CHRONIC CHOLESTATIC LIVER-DISEASE WITH ASSOCIATED TUBULOINTERSTITIAL NEPHROPATHY IN EARLY-CHILDHOOD

We report the clinical and morphological features of an unusual hepato renal disorder in 2 patients. The main clinical features were early on set of cholestatic liver disease and progressive tubulointerstitial ne phritis, leading to renal death in early childhood. Renal histology sh owed 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. Evaluati ng the 12 patients published in the literature, the long-term prognosi s of the liver function appears bad, suggesting the possibility of a c ombined liver and kidney transplantation.