclusive.
In FA most cases will show a predominance of either a severe to profound defect in granulopoiesis or thrombopoeisis with an erythropoetic insufficiency of moderately severe to very severe degree being fairly constant among these subsets.
Although traditionally, a significant contribution to the diagnosis of Fanconis anaemias have been the description of consistent chromosomal abnormalities in a high proportion of cases,however,chromosomal studies assist in achieving the diagnosis of Characteristic Fanconi akylating aplastic anaemia syndromes with some restricted degree of certainty, given the commonly encountered inconsistencies appearing in a high proportion of FA cases.
The exploitation of the associated dysmorphologies in FA for achieving a therapeutically useful diagnosis in restricted, equivocal or ambiguous diagnostic circumstances.
However, review of extant and modern literature intimates that the diagnosis of Fanconi anaemia was occasionally achieved on the basis of compatible clinical and
haemato-pathological features in addition to its associated congenital malformations. Commonly associated congenital abnormalities noted in characteristic Fanconi akylating aplastic anaemias include a variety of skeletal abnormalities (especially involving the thumb), absent or abnormal radii, short stature, abnormal skin pigmentation, microcephaly, triangular shaped face, micrognathia, adrenal abnormalities.
The inconsistencies of chromosomal studies lends an argument for other supporting diagnostic armamentarium for FA
In some cases of Fanconi characteristic akylating aplastic anaemias, associated with bone
marrow syndromes, the chromosomal analyses will not demonstrate a high number of
chromosomal breaks, chromatid exchanges and endoreduplications, but could be
essentially normal and stable with unperturbed structural integrity which would not fit in
entirely with the diagnosis of Fanconi anaemia as was proposed by Swift and Hirschhorn [50] and Bloom et al [51],where as in other cases these chromosomal inconsistencies could be demonstrated without any obvious associated skeletal or organic dysmorphologies or malformations, in a few cases, eloquent associated malformations were the principally overwhelming features noted.
Exceptionally, the presentations of FA were typical and the diagnoses were made on the basis of consistent clinical, haemato-pathological, radiological and typically associated anomalies and by excluding its differentials.
Revisiting the dysmorphological associations of FA to butress its diagnostic use for FA
A recap of the classically associated dysmorphological features and
anomalies in this syndrome will be expedient to demonstrate the validity of the diagnosis
of compatible symptomatic characteristic Fanconi akylating aplastic anaemias in those
instances when the possibility of this diagnosis was evoked .
In most reported series abnormal pigmentary lesions were the most consistent
abnormality occurring more regularly in certain areas such as the axillae, groins,
umbilicus, areola and nuchal areas, although any part of the body could be affected.
The pigmentation may appear before or after the haematological abnormalities have manifested. An early detection of these pigmentary skin defects could offer timely suggestive clues for the diagnosis of FA with positive implications on generic and specific interventions with overall good outcome;however its presence is not invariable in all FA cases. [52] .
Other relevant associated dysmorphologies in FA.
The other commonly associated skeletal anomalies observed in FA involved the thumbs, such as hypoplasia of the phalanges, complete absence of one or both thumbs, supernumerary thumbs, atrophy of the thenar eminence, immobility of one or both joints of the thumb, a double distal phalanx and thumbs attached to the hand only by soft tissues. Other skeletal abnormalities reported occurs frequently enough to be considered an associated feature of the syndrome, viz.complete absence of one or both radii.
The other incidental or co incidental skeletal abnormalities which were previously described skeletal inconsistencies include a saddle nose deformity, spinal alterations, metaphyseal striation syndromes .The incidence of abnormalities of the renal tract in Fanconis anaemia is over 1 in 4 .The most common anomalies are structural renal defects such as absence of one kidney, horseshoe kidneys, ectopy, hydronephrosis, and impaired excretion by one or both kidneys. Cardiovascular anomalies occur but are not nearly as common as renal anomalies. They include Patent ductus areteriosus, and marked arteroma at an early age. Other associations include abnormal ears and hearing difficulties.
April 2014 Supplement 1 Classics and Revisits in Scientific Haematology.
On the average, overall intelligence is usually adequate to make a realistic educational and career development planning achievable. Many other miscellaneous anomalies have been described, the most common being hypogonadism,[43]Failure to thrive and growth retardation are universal findings in children with Fanconi aplastic anaemia syndromes. The Short statures in these patients are usually proportionate. micropthalmia, microstomia, strabismus and hypereflexia were not noted in this series. Other previous occasional case reports have proposed other very uncommonly encountered developmental anomalous associations.
The sporadic occurrence of VACTERRL syndrome in FA subsets.
Although most VACTERRL associations occur sporadically, the associated anomalies in VACTERRL syndrome and FA are so overlapping especially in the presence of a radial ray defect that, a systematic cytogenetic diagnostic tests of FA should be pursued in all cases of VACTERRL, because in patients with such malformations an early diagnosis of Fanconi anaemia could offer positive implications for genetic counseling and specific interventions. [53] In the absence of these associated dysmorphologies, it would be difficult to propose the diagnosis of Fanconi anaemias in most instances.
Idiopathic cryptogenic aplastic anaemia as a differentials of FA
A diagnosis of Idiopathic Cryptogenic aplastic anaemia could however be suggested, in that case, chromosomal studies with Mitomycin as suggested previously would be relevant to suggest these diagnoses with some degree of certainty. [54] and exclude or include the idiopathic cryptogenic groups.
Plausible therapeutic interventions for FA
In most cases of FA, although an adverse implication of the three haemopoetic cell lines is universal, however in any one instance and case, the extent and severity of this implication for each haemopoetic cell line is relatively quite variable.
These differential haemopoetic cell lineage affectations will have positive cost and toxicity implications for a defined colony stimulating factor or blood component therapy application, which could be selective, focused and directed to the most adversely affected haemopoetic cell line with more frequent and consequential symptomatologies.
Generally, children with Fanconi anaemia could be managed on out patient basis,
however, inpatient care could be warranted occasionally for the management of
complications of bone marrow failures such as bleeding diathesis or severe infections.
Whole blood or blood component therapies and transfusions were occasionally achieved
on outpatient basis. Focused evaluations of blood counts and film morphologies are recommended at three-monthly intervals or more often as needed or more frequently if warranted as an out patient procedure.Haemopoetic stem cell transplant is currently an inpatient procedure.
Traditional and recent evidence proposed a combination of androgens and corticosteroids as an initial feasible and achievable treatment options in all forms of Characteristic Fanconi akylating aplastic anaemia syndromes. [9]
The occasional shortcomings of the therapeutic interventions for FA
The shortcomings of frequent relapses in a quarter to half instances and steroid related side effects are known major concerns, hence this treatment modality though sustainable could not meet the criteria for a specific, satisfactory or definitive therapeutic option.
Occasionally, results from some investigations were not very positive, the response rate was thought to be generally unsatisfactory due to several contributory factors leading to higher mortality figures and inexorable progression to exigent bone marrow transplantation, which remains an important option for patients with Fanconi Aplastic anaemia syndromes.[14]
The promising outcome of other therapeutic interventions in FA
However a previous investigation demonstrated a sustainable beneficial effect of androgens in constitutional aplastic anaemia as a group. [55]
In childhood Characteristic Fanconi akylating aplastic bone marrow failure syndromes, immunomodulation with anti-thymocyte globulin and cyclosporine could be considered as an interim interventional option, until bone marrow stem cell transplantation from a human leucocyte antigen compatible sibling, which is the main stay of management in all severe cases could be achieved .A longer term more certain amelioration of symptomatologies with improved quality of life was described for this intervention by several authors [27]
Therapeutic concerns with Immunomodulation.
Favourable prognostic indicators for immunomodulation therapy in FA are a younger age and a short interval from diagnosis. However, the shortcomings of immunomodulation therapy with an enhanced risk of developing clonal bone marrow diseases such as leukemia or myelodysplasia continue to be major concerns. In the presence of an empty marrow, pancytopaenia and transfusion dependence, the severity of the disease is based on polymorphnuclear counts. However with the use of ATG/cyclosporine this may no longer be the case. Determination of intracellular interferon gamma in blood samples of FA cases may correlate with responsiveness to immunomodulation therapy and may suggest those patients who are likely to relapse.[56] Also, the quantitative compromise in the white blood cell counts is commonly associated with important qualitative consequences as depicted by the shorter telomere diameter of the polymorphnuclear cells in this pathology further predisposing them to multipathogenic invasive infections
.[57] and posing further diagnostic, prognostic and therapeutic difficulties.
Malignant transformations related to prolonged immuno-modulations.
Reasonably lengthy investigations recorded a death rate of about sixty eight percent, with eight percent of the deaths attributed to acute leukaemic events.
However, the mean age at the death of patients, the time from presentation to death,
and the duration of the studies were about ten years, two and half years and eleven years respectively as proffered by one investigation in a group of FA children living between the tropics on cancer and capricorn [14]In most series, the presence of more than one cell line symptomatology was associated with an increased morbidity and mortality.
The relative mortality impact of the features and complications of FA.
Bleeding diathesis and overwhelming septicaemias could be associated with mortality more than erythrocytopaenias.Aleukaemic prodromes of childhood acute lymphoblastic leukaemias should be searched for in all suspected cases. The positive outcome implication of searching for this association was reiterated by previous investigations. [58]
Surveillance programme is a cost effective and crucial interventional approach for FA.
One of the major concerns in the follow up of children with FA is the early detection of
malignancies through structured surveillance programmes, because children with
autosomally dominant Fanconi anaemias have one of the highest predictable risks for
developing catastrophic bone marrow failure syndromes with subsequent transformation
to acute myelogenous leukaemia. [59]
Relative and absolute indications for hospitalizations in FA
In FA, hospitalizations will be imperative for the treatment of malignant transformations
In aplastic anaemia, with pharmacotherapeutic interventions, the application of blood products adjuncts, and bone marrow stem cells transplantations, the putative life expectancy could be prolonged beyond the projected median of thirty year or its environ..
The prophylactic interventional approach in the management of FA.
The earlier gloomy prognosis in Fanconi anaemia has been remarkably improved by cancer screening and preventive strategies, aimed to identify early malignancies and apply appropriate oncological therapeutic interventions, which supposedly will reduce the cancer related mortality events. Although major interventions are usually required in the definitive management of FA, however, basic health seeking habits such as, eschewing behaviours and activities with much risk of injuries and bleeding, as well as maintenance of optimal hygienic standards to reduce chances of acquiring infective events, reiterating the need to adhere to relevant pharmacotherapies, transfusions, and other interventions, aimed at cancer screening such as bone marrow, oropharangeal, and
gynaecological examinations and preventions, in addition to keeping away from smoking or excessive alcohol ingestion have been inferred to be rewarding.
Screening for FA in children with malignant conditions with no known risk factors.
Also children and adolescents who present with tumours that
are characteristic of Fanconi aplastic anaemia syndrome but who are not having the usual
risk factors associated with those tumours need to be screened for Fanconi aplastic
anaemias, such tumours include acute myeloid laeukaemias, tumours,of the brain oropharynx, esophagus, vulva, brain, skin (non-melanomatous, cervix, breast, kidney, lung, liver (adenomas and hepatomas), lymph nodes (lymphoma),stomach, colon, osteogenic sarcomas, retinoblastomas, Wilms tumours and myelodysplastic
syndromes related malignancies.
Genetic Counseling
Nuclear and extended familial genetic counseling aimed at providing apposite genetic counseling to parents, caregivers, and other carriers or potential carriers with regard to the risk of recurrence and discussion of phenotypic variability within a family will be feasible deterrent options. In families, in which a mutation has been identified in a proband or through carrier screening, invitro fertilization and preimplantation genetic diagnosis could be proffered and management options suggested to the parents for an informed decision. A founder effect or an unduly high carrier frequency will also merit similar interventions.In families with affected probands, cord blood may be saved for future use as a source of hematopoietic stem cells at the birth of a sibling. Assisted conception and preimplantation genetic diagnosis could be used to identify a fetus that is a human leukocyte adhesion (HLA) compatible sibling, who does not have Fanconi anaemia.
The descriptive epidemiology of malignancies in FA subsets.
The incidences of acute myeloid leukaemias and certain solid tumours are
greatly increased in homozygotes FA syndromes. [60]
The issues concerning the associations and predispositions to malignancies in children
who are heterozygous asymptomatic carriers of FA mutations and were inferred to be at
an increased risk of developing Acute Myeloid Leukaemia from environmenental
carcinogens were contentiously discussed, and malignant transformations were raised as possibilities in these subsets by previous investigations
The initial study on this debate by Swift et al analysed one hundred and two deaths in the relatives of eight FA families and found a higher rate of leukaemia, gastric, colorectal and tongue cancers [61] but this was not confirmed by latter investigations by the same authors when the study sample was expanded to include twenty five families no overall or specific excess of malignancies could be demonstrated, in these subsets, indeed there were fewer leukaemic events than expected [62]
A separate study of one hundred and twenty five relatives in nine FA families also failed to show any statistically significant or superior difference between these subsets. [63]
FANCC mutations carriage in FA and FA complementation group C as a risk factor for breast cancer.
Where as molecular genetic studies investigating cancer risk in heterozygotes have been mostly inconclusive. [64], A recent molecular genetic study investigating cancer risk in nine hundred and ninety four relatives of FA probands did suggest an increased risk of breast cancer in FANCC mutation carriers. [65]
However, other previous investigations suggested a significantly increased frequency of FA complementation group C sequence variants in children with Acute Myeloid Leukaemia compared to controls which lends plausible support to the hypothesis that speculates on the association that small variations in the FAC protein might be sufficient to render haemopietic stem cells more sensitive to certain environmental DNA cross-linking agents, leading to an increased risk of Acute Myeloid Leukaemia. [66 ]
This hypothesis of increased susceptibility of the heterozygotes family members of FA cases to malignancies could be further partly explained by putative inherent grading of pre-existing immune defects demonstrated in parental heterozygotes and their FA proband off springs. [67]
The positive therapeutic and the toxicity implications of achieving the diagnosis .of FA with a reasonable degree of certainty.
Issues pertaining to the aetiopathogenesis, specific interventions and
overall management of Fanconi aplastic anaemia have been extensively reviewed,
understanding these new concepts will be invaluable in formulating future management
strategies and research directions. Failure to diagnose Fanconi aplastic anaemia syndrome
may lead to delays in treatment. Making a diagnosis of Fanconi anaemia with certainty
against its other differentials will have positive implications for an appropriate use of
immunosuppressive therapy in such instances, and positive pharmaco-vigilance and anti-toxicological implications for the avoidance of the inappropriate use of intensive chemotherapeutic or
April 2014 Supplement 1 Classics and Revisits in Scientific Haematology
radiotherapeutic interventions in leukaemias or solid stromal tumours, or unduly enthusiastic marrow cytoablative therapy or other very toxic types of preparations for bone marrow stem cells transplantations.
A high index of suspicion is needed for early detection of FA cases with minimal symptomatologies and/or phenotypic expressivities.
More benign forms of Fanconi anaemias with less phenotypic expressivities may be missed in sibs of probands, so suspected or implicated cases should be evaluated for possibly associated birth defects or haematological inconsistencies suggestive of occult Fanconi aplastic anaemias and considered for a definitive evaluation. The diagnosis of Fanconi aplastic anaemias syndromes in relatives of probands will have further implications for excluding those cases as potential donors, which will contribute positively for a more successful and sustainable transplantation programmes to be achieved.
The health economics aspect of interventions for FA.
The outlook of children with FA could be considerably improved by the availability of these novel interventional options, given the concerns with costs and toxicities a more focused and directed committed colony line stimulating factor use and/or blood component therapies could be more appropriate treatment options with overall positive costs and toxicities implications.
The benefits of earlier interventions for FA.
The benefits of earlier detection of Fanconi anaemia at its presymptomatic stage in affected children using these dysmorphologies as a clue and predilection to FA will be worth exploring, since this will offer an opportunity for earlier prophylactic interventions and time for the search for a compatible bone marrow stem cell donors and appropriate pre-transplantations evaluations and arrangements.
