[Alpha-1-antitrypsin deficiency and renal involvement].
In a young girl with alpha 1-antitrypsin deficiency (Pi Z), cirrhosis, hypocomplementemia and proteinuria, renal biopsy showed features of type I membranoproliferative glomerulonephritis. Deposits contained IgA, IgG, IgM, Clq, C4 and C3 but not alpha 1-antitrypsin. A review of the literature found 10 similar cases. Type I membranoproliferative glomerulonephritis was present in all. The respective roles of alpha 1-antitrypsin deficiency and hepatic disease in the pathogenesis are discussed; membranoproliferative glomerulonephritis is probably the consequence of the chronic liver disease.
D002648:Child; D005260:Female; D005921:Glomerulonephritis / Q000209:etiology* / Q000473:pathology; D005922:Glomerulonephritis, IGA / Q000209:etiology; D006801:Humans; D008103:Liver Cirrhosis / Q000150:complications; D010641:Phenotype; D019896:alpha 1-Antitrypsin Deficiency
Déficit en alpha-1-antitrypsine et atteinte rénale.