Ivacaftor and symptoms of extra-oesophageal reflux in patients with cystic fibrosis and G551D mutation.
J Cyst Fibros
; 16(1): 124-131, 2017 01.
Article
em En
| MEDLINE
| ID: mdl-27475719
BACKGROUND: Extra-oesophageal reflux (EOR) may lead to microaspiration in patients with cystic fibrosis (CF), a probable cause of deteriorating lung function. Successful clinical trials of ivacaftor highlight opportunities to understand EOR in a real world study. METHODS: Data from 12 patients with CF and the G551D mutation prescribed ivacaftor (150mg bd) was collected at baseline, 6, 26 and 52weeks. The changes in symptoms of EOR were assessed by questionnaire (reflux symptom index (RSI) and Hull airway reflux questionnaire (HARQ)). RESULTS: Six patients presented EOR at baseline (RSI >13; median 13; range 2-29) and 5 presented airway reflux (HARQ >13; median 12; range 3 to 33). Treatment with ivacaftor was associated with a significant reduction of EOR symptoms (P<0â04 versus baseline) denoted by the reflux symptom index and Hull airway reflux questionnaire. CONCLUSION: Ivacaftor treatment was beneficial for patients with symptoms of EOR, thought to be a precursor to microaspiration.
Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Refluxo Gastroesofágico
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Quinolonas
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Regulador de Condutância Transmembrana em Fibrose Cística
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Fibrose Cística
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Aspiração Respiratória
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Aminofenóis
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Pulmão
Tipo de estudo:
Diagnostic_studies
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Etiology_studies
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Observational_studies
Limite:
Adult
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Female
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Humans
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Male
País/Região como assunto:
Europa
Idioma:
En
Revista:
J Cyst Fibros
Ano de publicação:
2017
Tipo de documento:
Article
País de afiliação:
Reino Unido