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  • Title: [Alpha-1-antitrypsin deficiency and renal involvement].
    Author: Lévy M, Gubler MC, Hadchouel M, Niaudet P, Habib R, Odièvre M.
    Journal: Nephrologie; 1985; 6(2):65-70. PubMed ID: 3875047.
    Abstract:
    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.
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