儿童白塞综合征45例临床特征分析

Clinical characteristics of Behçet syndrome in 45 children

摘要:

目的 探讨儿童白塞综合征（Beh?et syndrome, BS）的临床特征，为儿童BS的诊治提供参考。方法 回顾性分析2014年12月—2024年12月于河北医科大学第二医院儿科住院且符合BS诊断标准患儿的临床资料。结果 45例BS患儿中，男性26例（58%），临床表现以口腔溃疡最常见（43例，96%），其余包括生殖器溃疡23例（51%），消化系统受累18例（40%）等。生殖器溃疡女性多见，眼部病变男性多见（P<0.05）。针刺试验阳性10例（22%），人类白细胞抗原-B51阳性13例（29%）。粪便钙卫蛋白（fecal calprotectin, FC）升高16例（36%），FC升高患儿消化系统受累比例高于FC正常患儿（P<0.05）。符合1990年白塞病国际研究组诊断标准17例（38%），2014年白塞病国际评分标准33例（73%），2015年儿童白塞病诊断标准13例（29%）。结论 儿童BS临床表现异质性强，人类白细胞抗原-B51与其发病相关。

Abstract:

Objective To study the clinical characteristics of pediatric Beh?et syndrome (BS).Methods A retrospective review was conducted on the medical records of children hospitalized in the Department of Pediatrics at the Second Hospital of Hebei Medical University between December 2014 and December 2024 who met diagnostic criteria for BS.Results Among 45 children with BS, 26 (58%) were male. Oral aphthous ulcers were the most common manifestation (43/45, 96%), followed by genital ulcers (23/45, 51%) and gastrointestinal involvement (18/45, 40%). Genital ulcers were more frequent in girls, whereas ocular involvement was more common in boys (P<0.05). The pathergy test was positive in 10 (22%), and HLA-B51 was positive in 13 (29%). Fecal calprotectin (FC) was elevated in 16 (36%); gastrointestinal involvement was more frequent in children with elevated FC than in those with normal FC (P<0.05). According to the respective criteria, 17 (38%) patients met the International Study Group criteria (1990), 33 (73%) met the International Criteria for Beh?et Disease (2014), and 13 (29%) met the Pediatric Beh?et Disease criteria (2015).Conclusions Pediatric BS shows marked clinical heterogeneity. HLA-B51 is associated with disease susceptibility.