当前位置： SCI文献检索 > JOURNAL OF HEPATOLOGY期刊下所有文献 > Severe alpha1-antitrypsin deficiency (PiZ homozygosity) with membranoproliferative glomerulonephritis and nephrotic syndrome, reversible after orthotopic liver transplantation.

Severe alpha1-antitrypsin deficiency (PiZ homozygosity) with membranoproliferative glomerulonephritis and nephrotic syndrome, reversible after orthotopic liver transplantation.

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.

journal_name

J Hepatol

journal_title

Journal of hepatology

authors

Elzouki AN,Lindgren S,Nilsson S,Veress B,Eriksson S

doi

10.1016/s0168-8278(97)80478-3

subject

Has Abstract

pub_date

1997-06-01 00:00:00

pages

1403-7

issue

6

eissn

0168-8278

issn

1600-0641

pii

S0168827897804783

journal_volume

26

pub_type

杂志文章

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