Progressive tubulointerstitial nephritis and chronic cholestatic liver disease

We report the clinical and morphological features of a distinctive hepatorenal disorder in four patients and review the five similar patients in the literature. The main clinical characteristics were early onset of cholestatic liver disease and progressive tubulointerstitial nephritis leading to ren...

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Bibliographic Details
Published in:Pediatric nephrology (Berlin, West) West), 1993-08, Vol.7 (4), p.396-400
Main Authors: POPOVIC-ROLOVIC, M, KOSTIC, M, SINDJIC, M, JOVANOVIC, O, PECO-ANTIC, A, KRUSCIC, D
Format: Article
Language:English
Subjects:
Atrophy
Biological and medical sciences
Child, Preschool
Cholestasis, Intrahepatic - complications
Cholestasis, Intrahepatic - pathology
Chronic Disease
Female
Fibrosis
Humans
Infant
Interstitial nephritis
Kidney Tubules - pathology
Liver - pathology
Male
Medical sciences
Nephritis, Interstitial - complications
Nephritis, Interstitial - pathology
Nephrology. Urinary tract diseases
Nephrons - pathology
Nephropathies. Renovascular diseases. Renal failure
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Summary:We report the clinical and morphological features of a distinctive hepatorenal disorder in four patients and review the five similar patients in the literature. The main clinical characteristics were early onset of cholestatic liver disease and progressive tubulointerstitial nephritis leading to renal death in early childhood. Liver histology showed disturbed architecture with nodular and acinar formations and portal fibrosis and bile duct proliferation. Histological abnormalities in the kidney were severe interstitial fibrosis and tubular atrophy and dilatation, while the typical features of nephronophthisis were lacking. These clinical and morphological characteristics distinguish our patients from the majority described, as having nephronophthisis and congenital hepatic fibrosis or any other known syndrome with concomitant hepatorenal involvement. We suggest that the association of cholestatic liver disease and progressive tubulointerstitial nephritis represents a new syndrome.
ISSN:0931-041X
1432-198X
DOI:10.1007/BF00857550