Congenital hepatic fibrosis with polycystic disease of the kidneys.

Three cases involving simultaneous occurrence of congenital hepatic fibrosis (CHF) and polycystic kidneys (PCK) are reported. In two infantile forms hepatic fibrosis accompanying polycystic renal lesion was revealed only on autopsy; the third patient has been kept under observation for seven years. Emphasis is laid on the importance of early diagnosis if serious complications are to be prevented. The pathogenesis of the disease is discussed on the basis of light and electron microscopic investigations of repeatedly taken liver and kidney biopsy material.