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Case Reports
. 2019 Aug;54(8):E16-E18.
doi: 10.1002/ppul.24353. Epub 2019 May 7.

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

Rosemary Megalaa  1 , Vani Gopalareddy  2 , Elizabeth Champion  1 , Jennifer L Goralski  3   4
Affiliations
Case Reports

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

Rosemary Megalaa et al. Pediatr Pulmonol. 2019 Aug.

Abstract

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.

Keywords: cystic fibrosis; modulator therapy; pancreatic insufficiency.

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