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1: Clin Nephrol. 2008 Aug;70(2):159-62.Links

alpha1-antitrypsin (A1AT) deficiency presenting with IgA nephropathy and nephrotic syndrome: is renal involvement caused by A1AT deposition?

Ting SM, Toth T, Caskey F.

Renal Specialist Registrar, Renal Department, Birmingham Heartlands Hospital, Birmingham, United Kingdom. stephen_ting@yahoo.com

The role of severe a1-antitrypsin (A1AT) deficiency in the predisposition of early-onset pulmonary emphysema and juvenile hepatic cirrhosis is well-established. Associated glomerulonephritis is unusual although it is well-recognized in children and young adults with the severe phenotype. We report the first adult case of A1AT deficiency presenting with nephrotic syndrome secondary to IgA nephropathy and explore the direct role of A1AT deposits in the pathogenesis of the renal involvement.

PMID: 18793532 [PubMed - indexed for MEDLINE]