Descos B, Bernard O, Brunelle F, Valayer J, Feldmann D, Hadchouel M, Alagille D
Hepatology. 1984 Jul-Aug;4(4):678-83. doi: 10.1002/hep.1840040419.
Ten infants of less than 6 months of age presented with cholestatic jaundice and gallstones. Jaundice occurred after a lag period, and sepsis was present in three children. Ultrasound examination showed dilatation of intrahepatic and extrahepatic bile ducts in eight patients and detected cholelithiasis in three. Percutaneous transhepatic cholangiography and/or surgery allowed separation of the patients into two groups: (i) six children with lithiasis in the distal common bile duct, and (ii) four children with lithiasis associated with bile duct perforation at the junction between the cystic and common bile ducts with gallstones probably secondary to bile stasis and infection. Surgical treatment was confined to removal of calculi and drainage in eight children; biliary reconstructive surgery was necessary in the other two who had serious biliary duct lesions. No recurrence was observed after 1 to 7 years. The pigmentary nature of cholelithiasis was established by stone morphology in all cases, and by bile and stone analysis in several cases.
10名6个月以下婴儿出现胆汁淤积性黄疸和胆结石。黄疸在一段滞后期后出现，3名儿童存在败血症。超声检查显示8例患者肝内和肝外胆管扩张，3例检测到胆结石。经皮经肝胆道造影和/或手术将患者分为两组：(i) 6名胆总管远端有结石的儿童，(ii) 4名结石与胆囊管和胆总管交界处胆管穿孔相关的儿童，胆结石可能继发于胆汁淤积和感染。8名儿童的手术治疗仅限于结石清除和引流；另外两名胆管病变严重的儿童需要进行胆道重建手术。1至7年后未观察到复发。所有病例均通过结石形态确定胆结石的色素性质，部分病例通过胆汁和结石分析确定。
