SCLEROSING CHOLANGITIS WITH NEONATAL ONSET
SCLEROSING CHOLANGITIS WITH NEONATAL ONSET
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DOI：
10.1016/s0022-3476(87)80072-0
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发表时间：
1987-08-01
影响因子：
5.1
通讯作者：
ALAGILLE, D
中科院分区：
文献类型：
--
作者：
AMEDEEMANESME, O;BERNARD, O;ALAGILLE, D
硬化性胆管炎的特征是肝外或肝内胆管不规则狭窄，成人主要由放射学表现来定义。我们描述了8名从出生第一周起就有胆汁淤积的儿童，随后是早期的肝硬变和门脉高压症。肝脏组织学检查显示，两名患者在胆汁淤积期早期没有小叶间胆管，所有患者后来都出现了胆汁性硬化。在8个月至9岁时，在超声引导下进行经皮胆囊造影放射学检查，发现肝内胆管异常，节段分支稀疏，狭窄，局灶性扩张。肝外胆管受累6例。这些患者中没有发现肠道疾病。
Sclerosing cholangitis is characterized by irregular narrowing of extrahepatic or intrahepatic bile ducts, and in adults is defined mainly by radiologic findings. We describe eight children with cholestasis from the first week of life followed by early cirrhosis and portal hypertension. Histologic examination of the liver showed absence of interlobular bile ducts in the early cholestatic phase in two patients and biliary cirrhosis later in all patients. Radiologic examination by percutaneous cholecystography under ultrasound guidance, carried out at age 8 months ot 9 years, disclosed abnormal intrahepatic bile ducts with rarefaction of segmental branches, stenosis, and focal dilation. The extrahepatic ducts were involved in six patients. No intestinal disease has been found in these patients.