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PEDIATRICS Vol. 100 No. 3 September 1997, p. e10
Copyright ©1997 by the American Academy of Pediatrics

ELECTRONIC ARTICLE:
Chronic Cholestatic Liver Disease With Associated Tubulointerstitial Nephropathy in Early Childhood

Received Aug 20, 1996; accepted Mar 6, 1997.

Giovanni Montini

Carla Carasi

Lucia Zancan

Roberto Dall'Amico

Luisa Murer

Graziella Zacchello

Department of Pediatrics University of Padova Padova, Italy

Palma Sorino

Giovanni XXIII Hospital Bari, Italy

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.

Key words: cholestasis, tubulointerstitial nephritis, end-stage renal disease, liver and kidney transplantation, childhood.