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Paediatr Respir Rev · Jun 2018
Review Case ReportsCFTR modulator therapy in patients with cystic fibrosis and an organ transplant.
- Ruth M Mitchell, Andrew M Jones, and Peter J Barry.
- Manchester Adult Cystic Fibrosis Centre, Manchester University NHS Foundation Trust, Manchester, UK.
- Paediatr Respir Rev. 2018 Jun 1; 27: 6-8.
AbstractCFTR modulators are a class of drugs which directly target the defective CFTR protein in cystic fibrosis (CF), improving its function with resultant clinical improvements. Currently these drugs are confined to people with a limited selection of genetic mutations. New modulators are in development which will lead to the majority of patients with CF becoming eligible for treatment. CFTR modulators are currently considered contraindicated in patients with a solid organ transplant. This excludes many patients who may benefit from the multisystem effects of CFTR modulator treatment. In this review, we discuss issues regarding drug interactions, organ transplantation and CFTR modulation.Copyright © 2018 Elsevier Ltd. All rights reserved.
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