婴儿胆汁淤积症145例临床分析

Clinical analysis of 145 cases with infantile cholestasis

目的对婴儿胆汁淤积症各个病因临床表现、辅助检查及预后进行统计学分析,归纳婴儿胆汁淤积症诊断及鉴别诊断方法。方法收集2007年12月～2010年2月本院住院的145例婴儿胆汁淤积症患儿的临床资料,对比胆道闭锁组与肝内胆汁淤积症组临床特点;分析巨细胞病毒(CMV)与胆汁淤积的关系;统计及评价胆道闭锁目前的临床诊断试验。结果胆道闭锁患儿白便及肝大发生率、GGT值及腹部彩超异常方面与婴儿肝内胆汁淤积症比较差异具有统计学意义。CMV感染与婴儿胆汁淤积相关性分析中,CMV阳性与阴性临床特征比较差异无统计学意义;胆道闭锁常用诊断项目中,白便的敏感度较高(88%),GGT>300 U/L、腹部彩超见三角形纤维包块特异度相对偏高。结论婴儿胆汁淤积症患儿有胆汁淤积症表现为生长发育正常,临床发现白便,腹部彩超提示汇管区三角形纤维包块,GGT>300 U/L时高度提示胆道闭锁;CMV感染在胆汁淤积症的发病中只是致病原因之一或者夹杂感染因素。

Objective This study was aimed at analyzing the clinical etiology, laboratory examination of infantile cholestasis, evaluating parameters in the differential diagnosis of intrahepatic cholestasis and biliary atresia（BA）. Methods The clinical characteristics, laboratory examinations and prognosis of the 145 hospitalised children in pediatric GI ward of Shengjing hospital from December 2007 to Febraury 2010 were analyzed. The data of BA and IHC groups were compared （145 cases diagnosed with intrahepatic cholestasis were collected and the clinical information was analyzed）. Results Compared with intrahepatic cholestasis cases, the incidences of hepatomegaly, acholic stools, increased GGT level, and the abnormal ultrasonography are significantly higher in BA. By analyzing the correlation between the CMV infection and infantile cholestasis, the clinical characteristics between positive and negative serology for CMV groups had no significant differences. Acholic stools has a higher sensitivity （88%） for diagnosis of BA. The high GGT （〉300 U/L）, TC sign in ultrasonography had significant diagnostic value with higher specificity. Conclusion In the infantile cholestasis cases who presented acholic stools, hepatomegaly, a marked elevation in serum GGT （〉300 U/L）, and positive TC sign combined with either abnormal size or abnormal contractility of the gallbladder, the most likely diagnosis was biliary atresia. Cytomegalovirus infection is one of the common reasons or just an ＂innocent by-stander＂.