Document Detail


Registries in rheumatological and musculoskeletal conditions. Paediatric Behçet's disease: an international cohort study of 110 patients. One-year follow-up data.
MedLine Citation:
PMID:  21036877     Owner:  NLM     Status:  In-Process    
Abstract/OtherAbstract:
OBJECTIVE: To set-up an international cohort of patients suspected with Behçet's disease (BD). The cohort is aimed at defining an algorithm for definition of the disease in children.
METHODS: International experts have defined the inclusion criteria as follows: recurrent oral aphthosis (ROA) plus one of following-genital ulceration, erythema nodosum, folliculitis, pustulous/acneiform lesions, positive pathergy test, uveitis, venous/arterial thrombosis and family history of BD. Onset of disease is <16 years, disease duration is ≤3 years, future follow-up duration is ≥4 years and informed consent is obtained. The expert committee has classified the included patients into: definite paediatric BD (PED-BD), probable PED-BD and no PED-BD. Statistical analysis is performed to compare the three groups of patients. Centres document their patients into a single database.
RESULTS: At January 2010, 110 patients (56 males/54 females) have been included. Mean age at first symptom: 8.1 years (median 8.2 years). At inclusion, 38% had only one symptom associated with ROA, 31% had two and 31% had three or more symptoms. A total of 106 first evaluations have been done. Seventeen patients underwent the first-year evaluation, and 36 had no new symptoms, 12 had one and 9 had two. Experts have examined 48 files and classified 30 as definite and 18 as probable. Twenty-six patients classified as definite fulfilled the International Study Group criteria. Seventeen patients classified as probable did not meet the international criteria.
CONCLUSION: The expert committee has classified the majority of patients in the BD group although they presented with few symptoms independently of BD classification criteria.
Authors:
Isabelle Koné-Paut; Martha Darce-Bello; Farahd Shahram; Marco Gattorno; Rolando Cimaz; Seza Ozen; Luca Cantarini; Ilknur Tugal-Tutktun; Samir Assaad-Khalil; Michael Hofer; Jasmin Kuemmerle-Deschner; Saida Benamour; Souleymane Al Mayouf; Christine Pajot; Jordi Anton; Albert Faye; Wafa Bono; Susan Nielsen; Alexia Letierce; Tu-Anh Tran;
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't     Date:  2010-10-29
Journal Detail:
Title:  Rheumatology (Oxford, England)     Volume:  50     ISSN:  1462-0332     ISO Abbreviation:  Rheumatology (Oxford)     Publication Date:  2011 Jan 
Date Detail:
Created Date:  2010-12-14     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  100883501     Medline TA:  Rheumatology (Oxford)     Country:  England    
Other Details:
Languages:  eng     Pagination:  184-8     Citation Subset:  AIM; IM    
Affiliation:
Department of Paediatric Rheumatology, CEREMAI, Bicêtre University Hospital, Paris SUD University of Medicine, Le Kremlin Bicêtre, France. isabelle.kone-paut@bct.aphp.fr
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Investigator
Investigator/Affiliation:
S Ozen / ; H Ozdogan / ; A Gul / ; F Shahram / ; M Hofer / ; M Gattorno / ; I Koné-Paut /

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