AIRWAYS OBSTRUCTION AND 2-YEAR SURVIVAL IN PATIENTS WITH SEVERE ALPHA(1)-ANTITRYPSIN DEFICIENCY

Because of the limited number of donor organs available, the selection of patients for lung transplantation is crucial. One important issue in setting priorities is the life expectancy without transplantation. The purpose of this study was to estimate survival, based on lung func tion, in alpha(1)-antitrypsin deficient patients. Data from the Danish alpha(1)-antitrypsin deficiency registry were analysed. The basic ele ment of the analysis was two year intervals, characterized by date of spirometry and known mortality status 2 yrs later. We found a simple e xponential relationship between lung function (forced expiratory volum e one second (FEV(1))) and two year survival on conservative treatment . The formula implies an almost 100% two year survival until FEV(1) fa lls below one emphysema third of predicted normal; at this level two y ear mortality increases and will reach 50% at a FEV(1) of 15% predicte d. In conclusion, the two year mortality of emphysema patients due to alpha,antitrypsin deficiency increases exponentially with decreasing F EV(1), and the results imply that only a few patients who underwent lu ng transplantation would have had a better two year prognosis without this procedure.