13例先天性肝纤维化的临床表现与病理特征分析

武健; 尹芳; 夏琳; 罗贯虹; 李红; 朱绍华; 张静; 郑洋洋; 孙菡; 周新民

doi:10.3969/j.issn.1001-5256.2016.03.018

13例先天性肝纤维化的临床表现与病理特征分析

DOI: 10.3969/j.issn.1001-5256.2016.03.018

第四军医大学第一附属医院消化内科

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中图分类号: R575.2
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出版历程
- 收稿日期: 2015-08-31
- 出版日期: 2016-03-20

Clinical manifestations and pathological features of congenital hepatic fibrosis: an analysis of 13 patients

Department of Gastroenterology,Xijing Hospital,Fourth Military Medical University

摘要

摘要: 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。
- 肝硬化 /
- 高血压,门静脉 /
- 胆汁淤积 /
- 病理学,临床
Abstract: Objective To summarize the clinical manifestations and pathological features of congenital hepatic fibrosis( CHF),and to improve the experience in the diagnosis and treatment of this disease. Methods A total of 13 patients with a confirmed diagnosis of CHF based on histopathological examinations who were hospitalized and treated in Xijing Hospital,Fourth Military Medical University,from January2011 to June 2015 were analyzed retrospectively. The clinical data including age,clinical manifestations,laboratory markers,and imaging findings were summarized and analyzed. Results Of all the patients,there were 8 cases of portal hypertension type,1 case of cholangitis type,1 case of mixed type,and 1 case of latent type. The imaging findings suggested that 8 patients had liver and kidney cysts,and 4 patients had cavernous transformation of the portal vein. Conclusion CHF patients often have portal hypertension and normal liver function as prominent manifestations,with concurrent liver and kidney cysts and Caroli disease. Liver biopsy should be performed for unexplained liver cirrhosis,especially for patients with inconsistency between reduction in liver function and portal hypertension.
- liver cirrhosis /
- hypertension,portal /
- cholestasis /
- pathology,clinical

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参考文献(9)

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