Vitamin D intake, serum 25-hydroxy vitamin D and pulmonary function in paediatric patients with cystic fibrosis: a longitudinal approach.

Pancreatic-insufficient children with cystic fibrosis (CF) receive age-group-specific vitamin D supplementation according to international CF nutritional guidelines. The potential advantageous immunomodulatory effect of serum 25-hydroxy vitamin D (25(OH)D) on pulmonary function (PF) is yet to be established and is complicated by CF-related vitamin D malabsorption. We aimed to assess whether current recommendations are optimal for preventing deficiencies and whether higher serum 25(OH)D levels have long-term beneficial effects on PF.

Dietary intake and lipid profile in children and adolescents with cystic fibrosis.

Background: Cystic fibrosis (CF) patients are advised to derive 35% of their daily energy intake from dietary fat. Whether this high fat intake is associated with dyslipidaemia is unknown. We described the lipid profile and dietary intake in paediatric patients with CF.

Height Assessment in the Dutch-Origin Pediatric Cystic Fibrosis Population.

Background: Height evaluation is an integral part of cystic fibrosis (CF) care. Height is compared with reference values by converting it to height-for-age (HFA) z scores. However, HFA z scores do not adjust for genetic potential (ie, target height [TH]), which could result in an incorrect estimation of the height.

Vitamin A intake and serum retinol levels in children and adolescents with cystic fibrosis.

Background: Pancreatic insufficient cystic fibrosis (CF) patients receive vitamin A supplementation according to CF-specific recommendations to prevent deficiencies. Whether current recommendations are optimal for preventing both deficiency and toxicity is a subject of debate. We assessed the longitudinal relation between serum retinol levels and appropriate variables.

Pancreatic Enzyme Replacement Therapy and Coefficient of Fat Absorption in Children and Adolescents With Cystic Fibrosis.

Objectives: Pancreatic enzyme replacement therapy (PERT) is the proven therapy to substantially reduce fat malabsorption in patients with cystic fibrosis (CF). Few details of the daily practice regarding PERT and the resulting coefficient of fat absorption (CFA) are known. We therefore recorded the PERT and CFA in a large cohort of pancreatic insufficient pediatric patients with CF.

Vitamin E intake, α-tocopherol levels and pulmonary function in children and adolescents with cystic fibrosis.

Pancreatic insufficiency cystic fibrosis (CF) patients receive vitamin E supplementation according to CF-specific recommendations in order to prevent deficiencies. It has been suggested that higher serum α-tocopherol levels could have protective effects on pulmonary function (PF) in patients with CF. Whether current recommendations are indeed optimal for preventing deficiency and whether vitamin E has therapeutic benefits are subjects of debate.

The relationship between body growth and pulmonary function in children with cystic fibrosis.

Aim: To measure the weight and height of children with cystic fibrosis (CF) from 2 to 10 years of age and to investigate the relationship between these parameters and forced expiratory volume in 1 sec (FEV1) beginning at 6 years of age.

Methods: Weight and height were expressed as z-scores for weight-for-age (WFA), height-for-age (HFA), height-adjusted-for-target-height (HFA/TH) and weight-for-height (WFH). The children were categorised as having a z-score ≥0, between 0 and -1, or <-1 based on z-scores at 2 years of age.

Comparison of height for age and height for bone age with and without adjustment for target height in pediatric patients with CF.

Background: Height is a strong prognostic factor in cystic fibrosis (CF) and is usually compared to reference values of healthy children by expressing height as a z-score height-for-age (HFA). However, HFA does not take into account a potential delay in bone age (BA) and the genetic potential of the child and could therefore result in misclassification of short stature.

Methods: In 169 children with CF height, BA and target height (TH) were assessed.