Registries in rheumatological and musculoskeletal conditions. Paediatric Behçet's disease: an international cohort study of 110 patients. One-year follow-up data.

Détails

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Version: Final published version
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ID Serval
serval:BIB_83BD206A3B29
Type
Article: article d'un périodique ou d'un magazine.
Collection
Publications
Institution
Titre
Registries in rheumatological and musculoskeletal conditions. Paediatric Behçet's disease: an international cohort study of 110 patients. One-year follow-up data.
Périodique
Rheumatology
Auteur⸱e⸱s
Koné-Paut I., Darce-Bello M., Shahram F., Gattorno M., Cimaz R., Ozen S., Cantarini L., Tugal-Tutktun I., Assaad-Khalil S., Hofer M., Kuemmerle-Deschner J., Benamour S., Al Mayouf S., Pajot C., Anton J., Faye A., Bono W., Nielsen S., Letierce A., Tran T.A.
Collaborateur⸱rice⸱s
PED-BD International Expert Committee
Contributeur⸱rice⸱s
Ozen S., Ozdogan H., Gul A., Shahram F., Hofer M., Gattorno M., Koné-Paut I.
ISSN
1462-0332 (Electronic)
ISSN-L
1462-0324
Statut éditorial
Publié
Date de publication
2011
Volume
50
Numéro
1
Pages
184-188
Langue
anglais
Notes
Publication types: Journal Article ; Research Support, Non-U.S. Gov'tPublication Status: ppublish
Résumé
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.
Mots-clé
Adolescent, Age of Onset, Algorithms, Behcet Syndrome/genetics, Behcet Syndrome/physiopathology, Child, Child, Preschool, Cohort Studies, Databases, Factual, Diagnosis, Differential, Disease Progression, Female, Follow-Up Studies, Genetic Predisposition to Disease, Humans, International Cooperation, Male, Musculoskeletal Diseases/genetics, Musculoskeletal Diseases/physiopathology, Pedigree, Registries, Severity of Illness Index, Sex Factors, Statistics as Topic, Young Adult
Pubmed
Web of science
Open Access
Oui
Création de la notice
04/01/2013 11:22
Dernière modification de la notice
14/02/2022 7:55
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