Article ID | Journal | Published Year | Pages | File Type |
---|---|---|---|---|
10047438 | American Journal of Kidney Diseases | 2005 | 6 Pages |
Abstract
Primary biliary cirrhosis is a chronic cholestatic liver disease of unknown cause that predominantly affects middle-aged women. Distal tubular acidosis is the main renal complication of primary biliary cirrhosis. Tubulointerstitial nephritis and Fanconi syndrome have been reported more rarely. We report on 2 patients with primary biliary cirrhosis who presented with tubulointerstitial nephritis and Fanconi syndrome and review similar cases published previously. Serum from 1 patient exerted an inhibitory effect on pyruvate dehydrogenase and α-ketoglutarate dehydrogenase, 2 mitochondrial enzymes that are the main targets of antimitochondrial antibodies in primary biliary cirrhosis. Antimitochondrial antibodies may have a role in the genesis of tubulointerstitial nephritis and Fanconi syndrome, 2 typical renal features of mitochondrial cytopathies. Tubulointerstitial nephritis and Fanconi syndrome have to be added to the spectrum of renal diseases associated with primary biliary cirrhosis.
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Authors
Marie MD, Raynald MD, Laure-Hélène MD, Natacha MD, Pierre MD, Laurent MD, Jeanne MD, Anne MD, Philippe MD, Bertrand MD, PhD, Jean-Pierre MD, Fadi MD,