Alpha₁-Antitrypsin Deficiency

Relation of the Defect to Pulmonary DiseaseIt is clear that severely deficient subjects of phenotype PiZ and probably PiSZ are much more susceptible to the development of emphysema or chronic bronchitis (or both) than the general population, the large majority of whom are of phenotype PiM.57,58 The continuing question has been whether those with lesser degrees of deficiency, mainly PiMZ or PiMS, are at any greater risk for the development of such obstructive lung diseases than those of phenotype PiM.Initially, the problem was attacked by measurement of alpha1-antitrypsin levels in groups of patients with pulmonary diseases, but the . . .