ABSTRACT
BACKGROUND & AIMS: The recommendation for caloric intake in CF patients is to obtain intakes between 110 and 200% of the estimated average requirement (EAR) for age groups and gender, of which 35-40 energy% should be from fat. It is questionable whether the advice is met. METHODS: 1726 Completed 3-day dietary food records of 234 CF patients (111 girls) and 2860 completed two non-consecutive 24-h dietary assessments of healthy controls (1411 girls) were studied. The dietary intake in CF patients was compared with that of healthy controls by using independent sample t tests. RESULTS: Caloric intake in children with CF varied highly with age (88-127% EAR), which is below or in the lower range of the recommended 110-200% EAR. However the absolute caloric intake in CF children was significantly higher compared to controls at all ages. In addition, apart from boys aged 1-3 years, all CF children had a fat intake of 35 energy% or more. This fat intake was significantly higher than in controls, as was the consumption of saturated fat, the latter being well above 10% of the total energy intake. CONCLUSION: Although CF patients generally do not meet the EAR recommendations, they had a significantly higher caloric intake than controls. Moreover fat intake in CF patients does generally meet recommendations, but this resulted in a considerable consumption of saturated fat; a reduction of the latter seems appropriate.
Subject(s)
Cystic Fibrosis/diet therapy , Diet Records , Energy Intake , Nutritional Status , Adolescent , Body Height , Body Weight , Case-Control Studies , Child , Child, Preschool , Dietary Carbohydrates , Dietary Fats , Dietary Proteins , Female , Humans , Infant , Male , Nutrition Assessment , Nutritional Requirements , Retrospective StudiesABSTRACT
Cystic fibrosis (CF) is classically attributed to the dysfunction of the single CF transmembrane conductance regulator gene. The incidence of human leukocyte antigen (HLA) polymorphisms in different CF-associated diseases raises the question of an unequal distribution of HLA genotypes in CF. This study aimed to evaluate HLA gene frequencies and possible associations in CF patients compared with a control population. Frequencies of HLA-DRB1, HLA-DQA1 and HLA-DQB1, performed by intermediate resolution typing using Luminex sequence-specific oligonucleotide, and epitope counts were similar in 340 CF patients when compared with 400 control subjects. In conclusion, HLA-DRB1, -DQA1 and -DQB1 do not seem to influence susceptibility to CF. Whether HLA plays a role in the severity of CF disease needs to be investigated.
Subject(s)
Cystic Fibrosis/genetics , Cystic Fibrosis/immunology , HLA Antigens/immunology , Cystic Fibrosis Transmembrane Conductance Regulator/genetics , DNA Mutational Analysis , Female , Gene Frequency , Genetic Association Studies , Genetic Predisposition to Disease , Genotype , HLA Antigens/genetics , Humans , Male , Polymorphism, GeneticABSTRACT
BACKGROUND: To systematically assess the literature published after 1997 describing the effectiveness of nutritional interventions in Cystic Fibrosis patients. METHODS: An online search in PUBMED, EMBASE and COCHRANE databases was conducted. Original studies with 4 patients or more, describing a nutritional intervention and giving at least weight as an outcome parameter were included. RESULTS: The inclusion criteria were met by 17 articles, focusing on respectively behavioural interventions (n=6), oral supplementation (n=4) or enteral tube feeding (n=7). This latter intervention was universally successful to induce weight gain. One behavioural study and 2 oral supplementation studies also reported significant weight gain. CONCLUSION: Enteral tube feeding is effective to improve nutritional status, while the described effects of behavioural intervention and oral supplementation are not consistent at present.
