Article
Alpha-1 antitrypsin deficiency alleles and severe cystic fibrosis lung disease.
Thorax - 1 Dec 1998
Mahadeva R, Stewart S, Bilton D, Lomas D A
Abstract excerpt
BACKGROUND: Alpha-1 antitrypsin (alpha 1-AT) is the most abundant proteinase inhibitor within the lung. We have recently reported the surprising observation that cystic fibrosis patients with mild to moderate deficiency of alpha 1-antitrypsin have significantly better pulmonary function than non-deficient patients. This study may have been biased as it did not include the most severely affected patients who have...
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