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

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