Literature DB >> 31066218

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

Rosemary Megalaa1, Vani Gopalareddy2, Elizabeth Champion1, Jennifer L Goralski3,4.   

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.
© 2019 Wiley Periodicals, Inc.

Entities:  

Keywords:  cystic fibrosis; modulator therapy; pancreatic insufficiency

Mesh：

Substances：

Year:  2019        PMID： 31066218     DOI： 10.1002/ppul.24353

Source DB:  PubMed          Journal:  Pediatr Pulmonol        ISSN： 1099-0496


  11 in total

Review 1.  An Update on CFTR Modulators as New Therapies for Cystic Fibrosis.

Authors:  John A King; Anna-Louise Nichols; Sian Bentley; Siobhan B Carr; Jane C Davies
Journal:  Paediatr Drugs       Date:  2022-05-16       Impact factor: 3.022

2.  Cystic Fibrosis Transmembrane Conductance Regulator Modulator Use Is Associated With Reduced Pancreatitis Hospitalizations in Patients With Cystic Fibrosis.

Authors:  Mitchell L Ramsey; Yevgeniya Gokun; Lindsay A Sobotka; Michael R Wellner; Kyle Porter; Stephen E Kirkby; Susan S Li; Georgios I Papachristou; Somashekar G Krishna; Peter P Stanich; Phil A Hart; Darwin L Conwell; Luis F Lara
Journal:  Am J Gastroenterol       Date:  2021-12-01       Impact factor: 10.864

Review 3.  Increasing life expectancy in cystic fibrosis: Advances and challenges.

Authors:  Kimberly A McBennett; Pamela B Davis; Michael W Konstan
Journal:  Pediatr Pulmonol       Date:  2021-11-11

4.  Current clinical opinion on CFTR dysfunction and patient risk of pancreatitis: diagnostic and therapeutic considerations.

Authors:  Madhura Y Phadke; Zachary M Sellers
Journal:  Expert Rev Gastroenterol Hepatol       Date:  2022-06-02       Impact factor: 4.095

5.  Elexacaftor-Tezacaftor-Ivacaftor as a Final Frontier in the Treatment of Cystic Fibrosis: Definition of the Clinical and Microbiological Implications in a Case-Control Study.

Authors:  Giuseppe Migliorisi; Mirella Collura; Francesca Ficili; Tiziana Pensabene; Dafne Bongiorno; Antonina Collura; Francesca Di Bernardo; Stefania Stefani
Journal:  Pharmaceuticals (Basel)       Date:  2022-05-14

Review 6.  The Exocrine Pancreas in Cystic Fibrosis in the Era of CFTR Modulation: A Mini Review.

Authors:  Isabelle R McKay; Chee Y Ooi
Journal:  Front Pediatr       Date:  2022-06-27       Impact factor: 3.569

Review 7.  New Therapies to Correct the Cystic Fibrosis Basic Defect.

Authors:  Christelle Bergeron; André M Cantin
Journal:  Int J Mol Sci       Date:  2021-06-08       Impact factor: 5.923

Review 8.  Demographics and risk factors for pediatric recurrent acute pancreatitis.

Authors:  Cheryl E Gariepy; Chee Y Ooi; Asim Maqbool; Kate M Ellery
Journal:  Curr Opin Gastroenterol       Date:  2021-09-01       Impact factor: 2.741

9.  Pancreatitis in A Patient with Cystic Fibrosis Taking Ivacaftor.

Authors:  Argyri Petrocheilou; Athanasios G Kaditis; Ioanna Loukou
Journal:  Children (Basel)       Date:  2020-01-12

Review 10.  The Extrapulmonary Effects of Cystic Fibrosis Transmembrane Conductance Regulator Modulators in Cystic Fibrosis.

Authors:  Valentine Sergeev; Frank Y Chou; Grace Y Lam; Christopher Michael Hamilton; Pearce G Wilcox; Bradley S Quon
Journal:  Ann Am Thorac Soc       Date:  2020-02
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