Article
The epidemiology of alpha 1-antitrypsin deficiency.
Lung - 1 Jan 1990
Hutchison D C
Abstract excerpt
Alpha 1-protease inhibitor can exist as over 70 different biochemical variants (the Pi system) which are inherited as autosomal-codominant alleles. The majority of these variants are of no clinical significance. Epidemiologically, the most abundant are Pi types M, S, and Z. Homozygotes of type Z have only 10%-20% of the normal serum concentration of the inhibitor and have an increased risk of developing pulmonary...
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