CT Imaging of Pediatric Patients with Cystic Fibrosis on Ivacaftor Therapy

We present two pediatric cystic fibrosis (CF) patients with DF508/G551D genotype, who had signicant improvement on computed tomography (CT) imaging of the chest. Based on our experience with these two patients, ivacaftor is effective in the treatment of CF patients with CFTR-G551D mutation. Pulmonar...

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Bibliographic Details
Published in:Lung 2014-10, Vol.192 (5), p.823-824
Main Authors: Hayes, Don, Long, Frederick R., McCoy, Karen S., Sheikh, Shahid I.
Format: Article
Language:English
Subjects:
Adolescent
Age Factors
Aminophenols - therapeutic use
Analysis
CAT scans
CT imaging
Cystic fibrosis
Cystic Fibrosis - diagnostic imaging
Cystic Fibrosis - drug therapy
Cystic Fibrosis - genetics
Cystic Fibrosis - physiopathology
Cystic Fibrosis Transmembrane Conductance Regulator - agonists
Cystic Fibrosis Transmembrane Conductance Regulator - genetics
Diagnosis
Dosage and administration
Drug therapy
Forced Expiratory Volume
Genetic Predisposition to Disease
Humans
Ivacaftor
Lung - diagnostic imaging
Lung - drug effects
Lung - physiopathology
Male
Medicine
Medicine & Public Health
Mutation
Patient outcomes
Pediatrics
Phenotype
Pneumology/Respiratory System
Predictive Value of Tests
Quinolones - therapeutic use
Recovery of Function
Respiratory System Agents - therapeutic use
Risk factors
Time Factors
Tomography
Tomography, X-Ray Computed
Treatment Outcome
Vital Capacity
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Description
Summary:We present two pediatric cystic fibrosis (CF) patients with DF508/G551D genotype, who had signicant improvement on computed tomography (CT) imaging of the chest. Based on our experience with these two patients, ivacaftor is effective in the treatment of CF patients with CFTR-G551D mutation. Pulmonary function signicantly improved in one patient while not in the other. Despite no alteration in pulmonary function in the male patient, CT scan of the chest clearly showed an improvement with ivacaftor therapy. CT imaging of the chest is a useful tool to evaluate response to ivacaftor in children with CF having CFTR-G551D mutation.
ISSN:0341-2040
1432-1750
DOI:10.1007/s00408-014-9629-y