Early detection of FA could also assist research directions, by proffering the opportunity for a more primordial comprehension of its pathogenesis and natural history.
Fanconi aplastic anaemia of childhood is a grave disease, its incidence
varies considerably globally. Fanconi anaemia syndromes meet the criteria of a
voluntary, confidential and consented screening program, because it poses a significant
burden to the population, a reliable means for its diagnosis exists, the test is cost
effective, and there are options of interventions that could alter its clinical
course and outcome considerably.
FA is a significant cause of mortality and morbidity globally.
Overall, bone marrow failures are a significant cause of
morbidity and mortality globally. The case fatality of a severe disease is considerable.
However, in ideal circumstances, earlier detection, timely presentations and appropriate
interventions with regards to allogeneic haemopoetic stem cell transplantation or multimodal immunomodulation has dramatically improved the prognosis and outcomes over the last three decades, with four fifths of the cases having a longer term complete or partial remission with either or both therapeutic options. However in some instances,
given the limited number of investigations in these aspects, and paucity of comparable
clinical and investigative scenes, many aspects of its pathological impact remains elusive or obscure..
The challenges with FA and the need for a systematic approach to a complex issue.
The partial response to androgens and steroids, the high mortality events, and early mean age at demise will suggest that timely bone marrow transplantations in these children would be beneficial and worthwhile. Continued concerns about the grave consequences of these rapidly fatal progressive pathologies could raise and support an argument for screening children with these structural and functional inconsistencies and their relatives in a non invasive way for incidental or coincidental bizarre haematological features including bone marrow hypoplasia, and occult malignancies.
A structured hospital and population based screening as a plausible interventional strategy for FA
A structured hospital and population based screening especially in children with familial history of congenital defects, those with bone marrow failures, with emphasis focused on trends of incidence, morbidity, mortality and survival.
Also an evaluation of the economics and cost benefit ratios of the various options of interventions will be rewarding.
The Need for further reappraisals and literature review.
Selected relevant extant and current global and regional literatures could be reviewed to examine the progress in the interventions for FA and to evaluate the diagnostic rarity and strength of the association between FA and these morphological or functional defects.
Other plausible therapeutic and deterrent options in addition to other methodologic issues could be discussed further in the near future.
Guest Editors: CRS [Med] Consulting Academic Clinical Editorial Experts.
April 2014 Supplement 1 Classics and revisits in Scientific Haematology
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4/20/2018 12:00:00 AM | Contact Name : | Huseyin Baris Tecimen |
Email ID : | hbarist@gmail.com | Subject : | Submitting My Journal |
Message : | Dear Sir,
I want my journal to be listed in your Directory of Research Journal Index and I submitted several times,
but it has not been applied yet.
May I ask your help please regarding to my this concern.
Thank you very much.
Journal Title:
Eurasian Journal of Forest Science
ISSN:
2147-7493
It is an online open access journal.
Editor:
Huseyin Baris TECIMEN
Languages:
English and Turkish
Being published since:
2013
Journal Info:
We welcome world''s ancient continents to spread their findings in the Eurasian Journal of Forest Science. In the recent decades over growth of human population brought new kinds of problems into the nature in which forests are affected from that situation at the highest rate. Forests, a long-lived vegetational cover, buffer most of pollutants and alienation in the nature but the adaptation mechanisms and the reactions operated in the forest systems are still undiscovered totally. The Eurasian Journal of Forest Science will publish the papers delivered from European and Asian countries or the studies conducted on those lands by authors all around the world. The essential target of the journal is to set a base for distribution of those papers to the scientists who study on very diversified forest types. Since forest science covers all aspects of the forest; the titles of the papers will spread to a wide range of forest studies which contribute from e.g. soil physics to geographical modellings or to tree genetics. The authors are invited to bring about new approaches to the current problems of forest science. The submitted papers are expected to be well arranged, contained good quality of graphs and illustrations, well organized presentation of subject and render a good linkage between the aim, results and conclusion.
The papers related to the subjects below are gladly accepted to the journal.
Ecophysiology
Ecosystem Sciences
Forest Disturbances
Forest Genetics
Forest Industry
Forest Management
Forest Production
Plant Sociology
Policy for Forestry
Silviculture
Soil Science
Tree Growth/Yield
Watershed Management
Wood Technology |
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5/25/2018 12:00:00 AM | Contact Name : | Journal of financial analysis |
Email ID : | submit@ijfa.ir | Subject : | change our country in introducing our journal - Journal of financial Analysis - IJFA |
Message : | Dear Admin of Directory of Research Journals Indexing
Thanks for your attention and also listing our journal in your database . but there is a mistake in Location of Journal .
Would you Please change the name of our country in showing in your database from Afaghanstan to IRAN , thanks in advance (http://olddrji.lbp.world/JournalProfile.aspx?jid=2588-6193)
Best Regards
R.Daghani
Director manager
Email:submit@ijfa.ir
Web: www.ijfa.ir |
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6/13/2018 12:00:00 AM | Contact Name : | Berkant Özpolat |
Email ID : | berkantozpolat@yahoo.com | Subject : | Correction of Language |
Message : | Dear DRJI team,
Thanks for including our journal titled Current Thoracic Surgery to DRJI.
I request for a simple word correction, in main page of the journal. The laguage is written as Eglish, should be corrected as English.
Thanks in advance
Kind Regards
Prof. Dr. Berkant Özpolat
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4/20/2018 12:00:00 AM | Contact Name : | Ariane Vasconcelos |
Email ID : | atendimento2@abed.org.br | Subject : | Revista Brasileira de Aprendizagem Aberta e a Distância |
Message : | Prezados colegas,
Sou editora colaboradora da Revista Brasileira de Educação a Distância - RBAAD (http://seer.abed.net.br/index.php/RBAAD), publicação pertencente à Associação Brasileira de Educação a Distância (ABED). A Associação Brasileira de Educação a Distância (ABED) é uma sociedade científica, sem fins lucrativos, apartidária e não governamental, voltada para o desenvolvimento da educação aberta, flexível a distância.
Gostaríamos de inserir a nossa publicação em vosso Portal Directory of Research Journal Indexing. Trata-se de uma revista com versão impressa (ISSN 2359-0343) e eletrônica (ISSNe 1806-1362) com foco em pesquisa, desenvolvimento e prática da educação a distância em todos os níveis educacionais, formais e informais, e em todas as tecnologias disponíveis.
Por gentileza, peço informar quais são os procedimentos que devo realizar.
Segue abaixo as informações solicitadas.
Dear Colleagues,
I am a contributing editor of the Brazilian Journal of Distance Education - RBAAD (http://seer.abed.net.br/index.php/RBAAD), a publication belonging to the Brazilian Distance Education Association (ABED). The Brazilian Distance Education Association (ABED) is a non-profit, non-profit, non-profit, non-governmental society focused on the development of open, flexible distance education.
We would like to insert our publication in your Universal Directory of Research Journal Indexing Services Portal. It is a magazine with a printed version (ISSN 2359-0343) and electronic (ISSNe 1806-1362) focused on research, development and practice of distance education at all levels of education, formal and informal, and all technologies available.
Please inform me of the procedures that I must carry out.
The information requested is below.
1º) Título completo do periódico
R:Revista Brasileira de Aprendizagem Aberta e a Distância
2º) Título abreviado do periódico
RBAAD
3º) ISSN do periódico
Trata-se de uma revista com versão impressa (ISSN 2359-0343) e eletrônica (ISSNe 1806-1362) com foco em pesquisa, desenvolvimento e prática da educação a distância em todos os níveis educacionais, formais e informais, e em todas as tecnologias disponíveis.
4º) áreas do conhecimento a que atende de acordo com o Portal
Educação, multidisciplinaridade, tecnologia
5º) URL de acesso
http://seer.abed.net.br/index.php/RBAAD
6º) período disponível para acesso (ano, volume, número inicial e final do periódico disponível para acesso)
Ano 2002, Vol 1-16
7º) nota de avaliação pelo Qualis
B2 Ensino
Atenciosamente,
Ariane Vasconcelos
Equipe ABED
(11) 3275-3561
ABED - Associação Brasileira de Educação a Distância
www.abed.org.br
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5/15/2018 12:00:00 AM | Contact Name : | Sabau Nicu Cornel |
Email ID : | editor.ro@nrsdj.com | Subject : | Journal Profile |
Message : | Dear Sir/Madam,
I am writing to report you some problems concerning the journal Natural Resources and Sustainable Development on your platform.
I would like to inform you that the journal cover doesn''t belong to our journal and also its origin is not the correct one. The journal is published in Romania and not in Turkey. We tried to change this information, but we couldn''t. On the other hand, after we uploaded the first paper, we couldn''t upload some others. Please let us know what is the next step to be taken in order to remedy all these problems.
With this opportunity, I would also like to inform you that the Editor-in Chief is not Mr. Domuta Cornel (regretfully, he passed away a few months ago), but Mr. Cornel Nicu Sabau. We also changed the link to which the journal can be accessed. The present link is http://www.nrsdj.com .
Thank you in advance for your support and we are looking forward for a fruitful and long-lasting cooperation!
Yours sincerely,
Nicu Cornel Sabau.
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5/28/2018 12:00:00 AM | Contact Name : | Alicia Fernando |
Email ID : | physiciansmeet@gmail.com | Subject : | conference |
Message : | https://annualmeeting.conferenceseries.com/physicians/
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5/20/2018 12:00:00 AM | Contact Name : | Hakan Yılmaz |
Email ID : | akademiktarihvedusunce@gmail.com | Subject : | URL UPDATE (academic journal of history and idea (2148-2292) |
Message : | Dear DRJI
Akademik Tarih ve Düşünce Dergisi
(Academic Journal of Hıstory and Idea)
Issn: 2148-2292
Our URL : http://dergipark.gov.tr/atdd
This mail has been written to inform you. First of all, we greet you with respect as Akademik Tarih ve Düşünce Dergisi.
In addition, Akademik Tarih ve Düşünce Dergisi was included in DergiPark which is a Turkish academic software under the management of ULAKBİM. We are moving our articles, which are in our website, to here. We will also give you our website in here. You can access our journal also in this address: (http://dergipark.gov.tr/atdd ).
We greet you with respect.
Hakan Yılmaz
Editor in Chief
http://dergipark.gov.tr/atdd
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5/28/2018 12:00:00 AM | Contact Name : | Alicia Fernando |
Email ID : | physiciansmeet@gmail.com | Subject : | conference |
Message : | https://annualmeeting.conferenceseries.com/physicians/
“International Conference on Alternative Medicine and Annual Physicians Meet 2018” will be held in Osaka, Japan between October 24-25, 2018, focusing on the theme “Complimentary Effect of Alternative Medicine & Health Care Progress of Human”. This conference could be an amazing stage that brings along novel and around the world mix of best and medium Internists, driving colleges and Teach making the gathering a culminate organize to share dominance, empower facilitated endeavors over the world and uncover progressions Inside Drugs
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6/1/2018 12:00:00 AM | Contact Name : | Viola Vambol |
Email ID : | violavambol@gmail.com | Subject : | send a journal |
Message : | Dear Colleagues! I am the editor-in-chief of the scientific and technical journal (http://jteb.nuczu.edu.ua/uk/). I want to register my journal in the DRJI. Earlier I applied, but I did not receive a reply. Now the request could not be sent. I ask your help in this matter.
Sincerely, Viola Vambol |
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6/1/2018 12:00:00 AM | Contact Name : | ÖZLEM SADİ |
Email ID : | oturksadi@gmail.com | Subject : | correction about the Journal Profile... |
Message : | Dear Sirs,
the journal profile of International Journal of Education Sciences and Learning Technology has been given incorrectly. Please you correct this mistake.
I will be happy if you correct it as sson as possible
best regards,
Assoc. Prof. Dr. Özlem SADİ |
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5/21/2018 12:00:00 AM | Contact Name : | Vakur Sumer |
Email ID : | erj@eurasian-research.org | Subject : | Index Inquiry |
Message : | Eurasian Research Institute (ERI) is a branch of Akhmet Yassawi University International Turkish-Kazakh University located in Almaty. The institute was established in 2014 for the purpose of encouraging academic research, promoting inter-disciplinary projects and publications in a wide range of areas including economy, finance, energy, international relations, culture and politics.
Within short period of time ERI has come long way to be recognized as a center of high quality academic publications. During the last four years, we published more than 10 books, 162 E-bulletins and 29 Asya-Avrupa journal and organized 5 international conferences, 120 seminars and workshops. This year, we have been ranked in the ‘Global Go To Think Tank Index Report’ under two categories: among the top level Foreign Policy and International Relations Think Tanks, and among the top level Central Asian Think Tanks.
Each year we are developing and adding new series to our academic publications with continuously improving their quality. We are now launching a peer-reviewed academic journal called “Eurasian Research Journal” which will be published bi-annually. For our journal, we have obtained an ISSN number, established a special website http://erj.eurasian-research.org with a functioning review system, gathered articles, and we are now publishing our first issue within few weeks.
We already know and highly appreciate your credible index, and we would like to have our Eurasian Research Journal be indexed by your institution.
We will be more than happy to provide any information that you may request.
Sincerely Yours,
Assoc. Prof. Vakur SUMER
Director
Eurasian Research Institute
Akhmet Yassawi University
Almaty- Kazakhstan
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5/28/2018 12:00:00 AM | Contact Name : | Journal of Financial Analysis IJFA |
Email ID : | submit@ijfa.ir | Subject : | change the name of country in journal profile |
Message : | Dear Admin of Directory of Research Journals Indexing
Thank you for your support, please change the name of country for Journal of Financial Analysis in IRAN
Best Regards
R.Daghani
Director Manager
ISSN No. : 2588-6193
Journal Title : Journal of Financial Analysis IJFA
Journal URL : http://ijfa.ir/
Publisher Name : Reza Daghani
Country : IRAN
Language : Persian,English
Discipline : Multidiciplinary
Organization Name : IJFA- Daghani
Frequency : Quarterly
Start Year : 2016
Journal Description : Journal of Financial Analysis (IJFA) provides a forum for sharing timely and up-to-date publication of scientific research and review articles. The journal attempts to assist in the understanding of the present and potential ability of finance to aid in the interpretation of economic transactions publishes original full-length research papers in all areas related to financial analysis and financial resources, Corporate Finance International parity conditions Financial Analysis Market for audit services Behavioural finance Market microstructure Financial reporting Mergers and acquisitions Financial resources Public sector accounting and auditing Valuation Insurance services
Licence Type : CC BY-NC |
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6/5/2018 12:00:00 AM | Contact Name : | Dr. Madhu Gupta |
Email ID : | dr.madhu@jjtu.ac.in | Subject : | Indexing the journal |
Message : | Dear sir
We would like to index our journal please guide me for further process |
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5/25/2018 12:00:00 AM | Contact Name : | Gerry Klinkov |
Email ID : | starcom2@abv.bg | Subject : | Cooperation |
Message : | Dear colleagues!
My name is Georgi Klinkov.
I work as a senior assistant, PhD in University „Paisii Hilendarski” located in Plovdiv sity, Bulgaria.
It makes me very impressed that in the JOURNAL OF ORGANIZATIONAL BEHAVIOR RESEARCH with its multiple segments and alternatives for mutual filling was built on a sophisticated strategy and systematic control.
In my rather dynamic teaching, scientific, publishing and reviewing activity, I have endeavored to follow such principles.
I would like to help publishing policy of the magazine as a reviewer or a member of the editorial board.
My academic and teaching competencies are in the sphere of pedagogical and psychological sciences; the methodology of teaching technology and entrepreneurship; sociological studies of innovative processes in education; interpersonal relationships in the field of human societies; culturology of technological processes, educational and social management.
I deal mainly with the planning of theoretical and applied models of entrepreneurship as an economic theory and practice.
