[Kidney polycystic disease as the major feature in three adults with congenital hepatic fibrosis. 3 cases (author's transl)].
Congenital hepatic fibrosis is nearly always associated with ectasia of collecting tubules of the kidneys. This abnormality usually remains silent. In this study we report three cases of adult's CHF with associated renal failure treated by hemodialysis. In all three cases, renal injuries were indistinguishable from those found in adult-type of polycystic disease. The kidneys of our third patient, who underwent two nephrectomies at a 14-years interval, showed ectasia of the collecting tubules with only a few cortical cysts. The second one showed numerous large cysts and only a few ectatic tubules. Our data indicate that: renal failure can complicate the CHF course in adults. Uremia can be the pressenting feature; polycystic kidneys in CHF are microscopically different from those found in adult-type, they might be considered as the final stage in ectasia of collecting tubules.
D000328:Adult; D006801:Humans; D007676:Kidney Failure, Chronic / Q000209:etiology / Q000473:pathology; D008107:Liver Diseases / Q000150:complications / Q000151:congenital* / Q000473:pathology; D008297:Male; D008875:Middle Aged; D007690:Polycystic Kidney Diseases / Q000150:complications*
Polykystose rénale, principale expression de la fibrose hépatique congénitale. 3 observations.