ALPHA(1)-ANTITRYPSIN DEFICIENCY DEATHS IN THE UNITED-STATES FROM 1979-1991 - AN ANALYSIS USING MULTIPLE-CAUSE MORTALITY DATA

Objective: To describe trends of reported alpha(1)-antitrypsin deficie ncy mortality in the United States from 1979-1991. Methods: We analyze d death certificate reports in the multiple-cause mortality files comp iled by the National Center for Health Statistics. Results: Of the 26, 866,600 deaths that occurred during the 13-year period, 1,930 had alph a(1)-antitrypsin deficiency listed as a cause of death. Over this peri od, we would have expected 5,400 to 13,400 persons with this condition to die, The age-adjusted mortality rate with reported alpha(1)-antitr ypsin deficiency listed increased 86%, from 4.3 per 10 million in 1979 to 8.0 per 10 million in 1991, alpha(1)-Antitrypsin deficiency mortal ity rates were higher among whites than among blacks or persons of oth er races, alpha(1)-Antitrypsin deficiency was listed in 2.7% of all de aths with obstructive lung disease among persons aged 35-44 years old and in 1.2% of all deaths listing hepatic disease among children aged 1 to 14 years old. Conclusions: alpha(1)-Antitrypsin deficiency is an important risk factor for obstructive lung disease and hepatic disease in the United States, and it was reported with increasing frequency t hrough the study period, although it is still likely underreported.