先天性非溶血性黄疸的研究进展  

Research advances in congenital non-hemolytic jaundice

作　　者：鲁杰 李武[1] 刘叶 LU Jie;LI Wu;LIU Ye(Department of Infectious Diseases,The First Affiliated Hospital of Kunming Medical University,Kunming 650032,China)

机构地区：[1]昆明医科大学第一附属医院感染性疾病科,昆明650032

出　　处：《临床肝胆病杂志》2021年第1期216-220,共5页Journal of Clinical Hepatology

基　　金：云南省自然科学基金(2009CD087);国家十二五科技重大专项协助课题(2012ZX10002003);云南省科技厅-昆明医科大学联合基金重点项目(2017FE468〔-173〕)。

摘　　要：先天性非溶血性黄疸是黄疸性疾病中的一大类,除母乳性黄疸外,临床上相对罕见,多属遗传代谢类肝病,包括以非结合性胆红素升高为主的Gilbert综合征、Crigler-Najjar综合征、Lucey-Driscoll综合征,和以结合胆红素升高为主的Dubin-Johnson综合征、Rotor综合征等。回顾近期国内外文献,主要就六种遗传性先天性非结合性黄疸的发病机制、基因特点、诊治进展及鉴别诊断进行综述。Congenital non-hemolytic jaundice is an important type of jaundice diseases,and except breast milk jaundice,the other types of this disease are relatively rare in clinical practice.Most of them belong to genetic and metabolic liver diseases,including Gilbert syndrome,Crigler-Najjar syndrome,and Lucey-Driscoll syndrome with an increase in unconjugated bilirubin and Dubin-Johnson syndrome and Rotor syndrome with an increase in conjugated bilirubin.With reference to the recent literature in China and foreign countries,this article reviews the pathogenesis,genetic characteristics,diagnosis,treatment,and differential diagnosis of six types of hereditary congenital unconjugated jaundice.

关 键 词：黄疸 吉尔伯特病 Crigler-Najjar综合征 黄疸 慢性特发性 

分 类 号：R575[医药卫生—消化系统]