目的：了解婴儿胆汁淤积性黄疸的病因、临床特点及转归情况。方法：回顾性分析175例胆汁淤积患儿的临床资料，电话随访转归情况。结果：175例胆汁淤积患儿病因分析示胆道闭锁为42例，死亡19例，肝移植恢复良好4例，肝硬化等待肝移植8例，葛西手术后恢复良好 5例，失访6例；胆管发育不良2例均恢复良好；先天性胆管扩张2例均恢复良好。CMV感染29例均恢复良好。遗传代谢性疾病16例，其中Citrin 蛋白缺陷病13例，恢复良好10例，失访2例，死亡1例；酪氨酸血症3例，恢复良好1例，失访 1例，肝硬化等待肝移植1例。TPN相关性胆汁淤积4例均恢复良好。病因未明80例，恢复良好79例，失访1例。临床特点提示胆汁淤积性黄疸患儿常伴有粪便颜色改变、肝脾肿大等，常合并肺炎、低蛋白血症、凝血功能障碍等。结论：胆汁淤积性黄疸患儿病因较多，早期诊治，预后更好。%Objective To learn about the etiology , clinical characteristics and prognosis of infants with cholestasis jaundice. Methods The clinical data of 175 cholestatic patients were retrospectively analyzed , then the prognosis was followed-up with telephone. Results After analyzing the etiology , we found that among 175 patients , there were 42 with biliary atresia , of which 19 infants died , 4 recovered well after liver transplanta-tion , 8 had liver cirrhosis waiting for transplantation , 5 recovered well after Kasai Portoenterostomy and 6 lost contact. There were 2 patients with Bile duct dysplasia and 2 with congenital cholangiectasis and they had posi-tive outcomes. And 29 patients with Cytomegalovirus infection also had positive outcome. There were 16 patients with Heredity metabolic diseases , among which 13 patients were with Citrin protein deficiency; 10 had positive outcomes; 2 lost contact and 1 died. There were 3 patients with tyrosinemia , of which one had positive outcome;one lost contact and another got liver cirohosis waiting for liver transplantation. Four patients with TPN-related cholestasis all had positive outcomes. There were still 80 cases with unkown etiology , but 79 had positive out-comes and 1 case lost. The clinical characteristics showed that the infants with cholestatic jaundice often accom-panied by stool color changed , liver and spleen enlargement and so on , and often complicated with pneumonia , hypoalbuminemia and coagulation dysfunction and so on. Conclusion There are many etiologies for infants with cholestatic jaundice. Early diagnosis and early treatment would benefit the prognosis.
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