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Pediatric Behcet's Disease and Thromboses.
MedLine Citation:
PMID:  21078724     Owner:  NLM     Status:  In-Data-Review    
Abstract/OtherAbstract:
OBJECTIVE: To describe the characteristics of a group of pediatric patients with Behçet's disease (BD) who presented at least 1 episode of thrombosis during their disease course.
METHODS: We made a retrospective chart review of the clinical, biological, and radiological data of children with BD who presented at least 1 episode of either arterial or venous thrombosis. Data were extracted from both an international pediatric Behçet cohort and files referred from 7 French centers.
RESULTS: Twenty-one patients were included. Diagnosis of BD was based on the criteria of the International Study Group for BD. Main locations for thrombosis were the cerebral sinuses, in 11 patients (52.4%); and lower limbs, in 9 patients (40.9%). Recurrent episodes were observed in 4 patients (21%). Thrombophilia measurements were normal in 14 patients out of 21, while anticardiolipin antibodies were positive in 4 patients, and 2 out of 21 had protein C deficiency. One patient had lupus anticoagulant. All patients were treated with colchicine. Corticosteroids were also added for variable periods in 13 patients. Five patients out of 21 were treated with anticoagulants (heparin, then anti-vitamin K) and 3 with antiplatelets (acetylsalicylic acid).
CONCLUSION: Thromboses are a serious complication of BD and may occur early in the disease course. The presence of thrombophilic markers could increase the risk of thrombosis in BD, but the size of our population does not allow any conclusion. An international cohort (PED-BD) is currently in place and will allow study of such cases longitudinally, as well as assessment of the elements that correlate with an increased risk of thrombosis in children with BD.
Authors:
Brice Krupa; Rolando Cimaz; Seza Ozen; Michel Fischbach; Pierre Cochat; Isabelle Koné-Paut
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Publication Detail:
Type:  Journal Article     Date:  2010-11-15
Journal Detail:
Title:  The Journal of rheumatology     Volume:  38     ISSN:  0315-162X     ISO Abbreviation:  J. Rheumatol.     Publication Date:  2011 Feb 
Date Detail:
Created Date:  2011-02-02     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  7501984     Medline TA:  J Rheumatol     Country:  Canada    
Other Details:
Languages:  eng     Pagination:  387-90     Citation Subset:  IM    
Affiliation:
Service de Pédiatrie Générale, Hématologie et Rhumatologie Pédiatrique, Hôpital Bicêtre, 78 rue Général Leclerc, 94270 Le Kremlin Bicêtre, France. isabelle.kone-paut@bct.ap-hop-paris.fr.
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