Chronic cholestatic liver disease with associated tubulointerstitial nephropathy in early childhood.

G. Montini, C. Carasi, L. Zancan, R. Dall'Amico, L. Murer, G. Zacchello, P. Sorino

Research output: Contribution to journalArticlepeer-review

Abstract

We report the clinical and morphological features of an unusual hepatorenal disorder in 2 patients. The main clinical features were early onset of cholestatic liver disease and progressive tubulointerstitial nephritis, leading to renal death in early childhood. Renal histology showed interstitial fibrosis, tubular atrophy and dilatation, glomerular cysts in the cortex and periglomerular fibrosis; liver histology was characterized by portal fibrosis and bile duct abnormalities. Evaluating the 12 patients published in the literature, the long-term prognosis of the liver function appears bad, suggesting the possibility of a combined liver and kidney transplantation.

Original languageEnglish
JournalPediatrics
Volume100
Issue number3
Publication statusPublished - Sep 1997

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

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