Journal article
Pseudomonas eradication and clinical effectivness of Ivacaftor in four Hispanic patients with S549N
Pediatric pulmonology, Vol.52(7), pp.E37-E39
07/2017
DOI: 10.1002/ppul.23665
PMID: 28371569
Abstract
Ivacaftor was approved for rarer class-III CFTR mutations including S549N in 2014. Since these mutations are uncommon, ongoing reports of patient experiences with Ivacaftor and these mutations are important. This case series describes the clinical effectiveness (including airway infection status, lung function, and growth) of Ivacaftor therapy in four pediatric Hispanic patients with S549N and F508del over 24 months. In these patients, Ivacaftor was highly efficacious with no further Pseudomonas-positive cultures despite prior chronic colonization in three patients as well as notable improvements in lung function and growth. The remarkable improvements in lung function and growth were similar to G551D patients with more striking changes in airway infection status. Pediatr Pulmonol 2017;52:E37-E39. © 2017 Wiley Periodicals, Inc.
Details
- Title: Subtitle
- Pseudomonas eradication and clinical effectivness of Ivacaftor in four Hispanic patients with S549N
- Creators
- Abigail Strang - Department of Pulmonology, A.I. duPont Hospital for Children, Wilmington, DelawareAnthony J Fischer - Department of Pediatrics, University of Iowa Children's Hospital, Iowa City, IowaAaron Chidekel - Department of Pulmonology, A.I. duPont Hospital for Children, Wilmington, Delaware
- Resource Type
- Journal article
- Publication Details
- Pediatric pulmonology, Vol.52(7), pp.E37-E39
- DOI
- 10.1002/ppul.23665
- PMID
- 28371569
- ISSN
- 8755-6863
- eISSN
- 1099-0496
- Language
- English
- Date published
- 07/2017
- Academic Unit
- Pulmonary Medicine; Stead Family Department of Pediatrics
- Record Identifier
- 9984093353702771
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