Cystic Fibrosis Transmembrane Conductance Regulator Potentiation as a Therapeutic Strategy for Pulmonary Edema: A Proof-of-Concept Study in Pigs.

ABSTRACT:

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

SUBMITTER: Li X 

PROVIDER: S-EPMC5693779 | biostudies-literature | 2017 Dec

REPOSITORIES: biostudies-literature