先天性肝纤维化2例及鉴别诊断

肝脏 ›› 2017, Vol. 22 ›› Issue (8): 709-712.

先天性肝纤维化2例及鉴别诊断

沈小静,纪元

200032 上海复旦大学附属中山医院病理科

收稿日期:2016-10-10 出版日期:2017-08-15 发布日期:2020-06-16

Received:2016-10-10 Online:2017-08-15 Published:2020-06-16

摘要/Abstract

摘要： 目的通过分析先天性肝纤维化的临床病理学特征,进一步探究纤维多囊性疾病的临床病理特点,协助临床早期诊断及精准治疗。方法收集2例先天性肝纤维化病例的临床病理资料。结果其中1例行同种异体原位肝移植术,光镜下表现为先天性肝纤维化,临床上伴发肝肾囊肿,故诊断为Caroli′s综合征;另1例行肝穿刺明确诊断为先天性肝纤维化。两例患者均为年轻人,伴发门静脉高压及脾脏肿大,光镜下病理形态相似。2例病例均属于纤维多囊性疾病。结论先天性肝纤维化临床表现缺乏特异性,其隐匿性及复杂性极易导致误诊及漏诊。因此,提高对其临床及病理学特点的认识对于正确诊断及治疗具有重要意义。

关键词: 先天性肝纤维化, Caroli′s病, Caroli′s综合征, 鉴别诊断

沈小静,纪元. 先天性肝纤维化2例及鉴别诊断[J]. 肝脏, 2017, 22(8): 709-712.

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