Article
Ivacaftor-induced sweat chloride reductions correlate with increases in airway surface liquid pH in cystic fibrosis.
JCI insight - 9 Aug 2018
Abou Alaiwa Mahmoud H, Launspach Jan L, Grogan Brenda, Carter Suzanne, Zabner Joseph, Stoltz David A, Singh Pradeep K, McKone Edward F, Welsh Michael J
Abstract excerpt
BACKGROUND: Disruption of cystic fibrosis transmembrane conductance regulator (CFTR) anion channel function causes cystic fibrosis (CF), and lung disease produces most of the mortality. Loss of CFTR-mediated HCO3- secretion reduces the pH of airway surface liquid (ASL) in vitro and in neonatal humans and pigs in vivo. However, we previously found that, in older children and adults, ASL pH does not differ between...
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