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In Vivo and In Vitro Ivacaftor Response in Cystic Fibrosis Patients With Residual CFTR Function: N-of-1 Studies
Rationale: Ivacaftor, a cystic fibrosis transmembrane conductance regulator (CFTR) potentiator, decreases sweat chloride concentration, and improves pulmonary function in 6% of cystic fibrosis (CF) patients with specific CFTR mutations. Ivacaftor increases chloride transport in many other CFTR mutat...
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| Publicado no: | Pediatr Pulmonol |
|---|---|
| Main Authors: | , , , , , , , |
| Formato: | Artigo |
| Idioma: | Inglês |
| Publicado em: |
2017
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| Assuntos: | |
| Acesso em linha: | https://ncbi.nlm.nih.gov/pmc/articles/PMC5461115/ https://ncbi.nlm.nih.gov/pubmed/28068001 https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1002/ppul.23659 |
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