Research Article: Prevalence of Cardiovascular Disease and Rate of Major Adverse Cardiovascular Events in Severe Alpha- Antitrypsin Deficiency COPD
Introduction:
Alpha- antitrypsin deficiency (AATD) is an autosomal co-dominant disorder characterised by reduced serum levels of alpha- antitrypsin (AAT). AAT is an important serine protease inhibitor which has a role in limiting the destructive effects of proteases. Patients with severe forms of AATD, most commonly PiZZ, have significantly reduced levels of circulating AAT. Uninhibited activity of proteolytic enzymes leads to accelerated elastin degradation within alveolar tissue, resulting in early-onset pulmonary emphysema.…
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