Longitudinal follow-up in adults with severe alpha-1 antitrypsin deficiency (Pi*ZZ genotype): non-invasive fibrosis tests predict liver-related endpoints

05.02 - Monitoring airway disease(2022)

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摘要
Aims and objectives Homozygous Pi*Z mutation (Pi*ZZ genotype) confers a strong predisposition to lung and liver disease. Since the pace of liver disease progression and prognostic factors remain unknown, we evaluated risk factors and the predictive utility of non-invasive tests in the European Pi*ZZ liver cohort.
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关键词
antitrypsin deficiency,non-invasive,liver-related
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