Kim M H, Ryu J S, Yang S K, Lee S K, Kim H R, Joung Y H, Lee Y S, Min Y I
Department of Internal Medicine, Asan Medical Center, Seoul, Korea.
Korean J Intern Med. 1990 Jul;5(2):101-7. doi: 10.3904/kjim.1990.5.2.101.
Congenital hepatic fibrosis is a relatively rare disease of children and young adults characterized by hard hepatomegaly, portal hypertension with relative preservation of liver function and underlying architecture, and frequent renal involvement. We experienced 3 cases of congenital hepatic fibrosis with Caroli's disease in 3 siblings, whose clinical manifestations were diverse, such as repeated cholangitis, variceal hemorrhage, or intrahepatic stones. All of them had multiple renal cysts, so we supposed that the clinical entities of these patients were in the spectrum of fibropolycystic disease of the liver and kidney.
先天性肝纤维化是一种相对罕见的儿童和青年疾病，其特征为肝脏肿大变硬、门静脉高压伴肝功能及基础结构相对保留，且常累及肾脏。我们诊治了3例患有卡罗里病的先天性肝纤维化同胞患者，他们的临床表现多样，如反复胆管炎、静脉曲张出血或肝内结石。他们均有多个肾囊肿，因此我们推测这些患者的临床病症属于肝肾纤维多囊病范畴。
