PROGRESSIVE TUBULOINTERSTITIAL NEPHRITIS AND CHRONIC CHOLESTATIC LIVER-DISEASE

We report the clinical and morphological features of a distinctive hep atorenal disorder in four patients and review the five similar patient s in the literature. The main clinical characteristics were early onse t of cholestatic liver disease and progressive tubulointerstitial neph ritis leading to renal death in early childhood. Liver histology showe d disturbed architecture with nodular and acinar formations and portal fibrosis and bile duct proliferation. Histological abnormalities in t he kidney were severe interstitial fibrosis and tubular atrophy and di latation, while the typical features of nephronophthisis were lacking. These clinical and morphological characteristics distinguish our pati ents from the majority described, as having nephronophthisis and conge nital hepatic fibrosis or any other known syndrome with concomitant he patorenel involvement. We suggest that the association of cholestatic liver disease and progressive tubulointerstitial nephritis represents a new syndrome.