The Relative Contribution of Food Groups to Macronutrient Intake in Children with Cystic Fibrosis: A European Multicenter Assessment.

BACKGROUND: Optimal nutrition for children with cystic fibrosis (CF) improves prognosis and survival, but an increased caloric intake recommendation for this population raises concerns about the nutrient profile of their diets. OBJECTIVE: Our aim was to assess the relative contribution of food groups to the total macronutrient intake of European pediatric patients with CF. DESIGN: We conducted a cross-sectional study in which the participants recorded dietary intake from 2016 to 2017. Specifically developed nutritional composition databases were used to obtain nutritional data, including macronutrients and food groups, according to previously standardized criteria. PARTICIPANTS/SETTING: Two hundred and seven pediatric patients with CF from six European centers were involved in the My App for Cystic Fibrosis self-management project. MAIN OUTCOME MEASURES: Participants reported dietary intake with a detailed 4-day food record. STATISTICAL ANALYSIS PERFORMED: Descriptive analyses of nutrient intake, food group consumption, and dietary origin of macronutrients were conducted with R software. RESULTS: Similar patterns were found in nutrient and food group intake; both sugar and saturated fatty acids contributed >10% each to the total daily energy intake in all the centers. Large mean and median percent differences were observed in the intake of other nutrient and food groups, because sweets and snacks were consumed once or twice a day, and fruit and vegetables were consumed two or three times a day. Milk, meat, sweets and snacks, and oils were the main sources of fat in all centers. CONCLUSIONS: Study findings indicated less than optimal nutrient profiles, especially for sugars and saturated fatty acids, resulting from the high consumption of meat, dairy, and processed products and low consumption of fish, nuts, and legumes. These results can serve as a basis for future tailored interventions that target improved adherence to nutritional recommendations for patients with CF.

Expiratory muscle strength and functional exercise tolerance in adults with cystic fibrosis: a cross-sectional study.

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 (PE 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 PE max with 6MWW and other study variables. RESULTS: Twenty-five cystic fibrosis subjects aged 18-30 years and with FEV1 values ranging from 42.4% to 123.4% predicted completed the study. PE 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 PE 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.

Efficacy and tolerability of Creon for Children in infants and toddlers with pancreatic exocrine insufficiency caused by cystic fibrosis: an open-label, single-arm, multicenter study.

OBJECTIVES: To evaluate the efficacy and safety of a pancreatic enzyme preparation specifically developed for infants and small children with cystic fibrosis (CF). METHODS: Twelve patients with CF younger than 24 months with pancreatic exocrine insufficiency and a coefficient of fat absorption (CFA) less than 70% were treated with Creon for Children (Solvay Pharmaceuticals GmbH, Hannover, Germany) minimicrospheres for 8 weeks. The primary end point was the mean change from baseline in the CFA after 2 weeks of treatment, based on 72-hour fat balance assessments. RESULTS: Two weeks' treatment with Creon for Children resulted in a significant increase in the mean CFA from 58.0% at baseline to 84.7% (P=0.0013) in the full analysis sample. There was a significant reduction of mean stool fat (from 13.3 to 5.3 g/d; P=0.001) and mean fecal energy loss (from 238.5 to 137.9 kJ/d; P=0.018) at 2 weeks. Dietary fat intake did not change, whereas an improvement was observed in stool frequency and characteristics. Patient weight and height increased over 8 weeks of treatment. No serious adverse event was reported. CONCLUSIONS: Creon for Children was well tolerated and significantly decreased fat malabsorption in infants with pancreatic exocrine insufficiency due to CF.

Growth assessment of paediatric patients with CF comparing different auxologic indicators: A multicentre Italian study.

OBJECTIVES: To evaluate growth in Italian patients with cystic fibrosis (CF). PATIENTS AND METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

Dietary and circulating polyunsaturated fatty acids in cystic fibrosis: are they related to clinical outcomes?

OBJECTIVE: To assess the relationship between dietary intakes, plasma phospholipid (PL) fatty acid profile and clinical parameters in children with cystic fibrosis (CF) in comparison to healthy controls. PATIENTS AND METHODS: A cross-sectional survey including 37 patients with CF (ages 8.0 +/- 2.9 yrs) and a reference group of 68 healthy children (ages 8.0 +/- 0.7 yrs) was carried out by means of a food-frequency questionnaire. At enrollment, all subjects underwent blood sampling for plasma PL fatty acids (FA). In CF patients, pulmonary function tests (forced expiratory volume in 1 second and forced vital capacity), anthropometric measurements and the Shwachman score were also determined. RESULTS: In CF patients, mean z score for weight and height (-0.35 +/- 1.16 and -0.28 +/- 0.99) were lower than controls (0.83 +/- 1.73 and 0.55 +/- 1.11, respectively). Patients with CF showed higher energy intakes (110 +/- 43 kcal/d) compared with controls (75 +/- 22 kcal/d; P < 0.0001), with higher intake of total (saturated and monounsaturated) fats and lower intake of polyunsaturated FA (3.9 +/- 1.0% of total macronutrient intake vs 4.3 +/- 1.2%, P = 0.05). In CF patients, plasma and PL levels of linoleic and docosahexaenoic acids were lower, whereas those of arachidonic acid were similar compared with controls. The Shwachman score showed significant positive associations with plasma PL levels of arachidonic acid and total n-6 long-chain FA (r = 0.32, P = 0.05, and r = 0.35, P = 0.03, respectively). CONCLUSIONS: The data give suggestions that fat intake and CF-associated biomechanisms are bound in a vicious circle, concurring to create the clinical and biochemical picture of CF. The quantity and quality of fat supplementation in CF need careful attention to balance the fat supply with polyunsaturated FA.