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目的总结先天性肝纤维化(CHF)的临床表现及组织病理学特征,以提高临床诊治经验。方法回顾性分析2011年1月-2015年6月第四军医大学第一附属医院收治的经组织病理学明确诊断的CHF患者13例,统计患者年龄、临床表现、实验室指标和影像学表现等临床资料。结果 13例患者中,门静脉高压型8例、胆管炎型1例、混合型1例、隐匿型1例,影像学检查提示8例存在肝肾囊肿,4例合并门静脉海绵样变性。结论 CHF患者以门静脉高压和肝功能正常为突出表现,常伴发肝肾囊肿、Caroli病。对于不明原因肝硬化,特别是肝功能减退与门静脉高压不相符的患者,应行肝活组织检查协助诊断。
Objective To summarize the clinical manifestations and histopathological features of congenital liver fibrosis (CHF) so as to improve the clinical diagnosis and treatment experience. Methods Thirteen patients with CHF diagnosed by histopathology admitted to the First Affiliated Hospital of Fourth Military Medical University from January 2011 to June 2015 were retrospectively analyzed. The age, clinical manifestations, laboratory parameters and imaging findings of patients clinical information. Results Among the 13 patients, there were 8 cases of portal hypertension, 1 cholangitis, 1 mixed type and 1 occult type. Imaging examination revealed that there were 8 cases of hepatorenal cyst and 4 cases of spontaneous degeneration of portal vein. Conclusions CHF patients with portal hypertension and normal liver function as the outstanding performance, often associated with liver and kidney cysts, Caroli disease. For patients with unexplained liver cirrhosis, especially those with impaired liver function and portal hypertension, liver biopsy should assist in the diagnosis.