Hepatobiliary fibropolycystic diseases

摘要: Summary The clinical, radiological and hepatic histological features of 51 patients with hepatobiliary fibropolycystic disease were reviewed. Many the had more than one diseases; combination both congenital fibrosis (CHF) Caroli's was most striking. Twelve CHF (50% male) presented at 6 ± 2 years age (mean SEM) hepatosplenomegaly or variceal bleeding. Their main problems recurrent bleeds renal disease. Polycystic kidneys stones present in 79% chronic failure 30%. Six 8 male (75%) later (aged 37 years) hepatomegaly cholangitis. Recurrent cholangitis developed (7/8) polycystic kidneys. a presenting hepatosplenomegaly, bleeding As disease, (83%) male, but presentation (15 4 years), incidence (42%) (8%) intermediate between In these patients, always predated Histologically, acute superimposed on changes CHF. Adult liver (10 patients) (43 3 females (90%) pain, mass incidentally; 33%. Microhamartomas patients), which usually incidental findings, diagnosed latest (50 years). Three choledochal cysts seen. hazard cancer diseases reflected by bile duct cancers 1 pancreatic (incidence 6%). This study has confirmed that form part family are often associated together. However, greatly differing severity prognosis an individual patient is determined present.