Article
Persistent recovery of pancreatic function in patients with cystic fibrosis after ivacaftor.
Pediatric pulmonology - 1 Dec 2020
Munce Danielle, Lim Meerana, Akong Kathryn
Abstract excerpt
Exocrine pancreatic insufficiency (EPI), which leads to malabsorption and poor weight gain, is seen in 85% of patients with cystic fibrosis (CF). EPI is treated with pancreatic enzyme replacement therapy taken with each meal. The highly effective cystic fibrosis transmembrane conductance regulator (CFTR) modulator, ivacaftor, restores CFTR function in patients with responsive mutations. It is a widely held view...
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