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Liver disease progression in patients with alpha-1 antitrypsin deficiency and protease inhibitor ZZ genotype with or without lung disease.

Alimentary pharmacology & therapeutics - 1 Nov 2023

Wu Tiffany, Hagiwara May, Gnass Esteban, Barman Hannah, Sasson David, Treem William, Ren Kaili, Marins Ed G, Karki Chitra, Malhi Harmeet

Abstract excerpt

BACKGROUND: Alpha-1 antitrypsin deficiency (AATD) is caused by mutations in SERPINA1, which encodes alpha-1 antitrypsin, a protease inhibitor (Pi). Individuals with AATD and the homozygous Pi*ZZ genotype have variable risk of progressive liver disease but the influence of comorbid lung disease is poorly understood. AIMS: To characterise patients with AATD Pi*ZZ and liver disease (AATD-LD-Pi*ZZ) with or without...

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PMID: 37718576
DOI: 10.1111/apt.17715

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