5例Caroli病临床病理学特征分析

李莉; 姚晶晶; 杨江辉; 尹洪芳

doi:10.3969/j.issn.1001-5256.2019.11.026

5例Caroli病临床病理学特征分析

DOI: 10.3969/j.issn.1001-5256.2019.11.026

清华大学附属北京清华长庚医院清华大学临床医学院病理科

基金项目:

北京清华长庚医院青年启动基金资助项目(12017C1006)；

详细信息

中图分类号: R575
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出版历程
- 收稿日期: 2019-06-28
- 出版日期: 2019-11-20

Clinicopathological features of Caroli's disease: An analysis of 5 cases

Department of Pathology,Beijing Tsinghua Chang Gung Hospital,School of Clinical Medicine,Tsinghua University

Research funding:

摘要

摘要: 目的探讨Caroli病的临床特点、病理学特征、鉴别诊断及预后。方法对清华大学附属北京清华长庚医院2014年1月-2019年5月5例Caroli病患者的临床病理学资料进行回顾性分析并复习相关文献。结果 5例Caroli病患者平均年龄为20岁,男女比为3∶2,均因间断性上腹部疼痛就诊,4例伴有发热,2例伴脾肿大、门静脉高压,1例伴胆囊结石,4例伴双肾囊肿,4例肝功能异常。影像学检查示肝内胆管不同程度的囊状扩张,并与胆管树相通,囊内见纤维条索。病理示肝内胆管扩张,扩张的胆管壁内纤维组织增生,伴慢性炎细胞浸润,胆管腔内见纤维血管构成的条索状结构。Caroli病Ⅱ型还可见汇管区纤维化及增生、畸形的小胆管。结论 Caroli病好发于儿童及青少年,临床表现缺乏特异性,确诊依赖于影像学和病理学。肝内胆管囊性扩张,并与胆管树相通,囊内可见由纤维组织及血管构成的条索是Caroli病特征性的组织病理学表现。
- Caroli病 /
- 肝囊肿 /
- 病理学,临床
Abstract: Objective To investigate the clinical features,pathological features,differential diagnosis,and prognosis of Caroli's disease( CD). Methods A retrospective analysis was performed for the clinicopathological data of 5 parents with CD who were treated in Beijing Tsinghua Chang Gung Hospital from January 2014 to May 2019,and a literature review was also performed. Results There were 3 male and2 female patients,with a mean age of 20 years. All of them attended the hospital due to the presence of intermittent upper abdominal pain,and among these patients,4 had pyrexia,2 had splenomegaly and portal hypertension,1 had gallstones,4 had bilateral renal cysts,and 4 had abnormal hepatic function. Imaging examination showed varying degrees of cystic dilatation of the intrahepatic bile ducts which communicated with the biliary tree,and fibrous bands were observed in these cysts. Pathological examination showed intrahepatic bile duct dilatation with fibrous tissue proliferation and chronic inflammatory cell infiltration on the bile duct wall,and fibrovascular bundles were observed in the bile duct cavity. In addition,fibrosis and proliferation of the portal area and abnormal small bile ducts were observed in type Ⅱ CD. Conclusion CD is often seen in children and adolescents,and its clinical manifestations lack specificity. A confirmed diagnosis of CD relies on imaging and pathological examinations. Histopathological features of this disease include cystic dilatation of the intrahepatic bile ducts communicating with the biliary tree and the bands consisting of fibrous tissue and vessels in these cysts.
- Caroli disease /
- hepatic cyst /
- pathology,clinical

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参考文献(29)

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