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A significant increase in life expectancy in successive birth cohorts of people with cystic fibrosis (CF) is a result of more effective treatment for the disease. It is also now widely recognized that outcomes for patients cared for in specialist CF Centres are better than for those who are not. Key to the effectiveness of the specialist CF Centre is the(More)
BACKGROUND Lung function is an important indicator of cystic fibrosis disease status and those with better forced expiratory volume in 1 s (FEV(1))% predicted have tended to report a better health-related quality of life (HRQoL) in cross-sectional studies. The relationship between lung function and HRQoL over time is unknown. This work assesses the natural(More)
Patients with cystic fibrosis (CF) are at risk of developing low bone mineral density (BMD) and fragility fractures. This paper presents consensus statements that summarise current knowledge of the epidemiology and pathophysiology of CF-related skeletal deficits and provides guidance on its assessment, prevention and treatment. The statements were validated(More)
OBJECTIVE This study examined the role of coping in explaining health-related quality of life (HRQoL) in cystic fibrosis (CF). METHODS Coping, quality of life, and clinical variables were assessed. The CF Coping Scale measures four ways of coping: optimistic acceptance, hopefulness, distraction, and avoidance. The Cystic Fibrosis Quality of Life(More)
OBJECTIVES The insights that people with cystic fibrosis have concerning their health are important given that aspects of health-related quality of life (HRQoL) are independent predictors of survival and a decrease in lung function is associated with a decrease in HRQoL over time. Cross-sectional data suggest that key variables, other than lung function,(More)
OBJECTIVES This study aims to generate experiential data about the decision-making process encountered by 12 women with cystic fibrosis (CF) considering pregnancy. DESIGN This study uses a qualitative grounded theory methodology to analyse the data and develop a conceptual hierarchical model of salient issues. METHODS Twelve women completed a(More)
Recently, the merger of artificial intelligence tech­ niques with database management concepts has sparked much interest. One important area is the development of a theory for relational database design based on absolute, incomplete, and uncertain knowledge about the domain. Tire approach presented here brings a new dimension to the traditional theory of(More)
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