小儿原发性硬化性胆管炎

Primary Sclerosing Cholangitis in Children

原发性硬化性胆管炎是一少见的肝内外胆管均可受到侵犯的慢性炎症，病理表现为进行性胆管系统纤维性变，导致胆管不同程度、不同部位的狭窄或闭锁。多数认为病因与：①常染色体隐性遗传；②环境污染、药物中毒；③细菌病毒感染；④自身免疫异常有关。诊断主要依据病史、肝功能检查、肝穿刺活检。以保守治疗为主，可能时行胆肠内引流或经内窥镜、ＰＴＣ放导管扩张，弥漫性胆管病变可行肝移植术。本病预后差，多死于肝硬化、感染及出血。

Three cases of primary sclerosing cholangitis (PSC )are reported. Their clinical manifestations were abdominal pain, fever and progressive jaundice. Some of them were complicated by ulcerative colitis. The diagnosis relied on: 1. the above mentioned clinical manifestations;2.the increasing of bilirubin, serum copper, ceruloplasmin,alkaline phosphatase and cholesterol; 3. the findings of extrahepatic biliary obstruction including dilatation or stenosis in cholangiography and abdomninal B-ultrasonography.The treatment of PSC included: 1. use of corticosteroid, anticarcinogen and antibiotics; 2. balloon dilatation and wire stent application of biliary tract; 3. Internal drainage, reconstruction of biliary tract or liver transplantation. PSC has a poor prognosis. Children with PSC usually die of portal hypertention or liver failure in 5 ～7 years.