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α1-Antitrypsin Deficiency.
Clin Chest Med. 2016 Sep;37(3):487-504
Authors: Hatipoğlu U, Stoller JK
Abstract
α1-Antitrypsin deficiency is an autosomal codominant condition that predisposes to emphysema and cirrhosis. The condition is common but grossly under-recognized. Identifying patients' α1-antitrypsin deficiency has important management implications (ie, smoking cessation, genetic and occupational counseling, and specific treatment with the infusion of pooled human plasma α1-antitrypsin). The weight of evidence suggests that augmentation therapy slows the progression of emphysema in individuals with severe α1-antitrypsin deficiency.
PMID: 27514595 [PubMed - in process]