Restoration of exocrine pancreatic function in older children with cystic fibrosis on ivacaftor.

A L Nichols, J C Davies, D Jones, S B Carr

Journal: Paediatric respiratory reviews 2021;35():99-102

PMID: 32386958

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Abstract

["Prior to the use of cystic fibrosis (CF) modulator therapy, exocrine pancreatic insufficiency in CF was thought to be irreversible. Improvement in pancreatic function on ivacaftor has been reported in open label studies in 1-5\u202fyear olds. The mechanism by which ivacaftor might improve exocrine pancreatic function is unclear. Although the effect of ivacaftor on pancreatic function may be more significant in younger children, evidence is mounting that there may still be potential for improvement in older children on long term therapy.",{"copyright":"Copyright \u00a9 2020 Elsevier Ltd. All rights reserved."}]

Address: Department of Paediatric Respiratory Medicine, Royal Brompton and Harefield NHS Foundation Trust, London, UK. Electronic address: [email protected].; National Heart and Lung Institute, Imperial College London, London, UK; Department of Paediatric Respiratory Medicine, Royal Brompton and Harefield NHS Foundation Trust, London, UK.; Department of Paediatric Respiratory Medicine, Royal Brompton and Harefield NHS Foundation Trust, London, UK.; Department of Paediatric Respiratory Medicine, Royal Brompton and Harefield NHS Foundation Trust, London, UK; National Heart and Lung Institute, Imperial College London, London, UK.

DOI: 10.1016/j.prrv.2020.04.003

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MeSH Terms: Adolescent, Age Factors, Aminophenols, Carrier Proteins, Chloride Channel Agonists, Cystic Fibrosis, Duration of Therapy, Enzyme Replacement Therapy, Exocrine Pancreatic Insufficiency, Feces, Female, Humans, Pancreatic Elastase, Quinolones, Recovery of Function