Department of Pediatrics, Salmaniya Medical Complex, Kingdom of Bahrain.
Arch Iran Med. 2012 Aug;15(8):485-7.
This report studies the clinical features of Behçet disease (BD) in children and compares our results with other international studies.
We retrospectively reviewed patient data that included the clinical picture, HLA typing, and treatment in BD cases.
This study reviewed data from a total of nine children with BD. Median age at presentation was seven years, with a male to female ratio of 2:1. There was one patient who had Down's syndrome. Oral ulcers were present in all children, while genital ulcers were present in only 66% of cases. Skin manifestation was seen in 88% and uveitis in 55%. There was evidence of gastrointestinal (55%), neurological (55%), and musculoskeletal manifestations (77%). HLA B5 was positive in 66% of cases and 55% had positive family histories. Apart from gastrointestinal symptoms, our results were comparable with other studies.
Awareness of BD symptoms in the pediatric age group is crucial for early diagnosis and treatment. The coexistence of BD and Down's syndrome needs further genetic study, which may link these two major disorders.
本报告研究了儿童贝赫切特病（BD）的临床特征，并将我们的结果与其他国际研究进行了比较。
我们回顾性分析了包括 BD 患者临床表现、HLA 分型和治疗在内的患者数据。
本研究共回顾了 9 例 BD 患儿的数据。中位发病年龄为 7 岁，男女比例为 2:1。其中有 1 例患儿患有唐氏综合征。所有患儿均有口腔溃疡，而生殖器溃疡仅见于 66%的病例。88%的患儿有皮肤表现，55%的患儿有葡萄膜炎。55%的患儿有胃肠道受累，55%的患儿有神经系统受累，77%的患儿有肌肉骨骼系统受累。66%的患儿 HLA B5 阳性，55%的患儿有阳性家族史。除胃肠道症状外，我们的结果与其他研究相似。
儿童时期对 BD 症状的认识对于早期诊断和治疗至关重要。BD 和唐氏综合征的共存需要进一步的遗传学研究，这可能将这两种主要疾病联系起来。
