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Alpha-1 antitrypsin deficiency alleles and severe cystic fibrosis lung disease
BACKGROUND—Alpha-1 antitrypsin (α(1)-AT) is the most abundant proteinase inhibitor within the lung. We have recently reported the surprising observation that cystic fibrosis patients with mild to moderate deficiency of α(1)-antitrypsin have significantly better pulmonary function than non-deficient...
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Main Authors: | , , , |
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Formato: | Artigo |
Idioma: | Inglês |
Publicado em: |
BMJ Group
1998
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Assuntos: | |
Acesso em linha: | https://ncbi.nlm.nih.gov/pmc/articles/PMC1745129/ https://ncbi.nlm.nih.gov/pubmed/10195072 |
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