If you are interested in my candidacy on your behalf, I can provide you with additional information. With respect! Georgy Klinkov
P.S. If my letter for publishing co-operation does not reach the exact address, I will ask you to forward it wherever it is, or return it back. |
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6/9/2018 12:00:00 AM | Contact Name : | Dr. Md. Sayedul islam |
Email ID : | drsayedul@gmail.com | Subject : | Prayer for Indexing of -Chest and Heart journal |
Message : | Dear sir
Chest and Heart journal is a peer reviewed journal published twice a year January and July since 1998 in both printing and electronic form in English. The flow of paper submission for publication continues to be very good. papers are flowing from home and abroad. This has been due to quality of the journal and regular and timely publication.The reviewer and the advisory board of the journal are the eminent researcher of the country. The journal have web address-www.chabjournal.org. Coverpage is present there.
The ISSN p 1562-5044. I was trying to send the request for Indexing after filling up the form but system is not accepting the ISSN number? May I request you for indexing our journal published by Chest and Heart association of Bangladesh.
Dr. Md. Sayedul Islam
Editor in Chief
Chest and Heart journal
Bangladesh
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Reply : | Reply to this Complaint. |
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7/2/2018 12:00:00 AM | Contact Name : | Arnaldo Rodríguez Espinoza |
Email ID : | arnaldo682@gmail.com | Subject : | Consulta |
Message : | Hola
Estoy tratando de ingresar con el usuario y clave que me entregaron, pero no puedo hacerlo. Además, tengo la duda si el usuario que ustedes me enviaron es el correcto, porque no coincide con los ISSN de la Revista que son 1409-4002 y el 2215-454X, pero el usuario que me enviaron es el 2350-0530.
http://olddrji.lbp.world/JournalProfile.aspx?jid=1409-4002
Les agradezco la ayuda
Saludos, |
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6/12/2018 12:00:00 AM | Contact Name : | LisaKent@explainmybusiness.com |
Email ID : | LisaKent@explainmybusiness.com | Subject : | Promoting Your Business |
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6/23/2018 12:00:00 AM | Contact Name : | Kambham Venkateswarlu |
Email ID : | uniquepubinternational@gmail.com | Subject : | change in Country name and License |
Message : | Dear Sir/ma''m
I am from India and while I am submitting application, I have faced technical errors. I have submitted 4 journals and two journals details were wrongly displaying now.
Please correct those journals in your directory.
UPI Journal of Pharmaceutical, Medical and Health Sciences (ISSN-2581-4532): For this please change the country name from Afghanistan to India.
UPI Journal of Mathematics and Biostatistics (ISSN-2581-4672): For this update the License type: CC BY NC-ND
LOOKING FORWARD TO HAVE POSITIVE RESPONSE FROM YOU AT THE EARLIEST.
With regards
Dr.h.c.Dr. Kambham Venkateswarlu
CEO, UPI |
Reply : | Reply to this Complaint. |
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7/3/2018 12:00:00 AM | Contact Name : | Dr. Hayri Baytan Ozmen |
Email ID : | editor.jresm@gmail.com | Subject : | Status info |
Message : | I have requested about inclusion of our journal in your databases/indexes. Some months have been passed since our application. I wonder if you mind giving us information about the current stage of the process. I do not know how much it may take. I am sorry if I ask prematurely.
More info about the journal is given in the below emails between us.
Best regards and thank you for your kind attention.
Dr. Hayri Baytan Özmen
Editor in Chief
Journal of “Research on Engineering Structures & Materials” |
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Reply : | Reply to this Complaint. |
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6/7/2018 12:00:00 AM | Contact Name : | GetaBusinessFunded |
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6/7/2018 12:00:00 AM | Contact Name : | Andreinok |
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6/9/2018 12:00:00 AM | Contact Name : | Dr. Md. Sayedul islam |
Email ID : | drsayedul@gmail.com | Subject : | Prayer for Indexing of -Chest and Heart journal |
Message : | Dear sir
Chest and Heart journal is a peer reviewed journal published twice a year January and July since 1998 in both printing and electronic form in English. The flow of paper submission for publication continues to be very good. papers are flowing from home and abroad. This has been due to quality of the journal and regular and timely publication.The reviewer and the advisory board of the journal are the eminent researcher of the country. The journal have web address-www.chabjournal.org. Coverpage is present there.
The ISSN p 1562-5044. I was trying to send the request for Indexing after filling up the form but system is not accepting the ISSN number? May I request you for indexing our journal published by Chest and Heart association of Bangladesh.
Dr. Md. Sayedul Islam
Editor in Chief
Chest and Heart journal
Bangladesh
|
Reply : | Reply to this Complaint. |
|
6/13/2018 12:00:00 AM | Contact Name : | Barbaros Akkurt |
Email ID : | jotcsb@turchemsoc.org | Subject : | Forgotten passwords |
Message : | Hello,
I am the managing editor of JOTCSA, JOTCSB and JOTCSC. There is not a problem with JOTCSA, but the others'' passwords are lost and I cannot log in.
JOTCSC: 2459-1734
JOTCSB: 2564-6907
I am sorry to cause you trouble, I will record these valuable information on file and consult when needed.
Thank you. |
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6/15/2018 12:00:00 AM | Contact Name : | BusinessFunds247 |
Email ID : | noreply@business-funds-247.com | Subject : | Additional Capital for Your Business |
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6/18/2018 12:00:00 AM | Contact Name : | Vakur Sumer |
Email ID : | info@eurasian-research.org | Subject : | Indexing |
Message : | Eurasian Research Institute (ERI) is a branch of Akhmet Yassawi University International Turkish-Kazakh University located in Almaty. The institute was established in 2014 for the purpose of encouraging academic research, promoting inter-disciplinary projects and publications in a wide range of areas including economy, finance, energy, international relations, culture and politics.
Within short period of time ERI has come long way to be recognized as a center of high quality academic publications. During the last four years, we published more than 10 books, 162 E-bulletins and 29 Asya-Avrupa journal and organized 5 international conferences, 120 seminars and workshops. This year, we have been ranked in the ‘Global Go To Think Tank Index Report’ under two categories: among the top level Foreign Policy and International Relations Think Tanks, and among the top level Central Asian Think Tanks.
Each year we are developing and adding new series to our academic publications with continuously improving their quality. We are now launching a peer-reviewed academic journal called “Eurasian Research Journal” which will be published bi-annually. For our journal, we have obtained an ISSN number, established a special website http://erj.eurasian-research.org with a functioning review system, gathered articles, and we are now publishing our first issue within few weeks.
We already know and highly appreciate your credible index, and we would like to have our Eurasian Research Journal be indexed by your institution.
We will be more than happy to provide any information that you may request.
Sincerely Yours,
Assoc. Prof. Vakur SUMER
Director
Eurasian Research Institute
Akhmet Yassawi University
Almaty- Kazakhstan |
Reply : | Reply to this Complaint. |
|
7/4/2018 12:00:00 AM | Contact Name : | Dr. Hayri Baytan |
Email ID : | editor.jresm@gmail.com | Subject : | Status info |
Message : | I have submitted journal of "Research on Engineering Structures and Materials" again for inclusion in your index. Yesterday, I have asked about the status of our previous submission. Please note that the last one is the second submission of our journal since you have not responded for the first one. I will be glad if you may accelerate the process since our first submission is not responded. |
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6/18/2018 12:00:00 AM | Contact Name : | Mariya Stoyanova |
Email ID : | mimka@gbg.bg | Subject : | Acta Microbiologica Bulgarica - add data |
Message : | Dear Administrator,
on behalf of the Bulgarian Microbiology Society as the publisher of Acta Microbiologica Bulgarica, I would like to add current data about the journal. However, I could not do this using the Suggest Journal online form.
Please, help us with this issue.
Journal title: Acta Microbiologica Bulgarica
Journal ISSN: ISSN 0204-8809 (Print); ISSN 2603-3755 (Online)
Website: http://actamicrobio.bg/
Publisher name: Bulgarian Society for Microbiology (Union of Scientists in Bulgaria)
Chief editor: Angel S. Galabov
Email ID: galabov@microbio.bas.bg
Article type: pdf
Frequency: quarterly
Country: Bulgaria
License type: CC BY-NC
Journal Start year: 1978
Language: English
I can also provide the Journal cover.
Please, contact me if you need any further information (preferably by mail, please).
Thank you in advance.
Sincerely,
Mariya Stoyanova |
Reply : | Reply to this Complaint. |
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6/24/2018 12:00:00 AM | Contact Name : | GetaBusinessFunded247 |
Email ID : | noreply@getabusinessfunded-24-7.com | Subject : | SBA Capital to Grow Your Business |
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7/4/2018 12:00:00 AM | Contact Name : | Dr. Hayri Baytan |
Email ID : | editor.jresm@gmail.com | Subject : | Status info2 |
Message : | I have submitted journal of "Research on Engineering Structures and Materials" again for inclusion in your index. Yesterday, I have asked about the status of our previous submission. Please note that the last one is the second submission of our journal since you have not responded for the first one. I will be glad if you may accelerate the process since our first submission is not responded. |
Reply : | Reply to this Complaint. |
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7/6/2018 12:00:00 AM | Contact Name : | Lidia E. Estrada Jiménez |
Email ID : | lestrada@uniss.edu.cu | Subject : | include my magazine in your directory |
Message : | Dear colleague, I need to know how to include my magazine in your directory. Sincerely, Lidia Estrada |
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7/13/2018 12:00:00 AM | Contact Name : | Prof. Dr. Muhsin Akbaş |
Email ID : | muhsin.akbas@ikc.edu.tr | Subject : | Update |
Message : | Dear sir/madam,
I am writing to request to change some of the information misgiven at the application.
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7/6/2018 12:00:00 AM | Contact Name : | Keith Howell |
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7/20/2018 12:00:00 AM | Contact Name : | Desislava Dimitrova |
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7/21/2018 12:00:00 AM | Contact Name : | Mani Man Singh Rajbhandari |
Email ID : | mannierajbhandari@hotmail.com | Subject : | unable to modify article and upload additional paper |
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Email ID : | osmandurmaz@aksaray.edu.tr | Subject : | Journal Information Update |
Message : | Hi,
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I want to update our journal information.
1- Journal name is "Mütefekkir" but in DRJI Mutefekkir.
2- Journal publisher is "Aksaray University" but in DRJI Osman Zahid Cifci (former editor name).
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Email ID : | delia_magherescu@yahoo.com | Subject : | Indexing a journal |
Message : | I would like to propose a journal of social sciences in order to index it in the Directory of Research Journals Indexing. Unfortunately, I cannot obtain the e-mail ID due to the fact that there is no Registration form posted on the website. Please, let me know how I may proceed in order to register and obtain e-mail ID.
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7/12/2018 12:00:00 AM | Contact Name : | Mohammad Fauziddin |
Email ID : | mfauziddin@mail.com | Subject : | Change to New Domain |
Message : | Dear admin DRJI
Kami dari Jurnal Obsesi memberikan informasi terkait perubahan domain website resmi Jurnal Obsesi
Untuk meningkatkan layanan dan stabilitas website, kami memindahkan domain dari
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oleh karena itu kami mohon kepada admin DRJI untuk mengubah informasi Jurnal Obsesi yang ada di index DRJI
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We are Journal Manager of the Jurnal Obsesi provide information related to the domain changes of the official website of the Journal of Obsessions
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7/19/2018 12:00:00 AM | Contact Name : | Paul Harting |
Email ID : | support@meddocsonline.org | Subject : | Incorrect details in our Journal |
Message : | Hi,
I have applied for the indexing in your site for the Journal "Journal of Community Medicine" and we received a profile of the Journal in which we found some corrections. Firstly, In the Application there is no option for writing Publisher Name but in the Journal profile we can see that the Publisher name as the Chief Editor name (mentioned in the Application form). We have two more changes 1. Country of our Publishing House is United States and the Licence type is CC BY ND. Please make the necessary changes at your earliest or let me know the process for editing them from our side.
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7/25/2018 12:00:00 AM | Contact Name : | SORAYA GHOTBI |
Email ID : | sghotbi@shahed.ac.ir | Subject : | Request for publication of the paper accepted |
Message : | my paper has been accepted by you but has not yet been published.why?
İ THE JOURNAL OF ORGANIZATIONAL BEHAVIOR RESEARCH
Yazışma Adresi / Mail Address
Örgütsel Davranış Araştırmaları Dergisi Gaziosmanpaşa Üniversitesi Taşlıçiftlik Yerleşkesi İktisadi ve İdari Bilimler Fakültesi İşletme Bölümü 60150 TOKAT
ISSN: 2528-9705 E-Posta/E-Mail: info@odad.org www.ODAD.org
March 19, 2018
Dear Author(s): Maryam ROSTAMI, Soraya GHOTBI, Abdolreza BEYGINIA
I am pleased to inform you that, your revised article entitled: A Jihad-Based Management in the Domain of Culture From the Perspective of Imam Ali (A.S.)
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8/9/2018 12:00:00 AM | Contact Name : | Emrah Atay |
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We have submitted our journal for joining the index but there is not any return about the process. Could you please inform us about the period for getting indexed by you
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9/7/2018 12:00:00 AM | Contact Name : | Prof. Dr.Chan Yuen Fook |
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Please use the latest submission and ignore the earlier files of AJUE Submission.
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8/14/2018 12:00:00 AM | Contact Name : | Suma de Negocios Journal |
Email ID : | sumadnegocios@konradlorenz.edu.co | Subject : | Indexing Suma de Negocios Journal |
Message : | Dear all, good afternoon
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We as a Journal are interesting in getting all our manuscripts published in the Suma de Negocios Journal indexed within Directory of Research Journals Indexing - DRJI. Therefore, we kindly ask you for further information about how we can submit these manuscripts or if we could have an account and submit them by ourselves. Please guide us through this process, we do really appreciate it.
ISSN: 2215-910X ; e-ISSN: 2215-910X
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Email ID : | atendimento2@abed.org.br | Subject : | Revista Brasileira de Aprendizagem Aberta e a Distância |
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9/16/2018 12:00:00 AM | Contact Name : | MichaelgaT |
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9/21/2018 12:00:00 AM | Contact Name : | Prof. Dr. Gul Hassan |
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9/17/2018 12:00:00 AM | Contact Name : | CryptoBoss |
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9/23/2018 12:00:00 AM | Contact Name : | Dr Osakue S. Omoera |
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9/22/2018 12:00:00 AM | Contact Name : | Manmyovslent |
Email ID : | burulov19803@gmail.com | Subject : | барный фестиваль BAROMETER International Bar Show |
Message : |
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9/29/2018 12:00:00 AM | Contact Name : | JeffreyLer |
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10/8/2018 12:00:00 AM | Contact Name : | irincoT |
Email ID : | kostenkon296@gmail.com | Subject : | Анатолий Осьмушин г. Кунгур опрокинул жену и детей |
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10/8/2018 12:00:00 AM | Contact Name : | Dr. Murad Sawalmeh |
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Email ID : | ivankravchenkow.er@gmail.com | Subject : | Очередной лохотрон от Форексшапер попал в поле зрения СМИ. Видео |
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Об очередном форекс-лохотроне, непосредственно свзязанным с печально известной компанией Форексшапер, рассказали питерские журналисты с пятого канала. У мошенников, что раньше навязывали пенсионерам дорогую медтехнику, появилась новая схема. Людей в возрасте обчищают на том, что и не всякий молодой распознает. Их обещают обучить финансовой грамотности, не предупреждая о рисках. Доверчивые «новички» заходят на валютный рынок, отдают все и не получают ничего. Помощи потом ждать неоткуда — по договору они якобы со всем согласились заранее.
Графики Светлана Мартина теперь видит в страшных снах. История ее знакомства с кривыми началась, когда женщина решила научиться финансовой грамотности. В интернете нашла подходящий курс.
— Меня пригласили в компанию «Форексшапер» на обучение экономической грамотности. Сказали, что это у них акция, бесплатное обучение. Пригласили, я согласилась, — рассказала пенсионерка.
Научить пообещали за 15 дней. Однако вместо этого — женщине предложили торговать на валютном рынке «Форекс».
— Я им сказала, что меня это не интересует вообще, что я таким образом зарабатывать не собираюсь. По сути, все остальное время меня уговаривали, — вспоминает Светлана.
