Article
Epidemiology of alpha 1-protease inhibitor deficiency.
The European respiratory journal. Supplement - 1 Mar 1990
Hutchison D C
Abstract excerpt
Alpha 1-protease inhibitor can exist as over seventy 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 to 20% of the normal serum concentration of the inhibitor and have an increased risk of developing...
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