The association of autosomal
recessive polycystic kidney disease (ARPKD) with congential hepatic fibrosis
(CHF) is well known; a rare occurrence is that of congenital hepatic fibrosis
with autosomal dominant polycystic kidney disease (ADPKD). We report a
family with ADPKD in which congenital hepatic fibrosis with severe portal
hypertension (PHT) presented in a 4-year-old girl; the kidneys were initially
normal. Typical changes of autosomal dominant polycystic kidney disease
developed in the next decade and were also found in the mother and sister
(neither of whom had any evidence of portal hypertension). Severe variceal
bleeding was treated by sclerotherapy and beta receptor blocage.
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