title: Chronic cholestatic liver disease with associated tubulointerstitial nephropathy in early childhood.
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.
authors.affiliation: Department of Pediatrics, University of Padova, Padova, Italy.
authors.forename: G
authors.initials: G
authors.lastname: Montini
authors.forename: C
authors.initials: C
authors.lastname: Carasi
authors.forename: L
authors.initials: L
authors.lastname: Zancan
authors.forename: R
authors.initials: R
authors.lastname: Dall'Amico
authors.forename: L
authors.initials: L
authors.lastname: Murer
authors.forename: G
authors.initials: G
authors.lastname: Zacchello
authors.forename: P
authors.initials: P
authors.lastname: Sorino
mesh_terms: D017668:Age of Onset; D002675:Child, Preschool; D002780:Cholestasis, Intrahepatic / Q000150:complications* / Q000401:mortality; D002908:Chronic Disease; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007668:Kidney / Q000473:pathology; D007676:Kidney Failure, Chronic / Q000209:etiology; D016030:Kidney Transplantation; D008099:Liver / Q000473:pathology; D016031:Liver Transplantation; D008297:Male; D009395:Nephritis, Interstitial / Q000150:complications* / Q000401:mortality / Q000601:surgery