Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society
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Multicenter Study Comparative Study
Asians with cystic fibrosis in the UK have worse disease outcomes than clinic matched white homozygous delta F508 controls.
We tested the hypothesis that the Asian cystic fibrosis (CF) phenotype is comparable to the commonest genetic form of CF found in 50% of the white UK CF population using the UK CF Database, a national disease-specific patient registry. ⋯ These data suggest that the Asian CF phenotype is as severe as the white controls with the homozygous delta F508 phenotype but is worse in some outcomes, especially in Asian females. Socio-cultural factors and rare CF genotypes may contribute to the severity of CF in this vulnerable group.
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Comparative Study
Associations between clinical variables and quality of life in adults with cystic fibrosis.
The disease progression of cystic fibrosis (CF) is marked by an increase in clinical conditions and therapeutic interventions, which have the potential to affect health-related quality of life (HRQoL). This cross-sectional study explored associations between clinical variables and HRQoL. ⋯ While important associations were identified, much of the variance in HRQoL remains unexplained. Other clinical and psychosocial variables merit investigation. A longitudinal study is required to investigate how the disease trajectory and associated treatments affect an individual's quality of life.