Impact of CFTR modulation on intestinal pH, motility, and clinical outcomes in patients with cystic fibrosis and the G551D mutation
OBJECTIVES: A defect in bicarbonate secretion contributes to the pathophysiology of
gastrointestinal complications in patients with cystic fibrosis (CF). We measured
gastrointestinal pH, clinical outcomes, and intestinal transit profiles in patients with the
G551D mutation before and after treatment with ivacaftor, a CF transmembrane regulator
channel (CFTR) potentiator. METHODS: Observational studies of ivacaftor effectiveness
were conducted in the United States and Canada. A subset of subjects ingested a wireless …
gastrointestinal complications in patients with cystic fibrosis (CF). We measured
gastrointestinal pH, clinical outcomes, and intestinal transit profiles in patients with the
G551D mutation before and after treatment with ivacaftor, a CF transmembrane regulator
channel (CFTR) potentiator. METHODS: Observational studies of ivacaftor effectiveness
were conducted in the United States and Canada. A subset of subjects ingested a wireless …