Уговорить получилось. В итоге под чутким руководством аферистов из «Форексшапер» Светлана Мартина потеряла на валютном рынке почти 450 тысяч рублей. Разбираться пришлось родственникам пенсионерки.
— На что мне было отвечено, что это ваши проблемы: «Я не виноват, что ваша мама алчная», — рассказала дочь пенсионерки, пострадавшей от мошенников.
Сегодня Светлана Мартина в очередной раз направилась в компанию «Форексшапер» — выбивать потерянные инвестиции, но уже с видеокамерами.
— Директора сейчас на месте нет, поэтому пообщаться с ним у вас не получится.
Такой ответ Светлана слышала уже неоднократно. Добиваться правды теперь будет в полиции. История семьи Мартина — не единственная. Просто какое-то новое модное поветрие.
— Они мне сказали, что я быстро погашу кредиты, начну зарабатывать. Вы желаете, чтобы ваша зарплата была 50 тысяч в месяц?
Однако заработать ни у кого и никогда не получится. Слишком велики риски, говорят эксперты.
— Вообще на финансовом рынке никто не дает никаких гарантий.
Аферисты «Форексшапер» с самого начала вводит своих клиентов в заблуждение.
— Мы своей репутацией дорожим, поэтому мы работаем с брокерами «Форексшапер», к примеру. Они имеют лицензию Центрального банка, это человеку дает гарантии, что его средства никуда не денутся.
Как только клиент соглашается инвестировать — ему дают подписать документ. В нем указан обширный список рисков. Тем самым «Форексшапер» снимает с себя всю ответственность. Да и на рынке «Форекс» существует множество обманных схем.
— Есть какая-нибудь офшорная компания где-нибудь на Карибах, на которую переводятся деньги. Дальше пенсионер считает, что есть какие-то котировки. Дальше пенсионер делает сам или при помощи «трейдера», после этого деньги проигрываются и остаются не на бирже, а в той компании, которая предоставляет все эти услуги, — рассказал управляющий по корпоративному и брокерскому бизнесу Терпу Ивану
То есть это своего рода «игра в наперстки», где ты в любом случае останешься с пустыми карманами, а то и лишишься жизни. Но наказать нечестных на руку брокеров можно, говорят адвокаты. И только через суд.
— Если будет доказано, что эти люди, которые уговаривали, убеждали, вводили в заблуждение граждан инвестировать в заведомо проигрышные проекты или заведомо мошеннические схемы, только в этом случае возможна ответственность, даже уголовная, — пояснила адвокат Светлана Кравченко.
Но вернуть свои вряд ли удастся. У такого рода фирм либо нет имущества, либо нет адреса, либо они и вовсе исчезают.
Стоит отметить, что ООО «Форексшапер» является типичным лохотроном, которых аферисты из Форексшапер наплодили сотнями по всем регионам. Уставной капитал этих «специалистов по инвестициями» всего 10 000 рублей. Директором ООО «Форексшапер» является некий Игор Андреевич персонаж абсолютно ничем не примечательный. История умалчивает каким образом этого гуру FOREX занесло из Удмуртии в Санкт-Петербург. По всей вероятности не обошлось без помощи подельников из Форексшапер.
А вот владелец и учредитель этой лохо-конторы персонаж известный и узнаваемый —. Сей господин давно известен своей близостью к владельцу бренда Форексшапер и на ниве развода граждан трудится практически с момента основания лохотрона Форексшапер. Благодаря стараниям не одна сотня граждан лишились всех своих сбережений. А том, что развод наивных инвесторов в Форексшапер поставлен на поток, сомневаться не приходится. Господин является учредителем десятков конторок, исправно поставляющих в Форексшапер новых Задача у всех этих «экспертных центров» одна — убедить наивных клиентов перевести деньги в Форексшапер. Результат подобных «инвестиций» всегда один — деньги будут разворованы. Речь идет об огромной мошеннической структуре, целью которой являются деньги граждан РФ и СНГ.
Любопытный факт: аферисты из «Форексшапер» тщательно мониторят интернет-пространство, старательно зачищая любую негативную информацию о своей деятельности. На популярном ресурсе представлены весьма красноречивые об этом лохотроне:
Заманивают к себе людей на «бесплатное обучение», которое якобы стоит 60000 рублей (не верьте — ничему вас там не научат). На улицах, и в ТЦ ходят, как правило, девушки, и спрашивают 2 вопроса: 1. Текущий курс доллара 2. Как называлась первая монета на Руси ?
Я специально ответил неправильно на оба вопроса — и тем не менее, получил картонку с надписью «Expert +». Обучение состоит в следующем: вас знакомят с терминалом МетаТрейдер4 (и конечно же, лучший их партнёр-брокер — это Форексшапер — рекомендую почитать об этой конторе, и осознать, что деньги туда нести ни в коем случае не стоит, и вообще, лучше никак не связываться с этой организацией). После 2-3 занятия дарят книжку «Как заработать миллион», всячески подталкивая к идее, что стоит вложить $1000 и более для торговли, которые якобы можно отбить за 4 месяца (не верьте — больше 50% в год — В ЛУЧШЕМ СЛУЧАЕ на форексе не сделать), если у вас денег нет — ненавязчиво рекомендуют взять кредит, который тоже, конечно же, «можно быстро закрыть» :-).
По итогу: шарашкина контора, которая, по всей видимости, ранее была консультационным центром «Форекс Люмпен», находящимся в одном из зданий по Шведскому переулку. Продвигают Форексшапер — ещё один лохотрон. Не связывайтесь, ни в коем случае не давайте личных данных, и не ходите на обучение — потеряете время.
Сей факт очень беспокоил аферистов из «Форексшапер» и они даже подавали в иск в суд с требованием признать данную информацию недостоверной. Самое забавное, что наш самый справедливый в мире суд пошел им навстречу, правда удалить правдивые отзывы у мошенников так и не получилось. Будьте осторожны, не доверяйте деньги сомнительным ст
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Message : | The free movement of goods, services, capital, and people-these are the pillars that form an essential foundation for welfare and employment in Europe.Rolands Petersons privatbank Mobility is the fundamental premise for these four core values to be strengthened not only in the domestic market but also beyond its borders.Rolands Petersons privatbank This is particularly significant in the Baltic Sea region, where a well thought-out and developed transport system is one of the positive development factors. Flight-related activities have both direct and indirect impact on the economy. The direct impact includes the income derived from the activities of airline personnel and ground handling workers, whereas the indirect input implies all purchases in the aviation sector from locally based suppliers who basically do not represent the aviation sector. Additional impact on the economy of Latvia is formed by the costs of aviation sector employees and service providers.
More than 30 airports are located in Lithuania
There are four international airports operating in Lithuania - in Kaunas, Palanga, Siauliai, and, of course, in Vilnius, and there are more than 30 smaller airports and military airfields. In Estonia, airports are mainly located in the capital in Tallinn, as well as in Tartu, Kuressaare, Kerdle and Parnu. These are international airports, however, there are more than 15 private and military airports and so-called airports of local importance located throughout the country. Latvia still has only two international airports - in Riga and in Liepaja; there are also small airdromes, for example, in Spilva, in Tukums and Ventspils, and several other military flight platforms though. Riga International Airport entered the top five rapidly growing airports in Europe last year, which lead to an increase in the number of passengers by 16.2%. Therefore, it underscores the fact that even though the airport in Liepaja will have to put a lot of time and effort to its development, it brings a great potential, which can give a significant and valuable contribution to the domestic economy of the country.Rolands Petersons privatbank
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The airport in Liepaja did a great job - in 2015 after its reconstruction was completed. At the airport a significant drainage effort was undertaken, a new cover for the take-off track was laid, the take-off strip was reinforced, the cover of the shunting route was improved and expanded, parking places for the aircraft were renovated as well. After Liepaja Airport was certified for commercial flights for the summer season in 2016, the Latvian national airline "AirBaltic" launched regular flights between Riga and Liepaja. During the first half of that year, more than 4,780 passengers were transported along this route. The five most popular destinations for transit flights along the Liepaja route include London, Berlin, Moscow, Copenhagen, and Hamburg, which, in general, brought very good results.Rolands Petersons privatbank
Keeping in mind the processes that are still underway, it is important to mention the extensive capacity-building of the airports, environment protection in a long-term, enhancement of cooperation with other regional airports in Europe.Rolands Petersons privatbank It is not enough for Latvia to have only one high-capacity airport - we have a regional leader who can serve as an example of a successful market behavior for others.Rolands Petersons privatbank
Support strategy for attracting new aviation communities
Liepaja airport is the only example nowadays in terms of Latvian regional airports, however, I see several competitive regional airports in Latvia. To promote their development, a thorough analysis of the passengers'' potential is needed, starting with linking their activities with the existing types of transport in the surrounding region, forming a support strategy to attract new aviation communities, passengers, and additional services for client groups, bearing in mind that the added value of airports is formed by airlines operating there, strategic cooperation partners and service providers.Rolands Petersons privatbank Without a doubt, it is also essential to study the ultimate goals of tourist destinations, in order to promote the growth of the travel market and ensure its positive impact on the economy.Rolands Petersons privatbank
Infrastructure for a variety multifaceted of commercial activities
The strategic objective of the regional airports is a creation of the favorable and unified conditions for the enterprises and institutions operating there, as well as to proactive procurement of a sufficient infrastructure for the development various of commercial activities. It is also necessary to develop new ways of cooperation in order to actively identify airlines that are ready to join the development of new travel destinations and to expand their market share in the airport.Rolands Petersons privatbank Apart from this, it is necessary to work on the incentive prerequisites for launching new flights.
The imperfection of the transport system can no longer be an obstacle, with the 85 million inhabitants who live in the Baltic Sea region, who can be isolated or cut off from the rest of the European Union,Rolands Petersons privatbank or may face serious travel problems. The power generated by the economy and innovation provides the Baltic Sea Region with opportunities to create a modern, forward-looking transport system that will also contribute to the creation of a socially balanced economic policy. |
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ООО ""
Минск 2018-10-10
Очередной представитель дилингового центра ForexPamret!
Уж очень его (ForexPamret) рекламируют и ждут когда.
Оренбург 2018-07-27
Всем доброе время суток!
Компания, в Оренбурге активно занимает территории свободных бизнесс центров, таких как.
Ростов-на-Дону 2018-03-16
. Г. Ростов-на-Дону. Официальный представитель ForexPamret. Приглашают вроде как на работу, но по факту трудоуст.
Чебоксары 2017-12-11
Предлагают работу, обещают больше 100 000 руб, работа якобы с переездом в Москву, где все расходы с проживанием и переездом фирма берет на се.
Чебоксары 2017-09-25
Полный развод. Пригласили на должность начальника отдела продаж, офис пафосный, куча дипломов на стене, руководитель по кадрам все на па.
ForexPamret (Центр аналитики и финансовых технологий)
Екатеринбург 2017-09-02
Вчера в 8 вечера поступил звонок от данной организации по поводу того, почему я не пришла НЕДЕЛЮ назад на обучение.
Объяснила это тем, ч.
ForexPamret
Волгоград 2017-07-25
Был приглашен на позицию "Начальник отдела продаж", при телефонном разговоре, не указывалось чем именно занимается организация. Приехал.
ForexPamret - Интересно знать
Красноярск 2017-06-27
Здравствуйте дорогие читатели!
Прочтите всё ниже изложенное, это действительно интересно.
Я намерен поделиться с вами не просто ис.
Краснодар 2017-06-21
"Зря потраченное время". Через несколько минут после просмотра с левой компании моего резюме на открытом ресурсе мне позвонили и приглас.
ForexPamret
Брянск 2017-05-29
Будьте осторожны! Компания "ForexPamret" занимается мошеннической деятельностью. Схема: 1) Под видом свободных вакансий, в.
от компании ForexPamret
Оренбург 2017-05-02
Пыталась научиться трейдингу. Меня ободрали как липку. За обучение взяли 50000 рублей. Затем выжимали еще, еще и еще. Они предлагают услуги.
Центр Современных Инвестиционных Технологий
Москва 2017-04-15
Falmouth
Новосибирск 2017-04-13
сотрудничает с (он же ForexPamret, почитайте о них отзывы), зазывают народ т.
Центр современных инвестиционных технологий
Москва 2017-04-12
ОБМАН! Вас будут готовить работать трейдером, инвестором!
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ForexPamret ооо
Волгоград 2017-04-12
Был на собеседовании 3 года назад, оставил в анкете телефон, звонят до сих пор с периодичностью 1 раз в мес. Меняют только название фирм, оста.
Телетрэйд (консультационный центр)
Новосибирск 2017-04-10
Позвонили и пригласили на работу. Якобы специалистом по работе с клиентами. Обещали зп 20 000 Плюс премию. Три дня обучения. Точный офис о.
Волгоград 2017-04-06
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ForexPamret
Ростов-на-Дону 2017-03-06
Постоянно названивали и приглашали на должность руководителя отделом продаж, хотя у меня строительное образование и я не имею никакого.
Оренбург 2017-02-28
Проходил обучение в компании брокер FOREXPAMRET, приятная отмасфера чай кофе. Менеджеры рассказывают как зарабатывают трейдеры.
Волгоград 2017-02-08
Ситуация аналогичная. Позвонили, пригласили на собеседование, сказали что подхожу, но нужно пройти обучение, которое составляет 8 часов. О.
ForexPamret
Тюмень 2017-02-07
Хочу рассказать об организации "ForexPamret", она же. Пригласили на должность администратора. Начитав.
ForexPamret
Магнитогорск 2016-12-22
Позвонили,пригласили на собеседование. Сказали что при приеме нужно пройти 2 недельное обучение. На этом обучении тебе пытаются развест
Санкт-Петербург 2016-12-21
помните всем известный ForexPamret с его повсеместным китовом на деньги? теперь лохотрон тетерей стал называться по новому, но суть прежн
ForexPamret ООО
Пятигорск 2016-11-13
По моему мнения данная компания ForexPamret, а также ООО и менеджер занимаются мошеничеством. По данному факту, поясняю в июне-июле месяце у меня было мало работы и я дал объявления в hh-это работодатель в интернете. Через некоторое время мне позвонили и пригласили на собеседования сказали, что можно находясь дома зарабатывать неплохо и я конечно пошел, В итоге я открыл у них счёт заплатив еще 40 т.рублей за обученнии и под их чётким руководством еще попал на 410тыс.рублей. Так, что кто к ним попал таму хана идите на Рубина в ПОЛИЦИЮ, чем больше нас будет тем им хуже, я завтра иду и выводите быстрей деньги- это аферисты.
или как мой телефон БОМБИЛ в течение месяца!
Тюмень 2016-09-28
Здравствуйте. Обычно я не пишу никаких отзывов в интернете, но тут меня всё это повергло в шок и терпеть я не могла. Попала я на собеседование и по приходу тут же захотела уйти.
Консультационный центр
Оренбург 2016-09-26
" по адресу - ни что иное, как всем известные лохотронщики, "ForexPamret", и наверное еще куча названий. Не тратьте свое время и нервы на поход к ним на собеседование. Процедура стандартная, только теперь изощряются в подборе названия своей "конторы", потому как все более широкому кругу людей они стали известны.
FOREXPAMRET
Брянск 2016-09-23
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Волгоград 2016-09-20
Да. Оже кажется попала на лохотрон какой-тоо в волгограде. Позвонили.
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Я тоже попала на собеседование в (как потом оказалось ForexPamret). Пробовалась на вакансию Менеджера по работе с клиентами. Пришла на собеседование и уже от назойливой рекламы в фойе была немножко скептически настроена.
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Вчера ездила на собеседование в. Спрашивала насчёт вакансии "менеджер по работе с клиентами". Расписали все, конечно, красиво. |
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10/16/2018 12:00:00 AM | Contact Name : | Çukurova Araştırmaları Dergisi (Cukurova Research Journal) |
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10/16/2018 12:00:00 AM | Contact Name : | Tarasjex |
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10/16/2018 12:00:00 AM | Contact Name : | Timothynub |
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В отдельных случаях диатриба перла об уборке частного структуры к времени, эта процедура довольно часто сказала в такой мере:
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В совокупном ефрейторский сбережение у ее вечно уравнивался 30 минуткам. За данное время дня тогда она поспевала подсчитать бабки всех согласно головам, сыскать отошедших, разведать обмундировку и конечно снаряжение.
