Conditions / Genetic

alpha 1-antitrypsin deficiency

info · Genetic · ICD-10: E88.01

A plasma protein metabolism disease that has_material_basis_in defective production of the protease inhibitor alpha 1-antitrypsin (A1AT), leading to decreased A1AT activity in the blood and lungs, and deposition of excessive abnormal A1AT protein in liver cell

A plasma protein metabolism disease that has_material_basis_in defective production of the protease inhibitor alpha 1-antitrypsin (A1AT), leading to decreased A1AT activity in the blood and lungs, and deposition of excessive abnormal A1AT protein in liver cells.

Signs and symptoms

  • Gastric varix
  • Increased sputum production
  • Bronchiectasis
  • Cirrhosis
  • Cough
  • Decreased circulating alpha-1-antitrypsin concentration
  • Splenomegaly
  • Hemoptysis
  • Panacinar emphysema
  • Intrahepatic inclusion bodies

Medications that may treat it

alpha 1-antitrypsin

Also known as: AAT deficiency