Article
Association of alpha(1)-antichymotrypsin deficiency with milder lung disease in patients with cystic fibrosis.
Thorax - 1 Jan 2001
Mahadeva R, Sharples L, Ross-Russell R I, Webb A K, Bilton D, Lomas D A
Abstract excerpt
BACKGROUND: Cystic fibrosis (CF) is characterised by an excess of free proteinases that destroy lung tissue. Despite this, previous studies have shown that patients with CF with a mild deficiency variant of the proteinase inhibitor alpha(1)-antitrypsin have less, rather than more, severe pulmonary disease. Alpha(1)-antichymotrypsin is another important serine proteinase inhibitor that protects the lung against...
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