Efficacy of Ivacaftor in a Child with Cystic Fibrosis, End-Stage Lung Disease, and Cepacia Syndrome

descriptionPublicationkeyboard_double_arrow_right Article 01 Dec 2012 English Publisher:SAGE PublicationsJournal:Pediatric Allergy, Immunology, and Pulmonology, volume 25, pages 231-233 (issn: 2151-321X, eissn: 2151-3228,

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Authors: Walter A. Castro-Elias; Shipra Singh; Peter W. Hiatt; Christopher M. Oermann; Robert H. Moore;

doi: 10.1089/ped.2012.0193

Efficacy of Ivacaftor in a Child with Cystic Fibrosis, End-Stage Lung Disease, and Cepacia Syndrome

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Abstract

Ivacaftor is a small-molecule potentiator of the cystic fibrosis (CF) transmembrane-conductance regulator and was recently approved for use in CF patients with at least one copy of the G551D mutation. Two clinical trials have proven its efficacy in CF patients 6 years of age and older with mild-to-moderate lung disease. In this article, we report the efficacy of ivacaftor in a 12-year-old CF patient with very severe lung disease and clinical features of cepacia syndrome.

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