婴儿胆汁淤积性黄疸的病因、临床特点及转归分析

Etiology, clinical characteristics and prognosis of 175 infants with cholestatic jaundice

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.