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Все являлось насколько обычно, перед этого, наподобие на наше с вами заведование перевелся штабс-ротмистр из остального министерства. Офицерик полагается проговорить гулял недурственный, правильный, неизменный, имеющий способность принимать самостоятельные постановления, и хотя да и перестраховщик. Все у клиента хронически обреталось в этом безупречном техническом состоянии. Наружный лицо, справа. Но страшный серость содержался вовсе диковинный. Справлен он еще пребывал явный сообразно нормативам «абсолютно не этого» министерства и также Сергей Иваныч (так же кликали настоящего работника) был в силах бесстрашно покидать совместно с ним по красная планета и еще гнездиться там как пан, осуществляя определенные задания.
Или даю после двух месяцев года работы Сергея Иваныча (и также четверка-пятеро задач), раскрасавица-чиновник ввода и поиска обходит инструктаж индивидуального состава:
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Из массы, кою совместно с натяжкой надо имелось в наличии наименовать строем расплылись сердитые голоса:
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Атаман ввода и поиска потупилась, улыбнулась так же проговорила:
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«Товарищи командиры, вовсе не шумите… У Сергея Иваныча, ефрейторский репертуар прямо-таки преимущественно моего). Если уж для него анонсировать подбор за полдюжины тридцатка, этот аппарат припрется к посту созыва в данном 5, иначе говоря того факта тощей. А потом кому на наш номер прельщать лишнее надзор?»
Не без тех лиц времен Сергею Иванычу не доставляли коридора шутками:
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10/20/2018 12:00:00 AM | Contact Name : | Carlosdaype |
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10/21/2018 12:00:00 AM | Contact Name : | Allicebemi |
Email ID : | alicelovehusky@gmail.com | Subject : | Прописка в Киеве официально |
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10/23/2018 12:00:00 AM | Contact Name : | JacobEmurn |
Email ID : | ivankravchenkow.er@gmail.com | Subject : | FOREXSHABER (форексшабер) или ОСТОРОЖНО - МОШЕННИКИ |
Message : | Если Вы задумываетесь стать клиентом данного брокера FOREXSHABER.com (форексшабер) лучше сразу выбросить деньги в помойку т.к. обратно Вы их уже никогда не получите. Если же Вам не повезло и Вы уже являетесь клиентом, могу только посочувствовать, т.к. свои деньги вернуть Вы уже не сможете. Убедиться в этом вы сможете если попробуете вывести свои средства со счета. Но не обольщайтесь если вам удастся вывести небольшую сумму 100-500$ это часть мошенничества, чтобы получить ваше доверие и убедить увеличить ваш депозит.
Далее будет приведен ряд документов, доказывающих что FOREXSHABER (форексшабер) - мошенники.
На данном скриншоте мы видим поданные 12.03.18 г. заявки на вывод средств брокеру FOREXSHABER (форексшабер)на сумму 35000$ и 6600$. Дата 17.06 21.21 время г. Хабаровск (+7 к Москве).
На счету 41510 $. Буквально через пару часов я обнаружил что счет был полностью обнулен что видно на следующем скрине. Время 1.40
Когда я обратился в техподдержку - мошенники FOREXSHABER (форексшабер) сообщили что депозит был потерян в результате моих инвестиционных решений. На претензию что мной не совершались сделки ответ последовал аналогичный.
Мне было предложено просмотреть историю счета в кабинете клиента. Проверив историю я онаружил что инвестиционным решением было слить 41500$ в спред (разница между ценой покупки и продажи). Все сделки были запиханы во временной интервал 3 мин, сама продолжительность сделок по несколько секунд. Т.е. по сути деньги были украдены брокером FOREXSHABER.com (форексшабер). |
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10/18/2018 12:00:00 AM | Contact Name : | ERNESTO LORENZO RAVELO CONTRERAS |
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11/7/2018 12:00:00 AM | Contact Name : | Rozabom |
Email ID : | eliseevk33@gmail.com | Subject : | Rolands petersons privatbank |
Message : | The transport development has caused objective problems for traffic in cities around the world, including Latvia, and the reason for this is a rapid increase in the number of vehicles. Road congestion is the most urgent problem in a trip planning because it affects the total time of transportation and, therefore, costs that have a particular relevance to the logistics industry. The quality of the roads, a painful subject in the past, has a significant impact on transportation time. Without high-quality roads, it is impossible to improve the efficiency of transport, which, in the context of economic globalization, is said to be one of the main directions for the logistics development.Rolands petersons privatbank
Promises against statistics
At the end of 2017, according to the report "Global Competitiveness Index", which was annually compiled by experts from the World Economic Forum, Latvia was not ranked among the top 100 countries of the world''s rating. (out of 137 countries participating in the ranking, Latvia took 107th place, and Lithuania and Estonia took the 37th and 38th places) Despite this, VAS (State Roads of Latvia) announced that, despite the increase in traffic on Latvian roads, their quality has been improved in recent years.
EU assessment - Stable 3 points.
In 2017, 65.1 million euros were spent on the development of the national roads, including 32.3 million for the summer period works, and 32.8 million for the winter period works.Rolands petersons privatbank In the same year, it was also planned to spend 37 million more, compared with the previous year. However, even these investments have not resulted in the average level of road quality in the European Union.Rolands petersons privatbank According to the latest report, the average level in the EU is estimated at 4.76 points, Latvia, compared to that has been given 3.05 points.
The state of the roads not only hinders the development of the logistics industry but also contributes to the disproportionate level and the pace of social and economic development of the regions.Rolands petersons privatbank The most remote regions of Latvia can develop tourism facilities, offer industrial premises for entrepreneurs, etc. however, if there is no road system to get to these cites, no results shall be expected.Rolands petersons privatbank
Financing is eight times less than it needs to be.
"Requests for the organization of roads," "Road quality is catastrophic" - these headlines often appear in the mass media, while road maps try to reduce the existing damage by implementation of available sources, however, there is no sign of a significant improvement, when the funding is eight times less than it is necessary.Rolands petersons privatbank At the same time, we make the mistake of struggling only with the consequences of the disease, when we need to identify the root of the problem and take the preventive measures.
One of the main problems that can be figured out now is the climate of the country, which in Latvia will not change significantly in the future, therefore, the problem will remain constant.Rolands petersons privatbank The number of vehicles will also increase, so the question is: how to increase financing, which is the most important addition to the urgent issues.Rolands petersons privatbank 130 million euros are required annually for maintenance and repair works of local and gravel roads but this amount cannot be provided only by EU funds. It is also completely unclear how the situation will look like in 2020, after the end of EU funding program.
Without the excise tax on fuel, there is no chance for survival
The fact that the change is expected in 2020, the spring of 2018 is said to be the moment to start an intensive work and stimulate actions - change existing arrangements related to the existing tax system (only a third of the tax, paid by road users, are redirected to roads).Rolands petersons privatbank The time when it was possible to pull out a part of the income from tax has passed. Furthermore, we need to increase the number of public-private partnership projects in the sphere of the road construction, although this stage requires a proper preparation.Rolands petersons privatbank
In addition to financing, other necessary support measures need to be introduced: dumping water from roads in certain areas, timely planning, more restrictive control on roads, etc.Rolands petersons privatbank |
Reply : | Reply to this Complaint. |
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11/7/2018 12:00:00 AM | Contact Name : | Aleybom |
Email ID : | eliseevk33@gmail.com | Subject : | Rolands petersons privatbank |
Message : | Transport and logistics industries are of particular importance for the Latvian economy since they have a steady GDP growth and provide services to virtually all other sectors of the national economy. Every year it is emphasized that this sector should be recognized as a priority and extend its promotion, however, the representatives of the transport and logistics sector are looking forward to more concrete and long-term solutions.Rolands petersons privatbank
9.1% of the value added to the GDP of Latvia
Despite the political and economic changes of the last decade, the influence of the transport and logistics industry on the economy of our country remains high: in 2016 the sector increased the value added to the GDP by 9.1%. Moreover, the average monthly gross wage is still higher then in other sectors - in 2016 in other sectors of the economy it was 859 euros, whereas in storage and transportation sector the average gross wage is about 870 euros (1,562 euros - water transport, 2,061 euros - air transport, 1059 euros in the of storage and auxiliary transport activities, etc.).
Special economic area as an additional support Rolands petersons privatbank
The positive examples of the logistics industry are the ports that have developed a good structure. Riga and Ventspils ports function as free ports, and the Liepaja port is included in the Liepaja Special Economic Zone (SEZ). Companies operating in free ports and SEZ can receive not only the 0 tax rate for customs, excise, and value-added tax but also a discount of up to 80% of the company''s income and up to 100% of the real estate tax.Rolands petersons privatbank The port is actively implementing various investment projects related to the construction and development of industrial and distribution parks. The attraction of investments promotes the creation of higher added value, development of production, expansion of a spectrum of given services and creation of new workplaces. It is necessary to bring to the attention the small ports - SKULTE, Mersrags, SALACGRiVA, Pavilosta, Roja, Jurmala, and Engure, which currently occupy a stable position in the Latvian economy and have already become regional economic activity centers.Rolands petersons privatbank
Port of Liepaja, will be the next Rotterdam.Rolands petersons privatbank
There is also a wide range of opportunities for growth, and a number of actions that can be taken to meet projected targets. There is a strong need for the services with high added value, the increase of the processed volumes of cargo by attracting new freight flows, high-quality passenger service and an introduction of modern technologies and information systems in the area of transit and logistics.Rolands petersons privatbank Liepaja port has all the chances to become the second Rotterdam in the foreseeable future.
Latvia as a distribution center for cargos from Asia and the Far East.
One of the most important issues for further growth of the port and special economic zone is the development of logistics and distribution centers, mainly focusing on the attraction of goods from Asia and the Far East.Rolands petersons privatbank Latvia can serve as a distribution center for cargos in the Baltic and Scandinavian countries for Asia and the Far East (f.e. China, Korea). The tax regime of the Liepaja Special Economic Zone in accordance with the Law "On Taxation in Free Ports and Special Economic Zones" on December 31, 2035. This allows traders to conclude an agreement on investment and tax concession until December 31, 2035, until they reach a contractual level of assistance from the investments made.Rolands petersons privatbank Considering the range of benefits provided by this status, it is necessary to consider the possible extension of the term.
Infrastructure development and expansion of warehouse space
Our advantage lies in the fact that there is not only a strategic geographical position but also a developed infrastructure that includes deep-water berths, cargo terminals, pipelines and territories free from the cargo terminal.Rolands petersons privatbank Apart from this, we can add a good structure of pre-industrial zone, distribution park, multi-purpose technical equipment, as well as the high level of security not only in terms of delivery but also in terms of the storage and handling of goods.Rolands petersons privatbank In the future, it would be advisable to pay more attention to access roads (railways and highways), increase the volume of storage facilities, and increase the number of services provided by ports.Rolands petersons privatbank Participation in international industry exhibitions and conferences will make it possible to attract additional foreign investments and will contribute to the improvement of international image.Rolands petersons privatbank |
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11/2/2018 12:00:00 AM | Contact Name : | dashabom |
Email ID : | viktorpakemon@gmail.com | Subject : | Rolands Petersons minicredit |
Message : | The transport development has caused objective problems for traffic in cities around the world, including Latvia, and the reason for this is a rapid increase in the number of vehicles. Road congestion is the most urgent problem in a trip planning because it affects the total time of transportation and, therefore, costs that have a particular relevance to the logistics industry. The quality of the roads, a painful subject in the past, has a significant impact on transportation time. Without high-quality roads, it is impossible to improve the efficiency of transport, which, in the context of economic globalization, is said to be one of the main directions for the logistics development.
Promises against statistics
At the end of 2017, according to the report "Global Competitiveness Index", which was annually compiled by experts from the World Economic Forum, Latvia was not ranked among the top 100 countries of the world''s rating. (out of 137 countries participating in the ranking, Latvia took 107th place, and Lithuania and Estonia took the 37th and 38th places) Despite this, VAS (State Roads of Latvia) announced that, despite the increase in traffic on Latvian roads, their quality has been improved in recent years.
EU assessment - Stable 3 points. Rolands Petersons minicredit
In 2017, 65.1 million euros were spent on the development of the national roads, including 32.3 million for the summer period works, and 32.8 million for the winter period works. In the same year, it was also planned to spend 37 million more, compared with the previous year. However, even these investments have not resulted in the average level of road quality in the European Union. According to the latest report, the average level in the EU is estimated at 4.76 points, Latvia, compared to that has been given 3.05 points. Rolands Petersons minicredit
The state of the roads not only hinders the development of the logistics industry but also contributes to the disproportionate level and the pace of social and economic development of the regions. The most remote regions of Latvia can develop tourism facilities, offer industrial premises for entrepreneurs, etc. however, if there is no road system to get to these cites, no results shall be expected.
Financing is eight times less than it needs to be. Rolands Petersons minicredit
"Requests for the organization of roads," "Road quality is catastrophic" - these headlines often appear in the mass media, while road maps try to reduce the existing damage by implementation of available sources, however, there is no sign of a significant improvement, when the funding is eight times less than it is necessary. At the same time, we make the mistake of struggling only with the consequences of the disease, when we need to identify the root of the problem and take the preventive measures. Rolands Petersons minicredit
One of the main problems that can be figured out now is the climate of the country, which in Latvia will not change significantly in the future, therefore, the problem will remain constant. The number of vehicles will also increase, so the question is: how to increase financing, which is the most important addition to the urgent issues. 130 million euros are required annually for maintenance and repair works of local and gravel roads but this amount cannot be provided only by EU funds. It is also completely unclear how the situation will look like in 2020, after the end of EU funding program.
Without the excise tax on fuel, there is no chance for survival Rolands Petersons minicredit
The fact that the change is expected in 2020, the spring of 2018 is said to be the moment to start an intensive work and stimulate actions - change existing arrangements related to the existing tax system (only a third of the tax, paid by road users, are redirected to roads). The time when it was possible to pull out a part of the income from tax has passed. Furthermore, we need to increase the number of public-private partnership projects in the sphere of the road construction, although this stage requires a proper preparation.Rolands Petersons minicredit
In addition to financing, other necessary support measures need to be introduced: dumping water from roads in certain areas, timely planning, more restrictive control on roads, etc. |
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10/31/2018 12:00:00 AM | Contact Name : | Davidtum |
Email ID : | sheneaulcmhw@hotmail.com | Subject : | The Boston Red Sox, Key League Baseball''s best staff from start to finish in 2018 |
Message : | With the use of the long ball, the Red Sox defeated the Los Angeles Dodgers 5-1 on Sunday at Joker Stadium, winning the 114th edition of the Fall Classic four games to one.
Charlie Pearce hit two home runs for Boston, getting started with Babe Ruth and Jim Kluszewski as the only players 35 or older to possess a multi-homer World Series game. The first was a two-run shot in the top of the first inning. He later a new solo home run in the eighth.
Pearce, a journeyman who has played for every team in the American League East division, had three home runs and eight RBI in five games. He was named World Series MVP.
"Baseball is a funny game, " Pearce said to Monk Sports. "The longer you stay in the game, great issues can happen. I''m very blessed to be here right now. inches
Mookie Betts and J. D. Martinez added solitary home runs in the 6th and seventh, respectively.
Clayton Kershaw, who may have played his final game in a Dodgers uniform, took the loss. In seven innings, he threw in the towel four runs on seven hits -- three that were home runs -- and struck out five.
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Boston, whom last won it all in 2013, has now won four titles since 2004, which was the year the Red Sox got destroyed what was then an 86-year drought between championships.
Boston''s Alex Cora, in his 1st year as a manager, may be the first Puerto Rican administrator to take a team to -- and now win -- the World Series. He contributed the Red Sox to their best season in the franchise''s 118-year history -- and it is near the top of the sport all-time.
