儿童胆管缺失综合征行肝移植术治疗

Liver transplantation in treatment of child patient with vanishing bile duct syndrome

目的探讨胆管缺失综合征的临床诊治策略。方法回顾性分析四川大学华西医院收治的1例术前疑似胆道闭锁而行肝移植术患者的临床资料和诊治经过。结果该例患儿因肝功能衰竭而行肝移植手术,术后病理结果提示为肝内胆管缺失综合征伴继发性(不全分割性)胆汁淤积性肝硬变形成,术后恢复良好。已随访至术后11个月,肝功能、凝血功能及移植肝血流未见明显异常,身高、体质量与同龄儿童相近。结论胆管缺失综合征病因众多,发病机制复杂,肝移植为最终有效的治疗方法。

Objective To discuss diagnosis and treatment strategy of vanishing bile duct syndrome. Method The clinical data of 1 child patient with suspected biliary atresia who received the liver transplantation in the West China Hospital of Sichuan University was retrospectively analyzed. Results The child patient underwent the liver transplantation due to the liver failure, and the postoperative pathological results indicated the vanishing bile duct syndrome accompanied by the formation of secondary （incomplete segmentation） cholestatic liver cirrhosis, the postoperative recovery was good. After 11 months of follow-up, the liver function, coagulation function, and blood flow of the transplanted liver were not obviously abnormal. The height and weight were suitable for the same age. Conclusion At present, there are many etiologies and complicated pathogenesis of vanishing bile duct syndrome, and liver transplantation is an ultimate effective treatment.