OBJECTIVE To describe trends of reported alpha 1-antitrypsin deficiency mortality in the United States from 1979-1991. METHODS We analyzed death certificate reports in the multiple-cause mortality files compiled by the National Center for Health Statistics. RESULTS Of the 26,866,600 deaths that occurred during the 13-year period, 1,930 had alpha 1-antitrypsin deficiency listed as a cause of...

1-Antitrypsin (A1AT) 2 deficiency is a serious, lifethreatening disorder driven by abnormally low circulating concentrations of A1AT, which lead to protease– antiprotease imbalance. This imbalance accentuates protease activity and can lead to tissue damage, the most important of which leads to impairment of lung function (1 ). The apparent simplicity of diagnosing this disorder by a single meas...

An enzyme-linked immunosorbent assay (ELISA) has been developed for the quantification of cr,-antitrypsin-human leukocyte elastase (a,AT-E) complexes. In the ELISA, the a,AT-E complex is bound to a surface by rabbit antileukocyte elastase antibody, and the inhibitor-proteinase cornplex is quantified by a second antibody, rabbit anti-a1antitrypsin F(ab’)2. labeled with alkaline phosphatase. a1AT...

Alpha-1 antitrypsin deficiency is known as a significant genetic risk factor for COPD for carriers of phenotype PIMZ, and for phenotypes PIZZ and PISZ. Genetic epidemiological studies for alpha-1 antitrypsin deficiency conducted by others on both COPD patients and concurrent non-COPD controls were used to estimate the risk factors for all six phenotypic classes (namely, the normal phenotype PIM...