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Time for a gut check: Pancreatic sufficiency resulting from CFTR modulator use.

Rosemary MegalaaVani GopalareddyElizabeth ChampionJennifer L Goralski
Published in: Pediatric pulmonology (2019)
Pancreatic exocrine insufficiency in cystic fibrosis is genetically determined and generally felt to be irreversible. However, recent studies in young children started on cystic fibrosis transmembrane conductance regulator (CFTR) modulators have suggested improvement of pancreatic functioning over time. Here, we present the case of a 10-year-old child with pancreatic exocrine insufficiency since birth who regained pancreatic functioning after 4 years on the CFTR corrector drug, ivacaftor.
Keyphrases
  • cystic fibrosis
  • pseudomonas aeruginosa
  • lung function
  • emergency department
  • small molecule
  • transcription factor
  • chronic obstructive pulmonary disease
  • gestational age