Year to year change in FEV1 in patients with cystic fibrosis and different mutation classes.
J Cyst Fibros
; 16(2): 239-245, 2017 Mar.
Article
em En
| MEDLINE
| ID: mdl-27745801
ABSTRACT
In patients with cystic fibrosis, most treatments addressing the underlying basic defect are mutation or mutation class specific. These treatments are disease modifying if they lower the year to year change in lung function. We therefore calculated the current loss of lung function, measured by year to year change in forced expired volume in 1s in 11,417 patients included in the European Cystic Fibrosis Society Patient Registry. Whereas patients with at least one mutation of class IV or V have on average a lower year to year change, we did not find a difference between patients with a stop codon mutation, homozygous for F508del or at least one class III mutation. These data are useful background information to discuss the impact of different disease modifying treatments.
Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Volume Expiratório Forçado
/
Regulador de Condutância Transmembrana em Fibrose Cística
/
Fibrose Cística
/
Mutação
Tipo de estudo:
Diagnostic_studies
Limite:
Adult
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Female
/
Humans
/
Male
País/Região como assunto:
Europa
Idioma:
En
Revista:
J Cyst Fibros
Ano de publicação:
2017
Tipo de documento:
Article