Abstract
BackgroundBody habitus differences may explain some of the variation in lung function between individuals with cystic fibrosis (CF). We tested the hypothesis that measures of lean muscle mass and obesity are independently associated with lung function in CF. MethodsCross-sectional study design using UK CF registry data from 2096 clinically stable adults. ResultsSerum creatinine and BMI were positively and independently associated with FEV1 and FVC. One standard deviation increment in serum creatinine was associated with an FEV1 increase of 171ml (95% confidence intervals CI: +116 to +227ml) in males and 90ml (95% CI: +46 to +133ml) in females. Compared to the reference group of 20–24.9kg/m2, those with a BMI<20kg/m2 had lower FEV1 with values of −642ml (95%CI: −784 to −500ml) for males and −468ml (95%CI: −564 to −372ml) for females. ConclusionsProspective studies and controlled trials are required to ascertain if these associations have therapeutic potential in modifying disease progression.
Highlights
Cystic fibrosis (CF) is the most commonly inherited fatal disease in the Caucasian population
We have recently demonstrated that serum creatinine is inversely associated with mortality in adult patients with cystic fibrosis [25]
After stratification for gender, a single standard deviation change in serum creatinine was associated with changes in FEV1 + 171 ml (95% confidence intervals[CI}: + 116 ml to + 227 ml) for males and + 90 ml (95%CI: + 46 ml to + 133 ml) in females respectively with similar changes seen in forced vital capacity (FVC) (Table 2)
Summary
Cystic fibrosis (CF) is the most commonly inherited fatal disease in the Caucasian population. There have been a number of smaller cross-sectional studies demonstrating that lung function as measured by Forced Expiratory Volume in one second (FEV1) is positively associated with BMI, consistent with the hypothesis that both are measures of disease severity [6,7,8,9,10,11]. Data from the CF Foundation Registry has consistently demonstrated that this inverse association between BMI and lung function is present in population-based epidemiological data [12,13,14], and a study from the European Epidemiologic Registry of Cystic Fibrosis demonstrated lower function in those with decreased weight for height percentiles [15]. Using the national UK database for CF, we tested the hypothesis that two measures of body habitus, serum creatinine and BMI, are positively associated with lung function in clinically stable adults with a diagnosis of CF. Some of the results of these studies have previously been published in the form of an abstract
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