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α‎1-antitrypsin deficiency 

α‎1-antitrypsin deficiency
Chapter:
α‎1-antitrypsin deficiency
Source:
Paediatric Gastroenterology, Hepatology and Nutrition (Oxford Specialist Handbooks in Paediatrics) (1 ed.)
Author(s):

R. Mark Beattie

, Anil Dhawan

, and John W.L. Puntis

DOI:
10.1093/med/9780198569862.003.0049

Clinical features 354

Management 355

Liver disease in other forms of α‎1-AT deficiency 355

α‎1-Antitrypsin (α‎1-AT) is a 55 kD glycoprotein produced predominantly by hepatocytes, alveolar macrophages and intestinal endothelial cells. It acts as a protease inhibitor during an acute-phase response. It can be electrophoretically differentiated into four main variants: PiM (normal), PiZ, PiS, and Pi Null (abnormal). There are >80 other rare, clinically irrelevant, variants. ...

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