Original article
English
Elzouki AN, Lindgren S, Nilsson S, Veress B, Eriksson S.
Department of Medicine, Division of Gastroenterology and Hepatology, University Hospital, Malmo, Sweden.
J Hepatol. 1997 Jun;26(6):1403-7.
Abstract
BACKGROUND/AIMS: Nephropathy associated with alpha1-antitrypsin deficiency is assumed to be an unusual entity. We describe the case of a 23-year-old woman with severe alpha1-antitrypsin (PiZ homozygosity) deficiency who developed hepatic cirrhosis in childhood, and glomerulonephritis and nephrotic syndrome in adult life. METHODS/RESULTS: A renal biopsy was consistent with membranoproliferative glomerulonephritis. An immunofluorescence study revealed the presence of alpha1-antitrypsin (PiZ) in the subendothelial region of the glomerular basement membrane. The renal disease was reversible after orthotopic liver transplantation. CONCLUSIONS: The presence of abnormal PiZ protein in the subendothelial region of the glomerular basement membrane may suggest a possible role for this protein in the pathogenesis of glomerulonephritis. The case should add impetus to the search for alpha1-antitrypsin deficiency in any patient presenting with combined liver and renal disease, in the absence of evidence of hepato-renal syndrome, and illustrates that liver transplantation alone may reverse the nephropathy associated with alpha1-antitrypsin deficiency.
Keywords: Alpha 1-antitrypsin; Glomerulonephritis; Liver transplantation; Nephrotic syndrome.
Link/DOI: http://www.sciencedirect.com/science?_ob=ArticleURL&_udi=B6W7C-437YV9X-72&_user=10&_coverDate=07%2F31%2F1997&_rdoc=1&_fmt=&_orig=search&_sort=d&view=c&_acct=C000050221&_version=1&_urlVersion=0&_userid=10&md5=1a9040e1f13293daa68ce8928aba5cc3