Ivacaftor: Five-year outcomes in the West of Scotland cystic fibrosis population.
Clin Respir J
; 17(5): 473-477, 2023 May.
Article
en En
| MEDLINE
| ID: mdl-36938952
ABSTRACT
INTRODUCTION:
Ivacaftor has shown to be effective in patients with cystic fibrosis (CF) with a G551D mutation.OBJECTIVES:
This work aims to evaluate ivacaftor's effectiveness and safety in the real world, over 5 years, in the West of Scotland CF population.METHODS:
We evaluated ivacaftor's effect on pulmonary function, body mass index (BMI), hospital bed occupancy, and adverse effects in patients ≥6 years with at least one G551D mutation.RESULTS:
Statistically significant increases from baseline were observed in mean per cent predicted forced expiratory volume in 1 s (FEV1 ) at year 1 (which was maintained at years 2 and 5) and BMI over 5 years in our adolescent/adult cohort. Improvements were observed in per cent predicted FEV1 within the paediatric cohort with a suggestion of a plateau effect. The increase in paediatric BMI z-score was nonstatistically significant. There was a reduction in the number of pulmonary exacerbations requiring intravenous antibiotics and hospital bed occupancy. Ivacaftor was well tolerated.CONCLUSION:
Ivacaftor was effective in our population.Palabras clave
Texto completo:
1
Banco de datos:
MEDLINE
Asunto principal:
Quinolonas
/
Fibrosis Quística
Tipo de estudio:
Prognostic_studies
Límite:
Adolescent
/
Adult
/
Child
/
Humans
Idioma:
En
Año:
2023
Tipo del documento:
Article