Three cases of congenital hepatic fibrosis with Caroli's disease in three siblings.
Korean J Intern Med
; 5(2): 101-7, 1990 Jul.
Article
em En
| MEDLINE
| ID: mdl-2098093
ABSTRACT
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.
Texto completo:
1
Base de dados:
MEDLINE
Assunto principal:
Ductos Biliares
/
Cirrose Hepática
Tipo de estudo:
Etiology_studies
Limite:
Adult
/
Humans
/
Male
Idioma:
En
Revista:
Korean J Intern Med
Assunto da revista:
MEDICINA INTERNA
Ano de publicação:
1990
Tipo de documento:
Article