Alpha 1-Antitrypsin phenotypes in cor pulmonale due to chronic obstructive airways disease
Stockley, R.A.
Quarterly Journal of Medicine 48(191): 419-428
1979
ISSN/ISBN: 0033-5622 PMID: 317359 Document Number: 150198
The clinical and radiological features of 23 patients with cor pulmonale due to chronic obstructive airways disease were reviewed. Evidence of pulmonary vascular disease was seen in 22 patients and 52% had secondary polycythemia. Eighteen (78%) had radiological evidence of emphysema. Of the patients, 13 (56.5%) had .alpha.1-antitrypsin phenotypes associated with serum deficiency and of these the largest single group was the MZ phenotype (34.8%). No difference was found between the clinical features of patients with the MM phenotype or those associated with .alpha.1-antitrypsin deficiency, although radiological emphysema was more common in the latter group (92% vs. 60%).