primary ciliary dyskinesia
- related: Pulmonology
- tags: #literature
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disease: 1.primary ciliary dyskinesia
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screening test: 2.very low or absent nasal nitric oxide
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protease inhibitor deficiency
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associated with DNAl1 and DNAH5 gene mutation
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+/- liver cirrhosis
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IV infusion of pooled human alpha-1 proteinase inhibitor can be used to elevate AAT levels in blood1