Adherence to Dietary Recommendations, Nutrient Intake Adequacy and Diet Quality among Pediatric Cystic Fibrosis Patients: Results from the GreeCF Study.

Nutrition is an important component of cystic fibrosis (CF) therapy, with a high-fat diet being the cornerstone of treatment. However, adherence to the dietary recommendations for CF appears suboptimal and burdensome for most children and adolescents with CF, leading to malnutrition, inadequate growth, compromised lung function and increased risk for respiratory infections. A cross-sectional approach was deployed to examine the degree of adherence to the nutrition recommendations and diet quality among children with CF. A total of 76 children were recruited from Aghia Sophia's Children Hospital, in Athens, Greece. In their majority, participants attained their ideal body weight, met the recommendations for energy and fat intake, exceeding the goal for saturated fatty acids consumption. Carbohydrate and fiber intake were suboptimal and most participants exhibited low or mediocre adherence to the Mediterranean diet prototype. It appears that despite the optimal adherence to the energy and fat recommendations, there is still room for improvement concerning diet quality and fiber intake.

Comparison of International Growth Standards for Assessing Nutritional Status in Cystic Fibrosis: The GreeCF Study.

OBJECTIVE: The aim of the study was to compare 3 international growth references and explore their differences in assessing growth in Greek school-aged patients with cystic fibrosis (CF). METHODS: Sample included 114 patients (50 boys, age 11.5 ±â€Š3.9 years), provided care at Aghia Sofia Children's Hospital, Greece. Anthropometrics and predicted forced expiratory volume in 1 second (FEV1%) were measured. Body mass index (BMI) and height z scores were computed according to the Centers for Disease Control and Prevention (CDC), World Health Organization (WHO), and International Obesity Task Force (IOTF) references. Agreement between methods was analyzed with kappa statistics, repeated-measures analysis of variance, and Bland-Altman analysis. The relationship between FEV1% and BMI was explored with linear regression. RESULTS: Mean CDC BMI z score was the lowest (0.06 ±â€Š1.08), followed by WHO (0.17 ±â€Š1.14) and IOTF (0.35 ±â€Š1.05) (P ≤ 0.001 for all). The CDC and WHO growth references highly agreed for most weight status strata and stunting; all other comparisons produced lower agreements. Except for CDC and IOTF BMI z scores, all other comparisons produced wide levels of agreement and proportional bias. CDC reference classified more children as attaining low or normal weight, against WHO or IOTF (P ≤ 0.001 for all). Lowest prevalence of ideal and excess weight was recorded by CDC, compared to all other standards (P ≤ 0.001 for all). All BMI z scores provided moderate associations with FEV1%. CONCLUSION: Large variations across weight status classification were present when employing 3 growth standards in school-aged patients . Given than BMI z-scores from all references provided comparable associations with pulmonary function, our data indicate that no studied reference is better than others in assessing growth in CF.

High attainment of optimal nutritional and growth status observed among Greek pediatric cystic fibrosis patients: results from the GreeCF study.

BACKGROUND: Pediatric cystic fibrosis (CF) patients suffer high rates of undernutrition, subject to several parameters. We aimed to assess growth and nutritional status of Greek children and adolescents with CF. METHODS: Eighty-four patients (35 boys) formed the sample. Anthropometrics and FEV1 were measured, growth and weight status were assessed. Body mass index (BMI), arm circumference (MUAC), fat (MUAFA) and muscle (MUAMA) were calculated. RESULTS: In the total sample, 6.0% of the patients were underweight, 4.8% stunted, 8.3% wasted and 17.9% in nutritional failure, whereas 59.5% attained the ideal BMI for CF. FEV1 positively associated with BMI (B=0.03, p≤0.003), weight (B=0.03, p≤0.003) and MUAMA z-scores (B=0.04, p≤0.005). Meconium ileus negatively associated with FEV1 (B=-14.17, p≤0.003) and stature (B=-0.65, p≤0.043). Pancreatic insufficiency negatively influenced MUAC and MUAFA z-scores (p≤0.05 for both). CONCLUSIONS: The examined CF patients appear to be thriving. Unlike published research, the participants' sex, gene mutation and acquisition of pathogens did not affect growth.