Evidence of Small Airways Disease and the Immediate Effects of Lumacaftor/Ivacaftor in Children with Cystic Fibrosis.
Ir Med J
; 113(5): 70, 2020 05 07.
Article
en En
| MEDLINE
| ID: mdl-32603566
Aim The aim of this study was to explore risk factors for acute changes in lung function following initiation of lumacaftor/ivacaftor (LUM/IVA) in children with cystic fibrosis. Methods Retrospective review of all children commenced on LUM/IVA treatment over a one-year period. CT Thorax images were reviewed for evidence of air trapping using the Brody score. Results Data was collected from 15 children. A transient decline in ppFEV1 was observed after initiation of LUM/IVA in 93% (n=14) of patients with an absolute mean decline of -10.8%. There was a statistically significant inverse relationship between ΔFEV1 and baseline ppFEV1. There was no relationship between air trapping score and ΔFEV1 (p=0.41). Conclusion Pre-existing small airways disease is not a risk factor for acute changes in lung function following initiation of LUM/IVA. Our results suggest that a LUM/IVA-related decline in lung function is more significant in CF children with higher baseline FEV1.
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Colección:
01-internacional
Banco de datos:
MEDLINE
Asunto principal:
Volumen Espiratorio Forzado
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Quinolonas
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Fibrosis Quística
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Enfermedad Pulmonar Obstructiva Crónica
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Benzodioxoles
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Resultados Negativos
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Aminofenoles
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Aminopiridinas
Tipo de estudio:
Etiology_studies
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Observational_studies
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Risk_factors_studies
Límite:
Adolescent
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Child
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Female
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Humans
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Male
Idioma:
En
Revista:
Ir Med J
Año:
2020
Tipo del documento:
Article