先天性肝纤维化并髓质海绵肾1例报道  

Congenital hepatic fibrosis with medullary sponge kidney:one case report

作　　者：杜白雪 李东颖[1] 马怡辉[2] 李建生[1] 

机构地区：[1]郑州大学第一附属医院消化科,河南郑州450052 [2]郑州大学第一附属医院病理科,河南郑州450052

出　　处：《胃肠病学和肝病学杂志》2016年第12期1384-1385,共2页Chinese Journal of Gastroenterology and Hepatology

摘　　要：先天性肝纤维化(congenital hepatic fibrosis,CHF)是一种少见的常染色体隐性遗传病,临床上以肝脏纤维化、门脉高压为主要特点,多合并多囊肾、髓质海绵肾,与多囊肾相比,髓质海绵肾在CHF合并的肾脏病变中所占比例较低,在我国文献发表的病例数不足百例。本文报道CHF合并髓质海绵肾1例。Congenital hepatic fibrosis （CHF） is a rare autosomal recessive inherited disease, which is characterized by liver fibrosis and portal hypertension. It always associated with polycystic kidney, medullary sponge kidney. Com- pared with polycystic kidney disease, medulla sponge kidney in CHF combined renal disease accounts for a lower propor- less than 100 cases. This paper reported one case of CHF with medullary sponge

关 键 词：先天性肝纤维化 髓质海绵肾 

分 类 号：R575.2[医药卫生—消化系统]