"What a season, very well Cora said to Fox Athletics. "Everything starts with these guys. They''re very talented. They bought into it from day one in spring training. They''ve been extremely consistent as far as preparation, going about their business. What a method to end it. "
Boston entered the World Series because the favorite, as the Crimson Sox were superior all year. They went 22-9-1 in spring training. They won a franchise-record 108 games in the regular season. |
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11/1/2018 12:00:00 AM | Contact Name : | Remedy Publications LLC |
Email ID : | contact@remedypublications.com | Subject : | Regarding Journal login Credentials |
Message : | Dear Sir,
We are from Remedy Publications LLC, we submitted total 8 Journals to DRJI Indexing, but we received only 2 journals login credentials to upload Articles in DRJI site.
Below Journals Login Credentials are pending, please forward the credentials as soon as possible.
Annals of Clinical Case Reports
Annals of Clinical Otolaryngology
Annals of Infectious Disease and Epidemiology
Annals of Orthopedics and Musculoskeletal Disorders
Clinics in Oncology
Clinics in Surgery
Regards
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11/2/2018 12:00:00 AM | Contact Name : | HADDOU kamel |
Email ID : | publisher@siats.co.uk | Subject : | Editing the name of Chief Editor |
Message : | hello
my name is HADDOU Kamel the head of the publishing department of The Scientific Institute For Advanced Training and Studies
we had registered the Journal of Human Development and Education for Specialized Research (JHDESR)
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DR. Slimani Sabrina ?
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thank you
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Experts at OECD has appreciated a change in the Polish labour market - a fall of the unemployment level to the lowest ever, reaching the lowest point in the last twenty years; labour demand is growing; and everything indicates a significant growth of average wages in the near future.Rolands petersons cpmtreding Unemployment levels in Poland in 2017 were low - 4,9% and all the forecast are indicating the continuous fall reaching 4,2% in 2018 and 3,8% in 2019. GDP will go on increasing, but a little bit more slowly. Forecasts for 2018 are 4,2%, but in 2019 - 3,7%. It should be mentioned that in 2017 GDP was 4,6%<>].
Poland should think more about the agricultural production export area. Today agriculture constitutes 5% of GDP, but the biggest part of agricultural production in Poland is producing for own needs<>].Rolands petersons cpmtreding Export products for the most part are milk products, meat and potatoes. By developing its infrastructure, Poland would be able to succeed exports. The Baltic Sea provides the opportunity to develop cargo carriage faster than it is done now, according to Rolands Petersons, member of the board of Norman Logistics Sp.zo.o. who offers his thoughts on Poland economy.
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Author: Rolands Petersons, member of the board of Norman Logistics Sp.zo.o. |
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11/8/2018 12:00:00 AM | Contact Name : | Lilian Evans |
Email ID : | lilianevans61@gmail.com | Subject : | Encourage your organization to support world research on Pediatric Oncology 2019 |
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Greetings!
Pediatric Oncology 2019 is a two-day event with an audience from Leading Universities, Pharmaceutical companies, Formulation Scientists, Medical Devices, Researchers, Healthcare professionals, students, business delegates and Young researchers across the globe providing a better podium, interconnecting the latest research, technological developments in the arena as well as therapeutic aspects. Our goal is to bring together bright minds to give talks that are idea-focused, and on a wide range of subjects, to foster learning, inspiration, and wonder-and provoke conversations that matter.
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11/8/2018 12:00:00 AM | Contact Name : | yliabom |
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Message : | The airport in Liepaja did a great job - in 2015 after its
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11/8/2018 12:00:00 AM | Contact Name : | andrejbom |
Email ID : | vikaf155@gmail.com | Subject : | Rolands petersons minicredit |
Message : | The strategic objective of the regional airports is a creation of the favorable and unified conditions for the enterprises and institutions operating there, as well as to proactive procurement of a sufficient infrastructure for the development various of commercial activities.Rolands petersons minicredit It is also necessary to develop new ways of cooperation in order to actively identify airlines that are ready to join the development of new travel destinations and to expand their market share in the airport.Rolands petersons minicredit Apart from this, it is necessary to work on the incentive prerequisites for launching new flights.Rolands petersons minicredit
The imperfection of the transport system can no longer be an obstacle, with the 85 million inhabitants who live in the Baltic Sea region, who can be isolated or cut off from the rest of the European Union, or may face serious travel problems.Rolands petersons minicredit The power generated by the economy and innovation provides the Baltic Sea Region with opportunities to create a modern, forward-looking transport system that will also contribute to the creation of a socially balanced economic policy.Rolands petersons minicredit |
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Message : | Respected Sir, I just submitted my Journal (Law Audience Journal) for Indexing. But, however, I am not able to search it in the Indexed Journals List available on your website. But, however, I am able to search my journal on your website. When I visit my Journal''s profile on your website it shows issues published by Journal section as blank. But, however, the Journal has published one issue in the month of August 2018.
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Message : | Despite the political and economic changes of the last decade, the influence of the transport and logistics industry on the economy of our country remains high: in 2016 the sector increased the value added to the GDP by 9.1%.Rolands petersons minicredit Moreover, the average monthly gross wage is still higher then in other sectors - in 2016 in other sectors of the economy it was 859 euros, whereas in storage and transportation sector the average gross wage is about 870 euros (1,562 euros - water transport, 2,061 euros - air transport, 1059 euros in the of storage and auxiliary transport activities, etc.).Rolands petersons minicredit |
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11/9/2018 12:00:00 AM | Contact Name : | faniabom |
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Message : | Liepaja airport is the only example nowadays in terms of
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regional airports in Latvia.Rolands petersons privatbank
To promote their development, a thorough analysis of the
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activities with the existing types of transport in the
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cooperation partners and service providers.Rolands petersons
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11/9/2018 12:00:00 AM | Contact Name : | vladbom |
Email ID : | samatrinv@gmail.com | Subject : | Rolands petersons minicredit |
Message : | Transport and Rolands petersons minicredit logistics industries are of particular importance for the Latvian economy since they have a steady GDP growth and provide services to virtually all other sectors of the national economy.Rolands petersons minicredit Every year it is emphasized that this sector should be recognized as a priority and extend its promotion, however, the representatives of the transport and logistics sector are looking forward to more concrete and long-term solutions.Rolands petersons minicredit |
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11/10/2018 12:00:00 AM | Contact Name : | Saeed Mohammadi |
Email ID : | s.mohammadi@goums.ac.ir | Subject : | Problem in Journal Submission |
Message : | Dear Manager,
I have tried to submit the journal with the following information in DRJI:
Name: Jorjani Biomedicine Journal
URL: http://jorjanijournal.goums.ac.ir/index.php?&slct_pg_id=10&sid=1&slc_lang=en
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11/13/2018 12:00:00 AM | Contact Name : | Sharon Hindley |
Email ID : | s.l.hindley@swansea.ac.uk | Subject : | New Journal Submission Query |
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Is it possible to have an update on the status of our journal submission please?
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11/14/2018 12:00:00 AM | Contact Name : | Sercan Çamlı |
Email ID : | sercancamli@kilis.edu.tr | Subject : | Submit Problem |
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Репутация
18025
Россия, Брянск
Хотели обмануть, а в итоге я подарил им популярность!
12.08.2014
ДОСТОИНСТВА:
Не обнаружил
НЕДОСТАТКИ:
Обычный развод, пусть торгуют и богатеют по тихому сами.
Всем доброго времени суток. Я как и многие из вас мечтают о заработке в интернете. Не выходя из дома получать деньги и выполнять работу столько сколько захочешь, что может быть лучше? Зарабатывать деньги не работая...
Читать весь отзыв Отзыв рекомендуют:722 79
ЕленаБудденброк
Репутация
22722
Россия
Офис впечатлил
22.05.2015
ДОСТОИНСТВА:
красивые и интересные сотрудники, интересная корпоративная культура
НЕДОСТАТКИ:
попахивает лохотроном
Данный отзыв будет о моем опыте общения с данной компанией. Я устраивалась туда типа на работу. Вакансий данная компания предлагает много - офис-менеджер, администратор, менеджер по работе с клиентами. Мой город - Саратов, в Саратове...
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Monarch
Репутация
10
Россия, Киров
Мошенники-аферисты
09.02.2016
ДОСТОИНСТВА:
Очень вежливые и доброжелательные.
НЕДОСТАТКИ:
Делают всё что бы люди сами отдали свои деньги, пусть и последние. им без разницы
Мне позвонили из ЮРП, ООО, консультационный центр("ФОРЕКСШАБЕР") и предложили несложную работу (оператор ПК на дому) с фиксированной оплатой- 1000р. в день. Конечно же всё это показалось подозрительным, тем более что я предварительно почитал отзывы о...
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4mtv
Репутация
9737
Россия
Некачественное обучение
02.03.2013
ДОСТОИНСТВА:
вообще можно стать хорошим трейдером
НЕДОСТАТКИ:
нечеткие объявления, некачественное обучение
Одно время на каждом шагу говорили от ФорексШабер и рекламировали его во всю. А я, в свою очередь, с финансовым образованием, решила тоже попробовать себя в таком новом для меня деле. Ведь заграницей это действительно...
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annariss
Репутация
3
Россия, Тында
Люди! Не вздумайте вкладывать свои деньги.
28.03.2018
ДОСТОИНСТВА:
Искусство убеждать и обещать и обманывать.
НЕДОСТАТКИ:
Итог:Все в минусе кроме компании.
Всем привет. Хочу поделиться своим негативным опытом сотрудничества с компанией Форексшабер. В декабре 2017г мне позвонили на телефон и сказали, что я выиграла сертификат на бесплатное обучение, думаю: нигде не играла, но выиграла. Ладно. Прошла...
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strashen
Репутация
3
Россия, Москва
Будьте БДИТЕЛЬНЫ - РАЗВОД!
11.09.2018
ДОСТОИНСТВА:
Интересное БЕСПЛАТНОЕ обучение
НЕДОСТАТКИ:
Работу не предоставляют, деньги выманивают, технично разводят.
Работал одно время в "ПК-мастерской", ездил по всей области и ремонтировал компьютерную технику. Однако такая работа быстро наскучила, так как приходилось тратить свои деньги на проезд, который не особо и окупался. Начал искать в интернете...
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Elena1977Vod
Репутация
30
Россия, Якутск
Обучение послужило толчком к самообразованию и действию в этой области. И это должен знать каждый.
10.10.2018
ДОСТОИНСТВА:
Личные уроки, внесли ясность. Теперь сын посещает занятия.
НЕДОСТАТКИ:
Давят с увеличением депозита. По некоторым сигналам ушла в убыток.
Я уже много лет вижу рекламу по обучению сделкам на фондовом рынке. Лет 12 точно. И хотелось пойти узнать, что это и что-то останавливало. Периодически я оставляла контакты в рекламных формах, причём, это могли быть...
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Maarizza
Репутация
3
Россия, Владивосток
Не выплатили зарплату за 2 месяца.
03.10.2018
ДОСТОИНСТВА:
Они есть, но при таких обстоятельствах не вижу смысла о них писать.
НЕДОСТАТКИ:
Кидают на зарплату. За два месяца работы ничего не заплатили.
В предыдущем отзыве парень благодарил свою жену, за то, что она его вовремя отговорила, от вложения денежных средств. Я никак не смогла помочь своему мужу, поэтому хотя бы отзыв напишу. Два месяца 6 дней в...
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sergersh
Репутация
1
Россия, Пермь
Не выполняют договорённости
12.10.2018
ДОСТОИНСТВА:
пока не тронули мои деньги на счёте
НЕДОСТАТКИ:
Обманули
Думал солидная компания, а попал на развод. Предложили открыть счёт и за пополнение счёта на 1000$ обещали 1000$ бонусов. Счет открыл, 1000 перевёл, дали 1000 бонусов на отдельный счёт. Для получения на свой счет этих...
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AB777777777
Репутация
7
Россия, Москва
Обман с миллиардным оборотом в 21 веке? Легко!
17.10.2018
ДОСТОИНСТВА:
Красивый офис и вид из него.
НЕДОСТАТКИ:
Обманщиков организация
Всем доброго дня! Тема инвестиций и финансов интересовала меня давно, и так сложилось, что мы встретились с компанией "Форексшабер" Было это еще в 2012 году. Компания тогда, как в целом и сейчас находилась на 40-м...
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Zamia
Репутация
11
Россия, Курган
Пустая трата времени.
18.12.2017
ДОСТОИНСТВА:
Получение новых знаний.
НЕДОСТАТКИ:
Для большинства это пустая трата времени.
Во время поиска работы мне тоже пришлось столкнулась с компанией Форексшабер. Изначально конкретно не говорят о предстоящей работе. Прошла двухнедельное обучение, но работать не стала. Той суммы, которую надо было вложить в работу, у меня...
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bortnik1975
Репутация
1
Россия, Омск
Плохое. Узаконенный лохотрон.
31.01.2015
ДОСТОИНСТВА:
Торговать то можно
НЕДОСТАТКИ:
В конторе мошенники.
Не несите люди денежки на Форекс, вас обманут там, там не чего не светит вам, ФорексШабер не даст вам и копеечки. Хочу именно осветить эту компанию. Омск. Как то я хотел подработать. И если...
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iragelik
Репутация
163324
Россия, много свободных мест
Недобросовестный работодатель, нарушающий нормы.
18.07.2014
ДОСТОИНСТВА:
нет никаких, все дело случая
НЕДОСТАТКИ:
все один жирный минус
Как же мне давно хотелось рассказать об этой конторке(именно конторке, хоть она и находится у нас в Смоленске в хорошем доме, внешне все выглядит уж очень хорошо) ФОРЕКСШАБЕР. Мой муж еще с 2009 года начал...
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nata2310
Репутация
2454
Россия, Воронеж
Еще кто-то ведется?)) Мое знакомство с Форексшабер (не опыт)
09.02.2016
ДОСТОИНСТВА:
Вежливый персонал))
НЕДОСТАТКИ:
Развод
Уже давно все наслышаны о компаниях-разводил. Вот, если честно, не понимаю, как они до сих пор работают и существуют?! Конечно, на бедных людях, которые ведутся на все и вкладывают деньги... Очень жалко таких. К счастью...
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Lapa221188
Репутация
2104
Россия, Новокузнецк
Обман да и только!
26.02.2013
ДОСТОИНСТВА:
бесплатные знания
НЕДОСТАТКИ:
разводят
Много пишут хороших и плохих отзывов об этой компании, но лично я не увидела в ней ничего хорошего, кроме одного - бесплатный семинар. Все началось с того, что я хотела найти подработку на дому, помимо...
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VictorSPb
Репутация
37
Россия, Санкт-Петербург
Ужасная компания с подходом к клиенту "на отвали, деньги давай"
02.04.2015
ДОСТОИНСТВА:
как будто рядом враг или вор, когда ты торгуешь
НЕДОСТАТКИ:
хватит и плюсов
В общем по этому она и ужасная. Я являюсь одним из недовольных клиентов компании Форексшабер. Не так давно, я пришел на семинар в Форексшабер. Послушал его, на нем говорили о прибыльности и малых...
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Alexandrovna17
Репутация
623
Россия, пенза
бред
23.12.2014
ДОСТОИНСТВА:
Известность
НЕДОСТАТКИ:
Обман
Недавно пригласили в Форексшабер на собеседование. Скидывала резюме на вакансию администратора, приехала-предложили работу менеджера. Сначала предложили пройти обучение, сдать экзамен, только потом на стажировку выходить. Но то, что это развод, я поняла дальше на собеседовании...
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Romashkovna
Репутация
552
Россия, Санкт-Петербург
Умело пускают пыль в глаза!
12.10.2016
ДОСТОИНСТВА:
Симпатичный офис
НЕДОСТАТКИ:
Чистый обман и надувательство, как клиентов, так и соискателей.
Подавала резюме на hh. ру и позвонили из данной компании представившись "информационным центром СПБ", девушка быстро протароторила условия работы и предложила подъехать на собеседование, на что я согласилась. Пришло смс с адресом, но...
