Chapter

α‎<sub>1</sub>-antitrypsin deficiency

R. Mark Beattie, Anil Dhawan and John W.L. Puntis

in Paediatric Gastroenterology, Hepatology and Nutrition

Published on behalf of Oxford University Press

Published in print March 2009 | ISBN: 9780198569862
Published online October 2011 | e-ISBN: 9780191725968 | DOI: https://dx.doi.org/10.1093/med/9780198569862.003.0049

Series: Oxford Specialist Handbooks

α‎1-antitrypsin deficiency

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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. ...

Chapter.  626 words. 

Subjects: Gastroenterology ; Paediatrics

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