Abstract Objective A 6‐minute walk work (6MWW), that is the product of distance walked at the 6‐minute walk test (6MWT) multiplied by body weight, has been suggested as an appropriate variable for estimating functional capacity. Under the hypothesis that 6MWW strongly correlates with expiratory muscle strength (P E max), as found in Chronic Obstructive Pulmonary Disease (COPD), the aim of the study was to determine the degree of this correlation in adults with cystic fibrosis. Methods A cross‐sectional study was carried out at the Regional Referral Centre for Cystic Fibrosis, where patients with cystic fibrosis attending their regular follow‐up, aged 18 years or older with mild‐to‐normal lung disease, were asked to participate. Dietitians determined nutritional status before subjects performed spirometry and static measurement of respiratory muscles. The 6MWT was performed as the last study procedure. Spearman correlation test was used to correlate P E max with 6MWW and other study variables. Results Twenty‐five cystic fibrosis subjects aged 18–30 years and with FEV 1 values ranging from 42.4% to 123.4% predicted completed the study. P E max strongly correlated with 6MWW (rho .72, p = .0001) whereas it did not correlate with other study variables, including distance at the 6MWT. Distance walked multiplied by body weight ( p = .0019) and body mass index ( p = .0238) were significantly lower in those with P E max below the lower limits of normal. Conclusion Increased functional exercise tolerance is related to higher static expiratory pressure in patients with mild‐to‐normal cystic fibrosis lung disease. Difference in exercise performance might reflect a possible difference in body composition that needs to be further investigated.
To evaluate growth in Italian patients with cystic fibrosis (CF).A multicentre cross-sectional study was carried out on patients with CF attending Italian reference centres. Anthropometric data were evaluated using the Centers for Disease Control and Prevention 2000 reference data. Nutritional failure was defined as height-for-age percentile (HAP) <5th (all patients); weight-for-length percentile (WLP) <10th (patients <2 years); body mass index percentile (BMIp) <15th (patients between 2 and 18 years). The risk of malnutrition (defined as HAP, WLP, and BMIp <25th) and the proportion of patients below the "BMIp goal" (BMIp > or =50th) were also evaluated. Nutritional status was evaluated in the whole population and in relation to age, sex, pancreatic insufficiency, meconium ileus, and lung function.A total of 892 patients with CF (50.7% males, mean age 9.2 years, range 0.1-18 years) were enrolled. The proportion of children with HAP <5th, WLP<10th and BMIp<15th was 12.2%. 12.9%, 20.9%, respectively, and 54.4% did not fulfill the BMIp > or =50th goal. HAP <25th identified the highest proportion of children at risk of malnutrition, whereas BMIp <15th identified the highest proportion of children with nutritional failure. Whatever the criterion used to define malnutrition, the highest proportion of children with nutritional failure was found in adolescence (11-18 years). z scores for height, weight, and BMI were significantly associated with pancreatic status and lung function. Differences among centres for the auxologic parameters were not significant, except for BMIp.Nutritional failure is present in a minority of Italian patients with CF, particularly during adolescence. Different auxologic indicators should be used for identifying children at risk for or with actual malnutrition.
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