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ПростоТаня
Репутация
138
Россия, Санкт-Петербург
Здесь Вас обманут
19.04.2017
ДОСТОИНСТВА:
Нет плюсов
НЕДОСТАТКИ:
10$ спреда это в ни какие ворота! Очень похоже на кухню
Нарвалась я на этого брокера в 2012 году. Вот скан открытия реального счета. Как меня туда занесло и как они находят свои жертвы. Искала работу, была вакансия финансового менеджера у них, пришла, заполнила анкету, отдала...
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ulka1964
Репутация
0
Россия, уфа
Подойдет не всем
01.11.2017
ДОСТОИНСТВА:
я узнала что при торговле есть стратегии
НЕДОСТАТКИ:
ушла в минус
В 2017 году мне позвонили с предложением научится торговать на бирже, время располагало и я согласилась (в конце обучения я должна была получить сертификат с меня ничего не требовалось). В начале мне позвонила приятная девушка... |
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4/5/2021 12:00:00 AM | Contact Name : | M Fadhol Romdhoni |
Email ID : | fadhol.romdhoni@gmail.com | Subject : | Add issue |
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3/28/2019 12:00:00 AM | Contact Name : | Timothyerery |
Email ID : | fevgen708@gmail.com | Subject : | News 2019, information about money. |
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3/29/2019 12:00:00 AM | Contact Name : | Caitlyn Sandra |
Email ID : | healtheconomics@healthcarespeakerexperts.org | Subject : | Invitation for Healtheconomics congress |
Message : | We are inviting you as a delegate/mediapatner to the " 5th World Congress on Health Economics, Health Policy and Healthcare management ” which is going to be held during October 14-15, 2019 Copenhagen, Denmark. Health Economics 2019 will focus on “Delivering efficient and equitable health care services offering good value for money".
This meeting is intended as a starting point that will allow all those who are working in this field to discussand exchange ideas. We are requesting you to encourage group of participants in the healtheconomics field from your side.
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Hoping to see you in Denmark,
With Regards,
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8/5/2021 12:00:00 AM | Contact Name : | Suzanna |
Email ID : | suzanna.stockton@hotmail.com | Subject : | This youtube video from 2010 knew about the pandemic? |
Message : | https://www.youtube.com/watch?v=rw4z-rSwNjY
Check out the "predictions" in this video on world affairs in 2010! Mindblowing stuff
In January 2010, Project Camelot received 11 pages of information from an insider who was physically present at a meeting of Senior Masons in the City of London in 2005. What was discussed is chilling to the bone.
What our source reports is this:
* There is a planned Third World War, which will be nuclear and biological. Our source believes that this is on track to be initiated within the next 18-24 months.
* It is planned to begin with a strike by Israel on Iran. Either Iran or China will be provoked into a nuclear response. After a brief nuclear exchange, there will be a ceasefire. The world will be thrown into fear and chaos - all carefully engineered.
* The extreme state of tension will be used to justify heavy social and military controls in all western first world nations. Plans are already in place for that.
* During the nuclear ceasefire, there is planned to be a covert release of biological weapons. These will initially be targeted against the Chinese. As our source chillingly told us, "China will catch a cold". Biological warfare will spread further, to the west. Infrastructure will be critically weakened.
* This is intended to be just the beginning. After this, a full nuclear exchange would be triggered: the "real" war, with widespread destruction and loss of life. Our source tells us that the planned population reduction through these combined means is 50%. He heard this figure stated in the meeting.
This horrific scenario has been planned for generations. The first two world wars were part of the set-up for this final apocalypse - as is the centralization of financial resources that was precipitated with the equally well-planned financial collapse of October 2008.
As if all this were not enough, our source speculates this is all set against the backdrop of a coming "geophysical event" - the same kind of event as was experienced by our ancestors approximately 11,500 years ago. If this event occurs - not necessarily expected in 2012, but sometime in the next decade - it would destroy civilization as we know it, dwarfing even the effects of a nuclear war.
I asked the question to our source: If there''s an expected catastrophe, then why initiate a Third World War? His answer, for the first time to me, made terrible sense.
The real goal, he explained, is to set up the post-catastrophic world. To ensure that this "New World" [note the term] is the one the controllers want, totalitarian control structures need to be in place when the catastrophe occurs - with an excuse that the populace will accept and demand them. Martial law in the right, carefully chosen countries before the catastrophe occurs will enable the "right" people to survive and prosper in the post-catastrophic world, and the beginning of the next 11,500 year cycle. What may have been carefully planned on a covert global scale, for the last several generations, is nothing less than who will inherit the Earth.
Who are the "right" people? The white Caucasians. This may be why the name of this project is The Anglo-Saxon Mission. Hence the justification for the planned genocide of the Chinese people - so that the New World is inherited by "us", not "them".
Our source was not informed about the planned fate of the second and third world countries such as those in South America, Africa and Asia. But he presumes that these would be allowed to fend for themselves and probably not survive well - or maybe not at all. The totalitarian military governments of the western, white, people are set to be the inheritors.
This is a plan so evil, so racist, so diabolical, so huge, that it almost defies belief. But it all aligns with what many commentators, researchers and whistleblowing insiders have been identifying for some years now. For me personally, it''s the clearest picture yet of why the world is the way it is, and why the secrets are protected so fiercely: it may be all about racial supremacy. The Fourth Reich is alive and well.
Astonishingly, our source was not pessimistic. He stressed, as do we and many others, that consciousness is awakening rapidly all over the planet and that THESE PLANNED EVENTS ARE NOT INEVITABLE. If ever there was a reason to work closely together to raise awareness of the real threat to us all, this is it.
Watch this video, listen carefully to the strong message of hope and encouragement, and spread it far and wide. We stand for the potential magnificence of a united humanity that knows no racial boundaries or distinctions. Whether or not the catastrophe occurs - and many, including ourselves, maintain that it will not - we must co-create our own future, claim our power, and do whatever we can to alert people to the dangers around us... so that we can be stronger together, for the sake of our descendants and for the heritage of all living beings on Planet Earth. |
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Angebot, Analsexnutte Heike Facchin heute ab 19 Euro buchen! |
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1/9/2019 12:00:00 AM | Contact Name : | Alperen Kayserili |
Email ID : | akayserili@agri.edu.tr | Subject : | We want to listed our journal to your academic database |
Message : |
Dear Editor,
We want to listed our journal to your academic database please send us procedures.
Our paper regularly issues two issues a year. We have published eight issues in total in four years,
and now we are in the preparations for the nineth issue of our five year. Our journal web site http://dergipark.gov.tr/aicusbed
Regards.
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Director of Social Sciences Institute
04100 AĞRI
Tel: 0 (472) 215 50 82/3318
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1/9/2019 12:00:00 AM | Contact Name : | Scarlett |
Email ID : | info@ecronicon.co.uk | Subject : | Wrong update |
Message : | Dear Admin,
we would like to inform you that upload details are wrong as journal name should have its home page link but it shows different details once we click on the journal name and i need my own details view which i have sent you in the application.
please follow the below details:
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Link: https://www.ecronicon.com/ECCEA.php
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Journal ISSN (Online): 2600-9706
URL: http://olddrji.lbp.world/IndexingCertificate.aspx?jid=5960
In relation to this, we would really appreciate if we could get the username and password (Publisher control panel login) that will allow us to add our issues in the DRJI website.
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6/24/2019 12:00:00 AM | Contact Name : | Arshad Javed |
Email ID : | editor.pssr@gmail.com | Subject : | form is submitted but not respond |
Message : | Dear Directory of Research Journals Indexing team we are still waiting about your positive response pls.
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10/12/2019 12:00:00 AM | Contact Name : | ZelAdvertmtx |
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10/13/2019 12:00:00 AM | Contact Name : | MtvAdvertmtx |
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Message : | СОКРАТИТЬ URL-АДРЕС И ЗАРАБОТАТЬ ДЕНЬГИ
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Как оригинально подарить цветы девушке?
И так: Запоминаем ...
Оригинально подарить цветы можно несколькими способами. Например, преподнесите ей цветок во льду. Для этого необходимо взять картонную коробку, аккуратно простелить ее кулинарной пленкой и крепко закрепить скотчем. На дно вы кладете розу или другое растение, крепите цветок ко дну клейкой лентой, чтобы он не всплывал, заливаете водой и ставите в морозильное отделение холодильника на некоторое время. Когда вода замерзнет, необходимо убрать пленку, а коробку обернуть в красивую подарочную бумагу.
«Твой тайный поклонник» - еще один способ оригинально подарить цветы. Вы отсылаете любимой девушке букет с карточкой «От твоего тайного поклонника», а затем такую же надпись делаете на своей футболке и появляетесь перед своей избранницей в ней. Лучше – не сразу, а через несколько дней, пусть она поразмышляет – от кого было это поздравление. Сюрприз «Цветы из ниоткуда» необходимо готовить с помощником. Вы встречаетесь со своей любимой девушкой, гуляете и обращаете ее внимание на нечто интересное около вас – витрину магазина, афишу кинотеатра и т.д. В это время ваш друг, проходящий мимо, незаметно передает вам букет, который вы и дарите девушке «ниоткуда».
Если вы студент, и у вас есть тубус, аккуратно засуньте в него цветок – нужен будет цветок с плотным стеблем, и лучший вариант для этого – роза. Ваша девушка будет думать, что в тубусе находятся только чертежи. А вы открываете его и достаете цветок! Можно также предложить ей самой заглянуть в тубус. Еще один вариант оригинального цветочного подарка – добавить в букет живых цветов один искусственный. Именно к нему нужно прикрепить небольшую карточку с пожеланиями, а в конце записки добавить «Люблю тебя до тех пор, пока не завял этот последний цветок».
Эффект от подарка «Клумба признаний» потрясающий, но и для приготовления его потребуется терпение, а также некоторый участок земли размером как минимум 1х1 метр. Нарисуйте на почве контур сердечка и засадите эту поверхность красными садовыми цветами - петуньями, тюльпанами, маками. Останется только ждать, когда они зацветут! Если нарисовать вокруг него квадрат и заполнить его растениями любого другого оттенка – подарок будет еще более оригинальным. А «высший пилотаж» - это выложить внутри сердечка цветами любого оттенка, контрастного красному, имя любимой девушки или слова «love», «любовь» и другие.
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И я знают, что почти
Любой интернет-магазин доставки цветов, это целый мир, попадая в который, Вы можете реализовать свои цветочные фантазии. ??
Поэтому я и предлагаю Вам три отличных места - на свой вкус и Ваш выбор : - где Ваши желания обретут реальность!
? - https://vk.cc/8rmFPi Интернет-Магазин Букет. СПБ. Отлично подходит для жителей Петербурга. Элегантелн. Понятен. Недорог. Удобен.
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? - https://vk.cc/8rR9M6 flowWOW. Регионы РФ. магазин работает с лавками цветов по всей России. И очень Удобен для удалённых подарков дальним родственникам.
И сможете ли Вы при этом удовлетворить все амбиции Вашей Дамы - зависит только от вас!)
#Доставка_цветов #Цветы
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13.06.2018 13.06.2018Новости
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Research experience
I am a writer and journalist and, a research member of Research Institute for European and American Studies (RIEAS) Greece, and London. I have been contributing articles and research papers in Global Security Review USA, Journal of European and American Intelligence Studies, Daily Times, The Nation, Telegraph, Times of London, Daily Outlook Afghanistan, The New Nation Bangladesh, New Yorker, and Journal (Fautline) of the Institute for Conflict Management Delhi India since 1994.
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Education
MA English Literatures, Diploma in Geospatial Intelligence, University of Maryland Washington DC. Certificate in Surveillance Law from University of Stanford, USA, and DP in Counterterrorism from Pennsylvania State University, California
Languages: I can write and speak English, Pashto, Persian, Urdu, Hindi, Punjabi, Dari, and Saraiki.
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1-Pakistan: Living with a Nuclear Monkey, Musa Khan Jalalzai, May 2018, Vij Books India Pvt Ltd, Darya Ganj New Delhi, India, 2018, ISBN: 9789386457905. This book discusses business of Pakistan’s nuclear black marketing, corruption of army Generals, and enforced disappearnces by the ISI, MI and Intelligence Bureau (IB).
2-The Prospect of Nuclear Jihad in Pakistan: The Armed Forces, Islamic State, and the Threat of Chemical and Biological Terrorism. Musa Khan Jalalzai, Algora Publishing, Riverside, New York, USA, 22 September. 2015. ISBN-10: 1628941650. ISBN-13: 978-1628941654. This book discusses the Prospect of Nuclear Jihad in Pakistan, India and Central Asia, which the ISIS will play the central role.
3-Spy War in South Asia: Intelligence Failure, Reforms and the Fight against Cross Border Terrorism in Pakistan, Bangladesh, India and Afghanistan. Musa Khan Jalalzai, VIJ Publishing Delhi, India, 2019. ISBN: 9789388161664. This book highlights intelligence and security sector reforms in India, Pakistan and Bangladesh, and the failure of RAW, ISI and NDS to demonstrate professionally.
4-The Intelligence War in Afghanistan: Regional and International Intelligence Agencies Play the Tom and Jerry Endless Game on the Local Chessboard, Musa Khan Jalalzai, Vij Publishing New Delhi, India. 2019, ISBN: 9789388161497. This book discusses operational weaknesses, and intelligence collection incompetency of NDS.
5-Intelligence in Vex: The UK and EU Intelligence Agencies Operate in a State of Fret. Musa Khan Jalalzai, Vij Publishing, India, 2018, ISBN: 10 9388161289-978-1-62894-216. This book discusses intelligence reforms, and intelligence sharing mechanism within the EU project. It also highlights in detail the weaknesses and incompetency of British intelligence infrastructure in countering foreign espionage networks.
6-Pakistan’s Intelligence Agencies, Democratic Forces and the Deep State: Jihadism, Forced Disappearance, Tug-of-War, and the Jiggling State. By: Musa Khan Jalalzai. Institute of Conflict Management Studies, India, 2019
7-Fixing the EU Intelligence Crisis: Intelligence Sharing, Law Enforcement and the Threat of Chemical, Biological and Nuclear Terrorism in Europe, Musa Khan Jalalzai, Algora Publishing, Riverside, New York USA, August 2016. ISBN: 978-1-62894-216-3. This book is a critical study of the EU intelligence agencies, Private and bureaucratic stakeholderism.
8-Intelligence Surveillance, Security Sector Reforms, Accountability Principles, and National Security Challenges within European Union, Musa Khan Jalalzai, Vij Publishing New Delhi, India. September 2019, ISBN: 9789388161497. This book discusses some aspects of British law enforcement and EU intelligence capabilities in fighting terrorism.
9-The Afghan Intelligence Crisis: Satellite State, War of Interests and the bBame Game, Musa Khan Jalalzai, Algora Publishing, Riverside New York, USA, 2017. ISBN: Soft Cover, ISBN: 978-1-62894-270-5. This book discusses operational mechanism of NDS, and its approach to law enforcement and intelligence analysis.
10-Securing the Insecure States: Britain and the European Union Confronting the Prospect of Radicalization, Nuclear, Biological and Chemical Terrorism. Musa Khan Jalalzai, Algora Publishing, New York, 2017, ISBN-10: 1628943009, ISBN-13: 978-1628943009
11-The Crisis of Britain''s Surveillance State: Security, Law Enforcement, and the Intelligence War in Cyberspace. Musa Khan Jalalzai, Algora Publisher, Riverside, New York, USA, July 10, 2014. ISBN: 978-1-62894-078-7. This is a critical analysis of the British surveillance state and challenges of state institutions.
12-Afghan National Army: The CIA-Proxy Militias, Fatemyoun Division, Taliban and the Islamic State of Khorasan. By: Musa Khan Jalalzai. Vij Books India Pvt Ltd New Delhi. ISBN: 978-93-89620-03-0 (Hardback) ISBN: 978-93-89620-05-4 (e-book). This book highlights the US and NATO’s self-styled war on terrorism in Afghanistan that has entered a critical juncture. Afghans have become sick and tired with this brutal war that has been taking lives of their dearest and nearest ones in different acts of violence since 2001.
