貝歇氏症在兒童及青少年的臨床表現：8個病例報告

Clinical features of Behcet's disease in children and adolescents: Report of eight cases

目的：本研究的目的是分析兒童和青少年貝歇氏症患者的臨床症狀，眼睛症狀及HLA型別的變化。方法：統計從2006年到2015年，合計8位（7女1男）貝歇氏症患者的病歷紀錄。結果：統計病人的臨床症狀和HLA型別結果如下：口腔潰瘍有8人，生殖器潰瘍有7人，眼睛症狀有5人，皮膚症狀有1人，神經症狀有3人，關節疼痛有3人，針刺試驗（Pathergy test）陽性有5人，HLA B5型別有4人。結論：貝歇氏症會造成多器官症狀的疾病，眼部的葡萄膜炎在年輕病人族群常是主要的併發症。而在嚴重的葡萄膜炎年輕患者，類固醇脈衝療法可以有效減緩葡萄膜炎惡化。

Background: The aim of this study was to analyze the clinical features, visual results and HLA allelic type in young patients with Behcet’s disease. Patients and methods: Eight young patients (Seven males, one female) with Behcet’s disease were followed from 2006 to 2015. Results: Oral aphthosis was present in all patients (100%), with genital aphthosis present in seven (87.5%), ocular lesions in five (62.5%), skin lesions in one (12.5%), neurological manifestations in three (37.5%), arthralgia in three (37.5%), positive pathergy test in five (62.5%), and HLA B5 allele in four (50%). Conclusion: Behcet’s disease affects multiple-organs. Ocular involvement such as panuveitis is a major complication in children with Behcet’s disease. Steroid pulse therapy is useful to stop the progression in young patients with severe Behcet’s uveitis.