1. Chronic Cholestatic Liver Disease With Associated Tubulointerstitial Nephropathy in Early Childhood
- Author
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Giovanni Montini, Roberto Dall'Amico, Lucia Zancan, Palma Sorino, Graziella Zacchello, Carla Carasi, and Luisa Murer
- Subjects
Male ,medicine.medical_specialty ,Pathology ,Tubular atrophy ,Cholestasis, Intrahepatic ,Kidney ,Gastroenterology ,Fibrosis ,Internal medicine ,Humans ,Medicine ,Early childhood ,Age of Onset ,business.industry ,Bile duct ,Infant, Newborn ,Infant ,medicine.disease ,Kidney Transplantation ,Liver Transplantation ,Transplantation ,medicine.anatomical_structure ,Liver ,Child, Preschool ,Portal fibrosis ,Chronic Disease ,Pediatrics, Perinatology and Child Health ,Kidney Failure, Chronic ,Nephritis, Interstitial ,Cholestatic liver disease ,Liver function ,business - 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.
- Published
- 1997