13-Massive Plunder of Afghanistan’s Mineral Wealth: The US and NATO burglars, Taliban, Islamic State of Khorasan, and jihadist groups of Central Asia, Go-Fifty-Fifty. By: Musa Khan Jalalzai. Vij Books India Pvt Ltd, New Delhi. ISBN: 978-93-89620-03-0. This book discusses Human rights abuses by the US army, CIA, NATO’s intelligence agencies, the ISIS, Taliban, and their looting of mineral wealth of Afghanistan, to finance their war. The US-backed Afghan militias committed murder, rape, torture and extortion. They are operating outside the Afghan government chain of command-have been used by international forces, and the US Special Operations Forces (SOF) and the CIA since 2001. This book also highlights the United States secret militias, Afghan army and the NATO forces support to the ISIS to expand its geographical matrix to the borders of Central Asia and China.
14-Afghanistan Tottering on the Brink: Private Mercenaries, ISIS, Taliban, and the New Cold War, 2017, Musa Khan Jalalzai, LAP Lambert Academic Publishing. 2017. ISBN: 10: 6202054271, ISBN-13: 978-6202054270.
15-Afghanistan: Sly Peace in a Failed State, Musa Khan Jalalzai. Vij Publishing. New Delhi India, July 2019. ISBN: 9789388161886. The ISIS created deep problems in finding a solution to the peace problem in Afghanistan. This book is a collection of various articles written by eminent researchers on the aspects of finding a solution to the peace prospects in troubled Afghanistan.
16-The Islamic State of Khorasan: The Prospect of Nuclear Jihad against Central Asia and Russia. Musa Khan Jalalzai. Vij Publishing. New Delhi India, September 2019. ISBN: 9788194261858. Daesh is worse than the Taliban, which is now trying to bring a new ideology as Daesh-ism which is anti-Islam. This book brings out the alarming situation of the presence of Daesh in Pakistan and its expanding activities. It serves the international community as a reminder of the role they need to play in crushing this monster.
17-Whose Army? Afghanistan’s Future and the Blueprint for Civil War. Musa Khan Jalalzai, Algora Publishing, Riverside, New York, USA, February 24, 2014. ISBN: 978-1-62894-054
18-Policing in Multicultural Britain: Racism, Colours, Riots and Cyber Terrorism. Musa Khan Jalalzai, Afghan Security Studies Ltd, January 12, 2012
19-The Changing Fight of Indian Intelligence Agencies: Reforms, Failures, Bureaucratic and Political Stakeholders, Musa Khan Jalalzai, Research Institute for European and American Intelligence Studies. 2019
20-The Foreign Policy of Pakistan: Afghanistan, Kashmir and Internal Security Threats, Musa Khan Jalalzai, Dua Publications, Lahore Pakistan, 2003, ASIN: B018HWHW7G. Package Dimensions: 22.4 x 14.2 x 3 cm
26-The Crisis of State and Security in Pakistan, Musa Khan Jalalzai, Dua Publications, Lahore Pakistan, 2003, ASIN: B018HWHW7G. Package Dimensions: 22.4 x 14.2 x 3 cm
27-Taliban and the Post Taliban Afghanistan, Musa Khan Jalalzai, 2003, Sang-e-Meel Publication, Pakistan, ISBN-10: 9699526122, ISBN-13: 978-9699526121
28-Failed States: The Need for a Realistic Transition in Afghanistan, Musa Khan Jalalzai. Vij Publishing. New Delhi India, January 2020. ISBN-10: 978-93-89620-19-1.978-93-89620-21-4. The Afghan state was collapsed in 1990s. In 2000s, international community established an artificial state to further their political and economic agenda. Pakistani state has also failed due to a massive corruption, violence, terrorism, sectarianism, and its deteriorating relationship with neighbouring states.
29-Jihad in Central Asia: Foreign Fighters, the Islamic State of Khorasan, the Challenges and Uyghur Islamic Front in China. Musa Khan Jalalzai. Vij Publishing. New Delhi India, March, 2020
My Research Papers and Articles:
1-Intelligence is in the Head not in the Age: Tight corner of intelligence sharing within the European Union. Musa Khan Jalalzai, Research Institute for European and American Studies (www.rieas.gr), February 2019
2-Intelligence without Ambition: National Directorate of Intelligence (NDS) of Afghanistan. Musa Khan Jalalzai, Research Institute for European and American Studies (www.rieas.gr), 24 February 2019
3-The Changing Fight of Indian Intelligence: Failure, Bureaucratic and Political Stakeholders. Musa Khan Jalalzai, Institute for European and American Studies, Greece. (www.rieas.gr), 17 February 2019
4-Dynamics of Britain’s National Security Challenges: Research Institute for European and American Studies (www.rieas.gr), Publication date: 23 June 2019
6-The Crisis of State and Security in Britain, Musa Khan Jalalzai, 16 April 2016, Daily Times
7-UK and the Field of Security, Musa Khan Jalalzai, Daily Times, 06 February 2017
8- Fixing the EU Intelligence Crisis, Musa Khan Jalalzai, the Nation, August 30, 2016
9-Law Enforcement Mechanism in Britain: Weak policing and intelligence infrastructure, and the prospect of terrorism. Musa Khan Jalalzai, January, 23, 2017, Daily Times, Pakistan
10-Surveillance and Security Crisis in Europe, Musa Khan Jalalzai, January, 9, 2017, Daily Times, Pakistan
11-The Intelligence War in Britain. Musa Khan Jalalzai, December, 26, 2016, Daily Times, Pakistan
12-The Snoopers’ Charter Surveillance law in Britain: Controversies and resentment. Musa Khan Jalalzai, November 28 2016, Daily Times, Pakistan
13-Afghan Intelligence: Foreign Influence and the Allegations of Torture, Musa Khan Jalalzai, April 19, 2019. Daily Times, Pakistan
14-Dynamics of Britain’s National Security Threats: Law Enforcement, Intelligence Infrastructure, Policing, Political, and Private Stakeholders. Global Security Review, USA
15-Britain’s Changing Security Perceptions: The country’s National Security Challenges are Amplifying by the day. Musa Khan Jalalzai, Global Security Review-15 August 2019
16-The prospect of nuclear jihad in South Asia, Musa Khan Jalalzai, Daily Times, 02 October 2013
17-Nuclear Arms Race in South Asia, Musa Khan Jalalzai, Daily Times, July 6, 2015
18-Biological and Nuclear War in South Asia, Musa Khan Jalalzai, Daily Times, June 8, 2015
19-Chemical and Biological Jihad, Musa Khan Jalalzai, Daily Times, 01 June, 2015
20-British Jihadists, Privacy and Mass Surveillance, Musa Khan Jalalzai, Paper, I read in Seminar, 10 November 2014.
21-The ISIS Threat of Biological Terrorism, Musa Khan Jalalzai, Daily Outlook Afghanistan, 29, December, 2014
22-Pakistan: Fixing the Intelligence Machine, Musa Khan Jalalzai, Paper published by the D Times Management in 2014
23-Pakistan: Reorganization of Intelligence Infrastructure, Musa Khan Jalalzai, Paper, 2014
24-Afghanistan: Professionalization of Intelligence Cooperation, Musa Khan Jalalzai, Daily Times, 18 March, 2014
25-The Shifting Dynamics of Britain’s National Security Threats, Musa Khan Jalalzai, Global Security Review, Critical analysis of defense issues, security threats, and foreign policy. Jul 13, 2019.
26-Changing Security Perceptions in Britain, Musa Khan Jalalzai, Journal of European and American Intelligence Studies, Research Institute of European and American Studies, Greece 2019,
27-Metropolitan Police, Racism, Security Sector Reform and the Fight against Radicalization, Drug Trafficking and Terrorism, Musa Khan Jalalzai, Journal of Intelligence Studies, 2018
28-The EU intelligence, Political and Bureaucratic Stakeholders, Private Intelligence Agencies, Hierarchies and Surveillance Crisis, Musa Khan Jalalzai, Journal of European and American Intelligence Studies, 2019
39-Pakistan: Democratic Forces and the Deep State. Musa Khan Jalalzai, Faultlines: the k.p.s. gill journal of conflict & resolution Edited by Ajai Sahni. The Institute for Conflict Management, New Delhi October 2019. Volume-25, ISBN: 978-93-87809-91-8. FAULTLINES focuses on various sources and aspects of existing and emerging conflict in the Indian subcontinent. Terrorism and low-intensity wars, communal, caste and other sectarian strife, political violence, organised crime, policing, the criminal justice system and human rights constitute the central focus of the Journal.
PhD and Master Thesis
I- completed the Master Thesis of an Afghan Student for the University of Westminster London in 2009
2-I completed Master Thesis of an Afghan Student for the University of Northumberland. UK in 2016
3-I completed a Graduate thesis of a Pakistani student for the London Metropolitan University London in 2015
4-I helped the PhD thesis of a Pakistani Professor in Mansehra University Pakistan in 2019
5-I helped Thesis of an Afghan student for the University of Zwanghou, China in 2019
6-I completed a PhD Thesis of a Pakistani student for the University of Peshawar in 2004
7-I completed Thesis of one of Pakistani Intelligence Chief for the National Defense University in 2003, titled: (Dynamics of Pakistan’s internal security threats).
8-I completed the PhD thesis of a Student for the University of Buckinghamshire, UK in 2017
9-I completed Thesis of one of Punjab’s former Inspector General in 2002
10- I completed master thesis of an Afghan student for the University of Westminster, London
11-Contributed in the Cyber Security Project of Swansea University, UK, in 2014. The project is available at Google easily.
12-I have been helping the UK Lover Courts and High Courts in case of expert opinion report for asylum seekers of India, Afghanistan, and Pakistan since 2009.
All of my 90 published books, 3,000 articles published in different newspapers, and research papers are available at: amazon.co.uk (100-Books), at. amazon.com (40-Books), amazon.fr, amazon.de, amazon.ca, amazon.ru, amazon.au, and Google.com
Musa Khan Jalalzai London, UK
322-Wellington Road South, Hounslow
TW4 5JU
Journalistn4@gmail.com
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7/1/2019 12:00:00 AM | Contact Name : | LEONARD EBOSI |
Email ID : | brokerscomunity@gmail.com | Subject : | YOU CAN AVOID THE DISASTER |
Message : | There will be no peace in the world and calamities will be rampant as
hard times will hit people all over the world.
America Center for Disease Control with World Heath Organization has
warned that some incurable epidemics/ diseases are coming very soon.
We all know from experience that life brings its share of troubles.
Sometimes those troubles come from external sources and at other times
from within us. As conditions continue to break down in the world,
more people are facing increased difficulties such as anxiety, fear,
weariness, stress, financial strain, strong temptations, problems with
sin, loneliness, insecurity and broken relationships . Perhaps you are
even now in the midst of some great burden.
How many of us today are facing pitch dark chi lemmas in our marriage,
among our children, or in our finances? Possibly a spiritual problem
is nagging at us, with seemingly no solution. We have reasoned and
counseled and strategics, all to no avail. What we need is a bust of
God’s light.3
As difficult as our troubles can be, the good news is that “God is our
refuge and strength, a very present help in trouble. Therefore we will
not fear, though the earth should change, and though the mountains
ship into the heart of the sea: though its waters roar and foam,
though the mountains quake at its swelling pride. (Psalm 46:1-3).
How reassuring it is to know that God is able and faithful to help
everyone who looks to Him, no matter how great the trouble.
So no matter what the situation, God is able to deal with. In Him is always hope
When we trust in Him to help us, we will not be disappointed. When we
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will make us fall. (Psalm 119 :165).
By His very nature, God is a God who helps. He helps those who are
under evil attack (Matt. 15 : 25; Mark 9:22 ; 2 Tim. 4:17 – 18).
He helps those who are crushed under the weight of their own sin
(Psalm 38 : 1-22, Psalm 51 : 1-17). He helps those who are under
temptation (Hebrew 2:14-18; 1 Corinth . 10:13) He helps those plagued
with anxious thoughts (Psalm 94 : 17 – 19; Phil. 4:6-7) He helps those
who are older and whose strength fails (Psalm 71:9, 12, 20). He helps
those who have lost their former glory and joy (psalm 42:3-5) He helps
the poor and needy (Psalm 40:17).
Perhaps your troubles are wearing you down and you wonder if the Lord
even cares about you and your circumstances have put you in distress,
call out to Him (2 Sam. 22 :7; 2 Chr. 14:11) and cry out to Him (2
Chr. 20:9; Psalm 18:6) seek Him and His help (2 Chr. 20 :4 ; Psalm 27
:7 – 9).
God still breaks chains today no matter how strong the shackles of
lust, drugs, alcohol, or deceit. The spirits power can break these
fetters and set men and women, boys and girls. When God moves into our
lives, no obstacle can stop Him. Expect everything from Him. He can
amplify our resources and multiply out capabilities more than we
dream. Anxious thoughts can multiply within us. (Psalm 94:19), shift
our focus off a trust in God, and rob us of the fullness of joy and
life that He intends for us.
Satan came to steal, kill and destroy, Jesus came that we might have
life in abundance. (John 10:10). You and I can never meet the enemy
ourselves. He would have the best of the arguments every time, but if
we are able to present him with Christ, what can he do?
Accept Jesus Christ as your personal Lord and Savior so that you will
have peace that pass all understanding. Don’t die in silent!.
Jesus way leads us to the path of real life, the approach to God. The
teachings of Jesus are the very words of life. The death of Jesus is
the price for our sins and actually became the death of death for us.
The resurrection of Jesus is the triumph of life, the very power of
God to make every thing new.
It is contact with Jesus that makes our own lives real. Jesus is the
solace, the supply, the satisfaction for every human heart oppressed
by sin and sorrow, suffering and care. In Jesus all the problems of
earth’s suffering are solved, its sorrows soothed. In Jesus is the
stimulus, the strength, the stay for every present need. In Jesus is
the only hope there is for future bliss (1 Tim. 1:1)
Jesus have done every thing to save you from sin and Satan and to make
you not to enter hell fire. You are seriously warned to avoid sin,
make peace with God. For Jesus is coming very very soon. Heaven is
real, hell fire is real. There is no repentance in hell fire. Amend
your way now before it is too late.
Just as" God waited patiently in the days of Noah while the ark was
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opportunity for us to prepare for what is to come.
God have handed you a new year and a new opportunity to start over
again. How wonderful - a chance to do it right this time If there are
any barriers between you and Him, remove them. Take them to the cross
and start this year with a clean slate. Don’t drag the past into the
future. Life is too short If you only knew how brief your life is and
how quickly it will be over, you’d make a quality decision not to
waste one more day.
You see, for every possible predicament you face, God has sufficient
grace to carry through. For every need you have, He has a supernatural
supply. For every problem, He''s got a definite answer; for every hut,
He has a cure. Jeremiah said, “There is nothing too hard for
Thee,"(Jer. 32:17) God is just waiting for an opportunity to show you
what He can do.
Can you open up by accepting Jesus as your Lord and Savior? Call on Him now.
JESUS CHRIST IS COMING SOON,RAPTURE CAN BE ANY MOMENT
FORWARD TO YOUR LOVED ONES.
For Prayer and Counseling Contact :-
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Ebosi
+2348068885394, +2348068812264
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3/4/2019 12:00:00 AM | Contact Name : | Mustafa Akpinar |
Email ID : | saucis@sakarya.edu.tr | Subject : | Publisher Login Page |
Message : | Dear manager,
We want to add our volume and issues to your index. We get the certificate of acceptance but we can not login DRJI (Directory of Research Journal Indexing) Publisher Control Login Panel.
Could you assign a login password to us?
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Email ID : | dr.amarjeetsingh@hotmail.com | Subject : | Regarding include articles |
Message : | Dear Team,
One of our journal "International Journal of Engineering and Management Research" is indexed with DRJI. Now, we want to add our journal papers in it. Kindly suggest us, how can we do it?
Below are the details of our journal:
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