儿童药物性胆管消失综合征3例报告并文献复习

目的分析儿童药物性胆管消失综合征(VBDS)的临床特点及病理。方法收集2019年1月至2019年12月收治的3例诊断为药物性VBDS患儿的临床资料,并结合文献进行分析。结果3例患儿,女1例、男2例,年龄9月龄~8岁,临床均以黄疸型胆汁淤积伴瘙痒为主要表现;予护肝、退黄,以及免疫抑制剂等治疗,其中2例经治疗3个月后临床症状及生化指标基本恢复;1例治疗1年余仍有轻度的黄疸型胆汁淤积表现,仍在随诊中。结论早期识别药物相关性VBDS,停用相关药物并予积极治疗能改善预后。

Drug-induced biliary disappearance syndrome: a report of 3 cases and literature review

Objective To explore the clinical features and pathology of drug-induced vanishing bile duct syndrome(VBDS)in children.Method The clinical data of drug-induced VBDS in 3 children from January 2019 to December 2019 were collected and analyzed in combination with the literature.Results Three children(1 girl and 2 boys)aged from 9 months to 8 years had jaundice cholestasis with pruritus as main clinical manifestations.All the patients were treated with liver protection,anti-jaundice and immunosuppression.After 3 months of treatment,the clinical symptoms and biochemical indexes of 2 patients were basically recovered.One patient still presented mild jaundice cholestasis after 1 year of treatment and was still in follow-up.Conclusion Early identification of drug-related VBDS,discontinuation of related drugs and active treatment can improve the prognosis.