ABSTRACT

Spirometry in a 1 AT-deficient individuals will be emphasized, as these tests are performed most often and are reproducible and frequently reported. The spirometric abnormalities include reductions in expiratory flow rates (FEVj and FEF25_75) and a normal or slightly reduced forced vital capacity (FVC). The

cal changes are primarily due to loss of elastic recoil from parenchymal disease (emphysema) and narrowed or tortuous airways (2). The reduced elastic recoil pressure and increased compliance allow for lung hyperinflation with increases in residual volume and total lung capacity (3). Gas exchange is impaired, with reductions in the diffusing capacity DLCO (4) and widening of the alveolararterial gradient for oxygen (4,5).