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Cystic Fibrosis Transmembrane Conductance Regulator Potentiation as a Therapeutic Strategy for Pulmonary Edema
| Content Provider | Scilit |
|---|---|
| Author | Li, Xiaopeng Buonfiglio, Luis Guillermo Vargas Adam, Ryan J. Stoltz, David A. Zabner, Joseph Comellas, Alejandro P. |
| Copyright Year | 2017 |
| Description | Journal: Critical Care Medicine Objectives: To determine the feasibility of using a cystic fibrosis transmembrane conductance regulator potentiator, ivacaftor (VX-770/Kalydeco, Vertex Pharmaceuticals, Boston, MA), as a therapeutic strategy for treating pulmonary edema. Design: Prospective laboratory animal investigation. Setting: Animal research laboratory. Subjects: Newborn and 3 days to 1 week old pigs. Interventions: Hydrostatic pulmonary edema was induced in pigs by acute volume overload. Ivacaftor was nebulized into the lung immediately after volume overload. Grams of water per grams of dry lung tissue were determined in the lungs harvested 1 hour after volume overload. Measurements and Main Results: Ivacaftor significantly improved alveolar liquid clearance in isolated pig lung lobes ex vivo and reduced edema in a volume overload in vivo pig model of hydrostatic pulmonary edema. To model hydrostatic pressure-induced edema in vitro, we developed a method of applied pressure to the basolateral surface of alveolar epithelia. Elevated hydrostatic pressure resulted in decreased cystic fibrosis transmembrane conductance regulator activity and liquid absorption, an effect which was partially reversed by cystic fibrosis transmembrane conductance regulator potentiation with ivacaftor. Conclusions: Cystic fibrosis transmembrane conductance regulator potentiation by ivacaftor is a novel therapeutic approach for pulmonary edema. |
| Related Links | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5693779/pdf |
| Ending Page | e1246 |
| Page Count | 7 |
| Starting Page | e1240 |
| ISSN | 00903493 |
| e-ISSN | 15300293 |
| DOI | 10.1097/ccm.0000000000002720 |
| Journal | Critical Care Medicine |
| Issue Number | 12 |
| Volume Number | 45 |
| Language | English |
| Publisher | Ovid Technologies (Wolters Kluwer Health) |
| Publisher Date | 2017-12-01 |
| Access Restriction | Open |
| Subject Keyword | Journal: Critical Care Medicine Respiratory System Animal Models of Human Disease Cystic Fibrosis Transmembrane Conductance Regulator Ion Channels Pulmonary Edema |
| Content Type | Text |
| Resource Type | Article |
| Subject | Critical Care and Intensive Care Medicine |
