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Lung deposition of inhaled Alpha-1-proteinase inhibitor (Alpha(1)-PI) - problems and experience of Alpha(1)-PI inhalation therapy in patients with hereditary Alpha(1)-PI deficiency and cystic fibrosis
Alpha-1-proteinase inhibitor (α(1)-PI) is the most relevant protease inhibitor in the lung. Patients with hereditary deficiency of α(1)-PI suffer from an impaired hepatic synthesis of α(1)-PI in the liver and in consequence an insufficient concentration of the protease inhibitor in the lung followed...
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| Formato: | Online Artículo Texto |
| Lenguaje: | English |
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BioMed Central
2010
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| Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4360286/ https://www.ncbi.nlm.nih.gov/pubmed/21147646 http://dx.doi.org/10.1186/2047-783X-15-S2-164 |