The spectrum of clinical sequelae associated with alpha-1 antitrypsin deficiency

• मुख्य लेखकों: Vickram Tejwani (लेखक), James K. Stoller (लेखक)
• स्वरूप: पुस्तक
• प्रकाशित: SAGE Publishing, 2021-06-01T00:00:00Z.
• विषय: article
• ऑनलाइन पहुंच: Connect to this object online.

Alpha-1 antitrypsin (AAT) deficiency (AATD) is an autosomal co-dominant condition that predisposes to the development of lung disease, primarily emphysema. Emphysema results from the breakdown of lung matrix elastin by proteases, including neutrophil elastase, a protease normally inhibited by AAT. A...