PW03-010 - MHC complexity in Behçet's disease

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PW03-010 - MHC complexity in Behçet's disease

Introduction Family studies support a genetic contribution to Behçet’s disease (BD), with a sibling recurrence-risk ratio of 1152. The class I MHC molecule, HLA-B*51 (B*51), is the strongest known genetic risk factor for BD, however the gene immediately centromeric to HLA-B, MICA, has also been implicated in BD. Because of strong linkage disequilibrium (LD) between HLA-B and MICA, their respect...

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Neuro-Behçets in a Child

We describe a case of neuro-Behçet disease diagnosed in a 12-year-old girl. This patient presented with recurrent oral ulcers, incontinence, spastic gait, blurry vision, and asymmetrical lower extremity hypertonia. Extensive testing revealed punctate lesions through the central nervous system, vitritis, papillitis, and uveitis. A thorough infectious and neoplastic workup was negative. She was t...

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PW03-013 - Behçet's disease: genotype-phenotype correlations

Introduction Although the therapy for Behçet’s disease (BD) has improved since infliximab was approved for refractory retinochoroiditis therapy in Japan, the exact pathogenesis of BD remains unclear. Our recent genome-wide association study has identified the IL10 and IL23RIL12RB2 loci as susceptibility genes for BD, in addition to HLA-A*26 and B*51. rs1495965 is located in the intergenic regio...

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PW03-022 – Neutrophilic skin disease and inflammation

Introduction Robert Sweet first described a syndrome with a painful, erythematous nodular plaques, neutrophilic dermal infiltrates, fevers and peripheral neutrophilia. This cluster of syndromes became known as Sweet’s syndrome. There have been many published cases in children of neutrophilic dermatoses and fever which are labeled as Sweet’s syndrome. Recently, however, neutrophilic dermatoses h...

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PW03-012 – Unmet need in Behçet's disease: remission is rare

Results A total of 1757 visits of 258 patients were overviewed. Mean visit number was 6,8± 2,7 (range:1-10) and mean follow-up duration was 45.8±36.5 months (2-165). One hundred twenty-five patients (48.4%) were of mucocutaneus type, whereas 133 patients (51.6%) had major organ involvement. When all visits combined, 19.843.9% of the patients were using immunosuppressives (IS), whereas 35.3-59.3...

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ژورنال

عنوان ژورنال: Pediatric Rheumatology

سال: 2013

ISSN: 1546-0096

DOI: 10.1186/1546-0096-11-s1-a236