Protease inhibitor variants in children and young adults with chronic asthma.
The results of systematic medical histories and pulmonary function tests on 26 children and young adults, 10 with Pi variant alpha1-antitrypsin phenotypes and 16 Pi normals, are reported. These subjects were selected from 57 consecutive patients who had chronic asthma. The incidence of Pi variants was 20.4% among 46 Caucasian subjects (ages seven to 21 years). The Pi variant patients required more bronchodilators and long-term corticosteroid therapy. Among the parents and siblings, 50% of those with abnormal Pi type had chronic obstructive lung disease. Grandparents of the Pi abnormal probands had greater incidence of chronic obstructive lung disease (P less than 0.05). Baseline specific airways conductance and maximal midexpiratory flow rates did not discriminat between the two groups. However, bronchodilator inhalation showed the Pi variant group to be significantly less responsive than the Pi normal group. Pi heterozygous state, particularly Pi MZ, should be considered when a young person with chronic asthma is unresponsive to adequate doses of bronchodilators and needs prolonged use of corticosteroids.
D000293:Adolescent; D001249:Asthma / Q000188:drug therapy / Q000235:genetics*; D002648:Child; D002908:Chronic Disease; D005260:Female; D014644:Genetic Variation; D005838:Genotype; D006801:Humans; D008297:Male; D010641:Phenotype; D011480:Protease Inhibitors / Q000235:genetics*; D012129:Respiratory Function Tests; D019896:alpha 1-Antitrypsin Deficiency
D011480:Protease Inhibitors