【摘要】 介绍临床以肝脾肿大、门脉高压、上消道出血为主要症状的一种少见的遗传病一先天性肝纤维化的概念、诊断与治疗。１９９３年以来收治先天性肝纤维化３例，均经临床及病理证实，行脾切除、贲门胃底断流术。术后随访１～３年，患儿发育、营养佳，无再出血，肝功能正常，近期疗效满意。复习文献结合本组病例诊治认为，临床发现肝脾肿大、门脉高压、肝内胆管多发性扩张、肝功能基本正常者应想到该病。肝脏组织学特点为汇管区纤维增生明显，肝小叶结构正常。为预防术后并发症应早期诊断，及时治疗。更多还原

【Abstract】 Three cases of congenital hepatic fibrosis underwent. splenectomy and cardiofundo- transectomy are reported. The clinical manifestations intcluded hepatosplenomegaly , portal hypertension and upper gastrointestinal hemorrhage. The diagnosis was confirmed by pathologic examination. They were followed-up for 1 to 3 years with normal physical development , hepatic functions , and without GI bleeding. The authors state that patients with hepatosplenomegaly , portal hypertension , multiple dilation of intrahepatic bile ducts but without abnormal liver functions , congenital hepatic fibrosis should be considered. The histologic characteristics include fibrous proliferation of interlobular portal areas , and normal structure of hepatic lobules. Early diagnosis and treatment should be stressed to prevent postoperative complications.更多还原