Serum α1-antitrypsin (α1AT) levels and protease inhibitor (PI) phenotypes were determined in 176 patients with bronchial asthma. The results were compared with 121 nonasthmatic controls. In the bronchial asthma group, 144 patients (81.8%) had M phenotype, compared to 118 (97.5%) in the controls. Severe deficiency of α1AT associated with Z phenotype were found in 18 patients (10%), compared to 2 (1.6%) in the controls. Intermediate deficiency associated with MZ and FZ phenotypes were found in 9 (5%) patients with bronchial asthma. MS phenotype was found in 2, MI in 2, and FM in 1 patient with bronchial asthma. The severely deficient group of asthmatics with Z phenotype was found to be heterogenous in clinical presentation. When compared to the asthmatic patients with M phenotype, no statistically significant difference was found between the two groups regarding the type of asthma, severity of the disease, steroid dependency, smoking habits or age at onset. The finding that deficient phenotpyes were more frequent in the asthmatic group suggests that this may be an important contributing factor in the development of bronchial asthma. Exaggerated immunologic and inflammatory responses in such patients, together with the harsh environmental conditions prevailing in Kuwait, may lead to the development of bronchial asthma in subjects deficient in α1AT.
SUMMARY This is a report of haemoglobin E trait in a black American family with no known Asian ancestory. The father appears to be heterozygous for both haemoglobin E and o-thalassaemia. The mother is normal both clinically and haematologically. Three children carry Hb E trait alone. The youngest son has a normal haemoglobin pattern and appears to have oc-thalassaemia.
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