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Pediatr Transplant ; 25(4): e14007, 2021 Jun.
Article in English | MEDLINE | ID: mdl-33742748

ABSTRACT

Evidence for the extrapulmonary benefits of (CFTR) modulators is rapidly expanding. The use of CFTR modulators in CF patients who have undergone lung transplantation is not clear without guidance published in the medical literature to assist clinicians in the care of these patients. We discuss the potential benefits of CFTR modulators and provide insight into their use based on our experience in a small cohort of CF LTx recipients. We present pros and cons of CFTR modulator therapy for LTx recipients with CF. CFTR modulators should be considered in CF patients after lung transplantation for the time being until further research defines how to best use these therapies in transplant recipients.


Subject(s)
Aminophenols/therapeutic use , Benzodioxoles/therapeutic use , Cystic Fibrosis Transmembrane Conductance Regulator/metabolism , Cystic Fibrosis/drug therapy , Cystic Fibrosis/surgery , Indoles/therapeutic use , Lung Transplantation , Postoperative Care/methods , Pyrazoles/therapeutic use , Pyridines/therapeutic use , Quinolines/therapeutic use , Quinolones/therapeutic use , Adolescent , Biomarkers/metabolism , Combined Modality Therapy , Cystic Fibrosis/genetics , Cystic Fibrosis/metabolism , Cystic Fibrosis Transmembrane Conductance Regulator/genetics , Drug Combinations , Humans , Treatment Outcome , Young Adult
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