RESUMO
BACKGROUND: Gastrostomy tube (GT) feeding is used to promote nutrition and growth in children with chronic kidney disease (CKD). We explored the relationship between gastrostomy feeding and growth parameters in children with CKD, looking specifically at the nutritional composition of feeds. METHODS: Children with CKD stages 3-5 or on dialysis in a tertiary children's kidney unit were studied. Data on anthropometry, biochemistry, and nutritional composition of feeds were collected from the time of GT insertion for 3 years or until transplantation. RESULTS: Forty children (18 female) were included. Nineteen children were on peritoneal dialysis, 8 on hemodialysis, and 13 had CKD stages 3-5. The median (interquartile range [IQR]) age at GT insertion was 1.26 (0.61-3.58) years, with 31 (77.5%) under 5 years of age. The median duration of gastrostomy feeding was 5.32 (3.05-6.31) years. None received growth hormone treatment. Children showed a significant increase in weight standard deviation score (SDS) (p = 0.0005), weight-for-height SDS (p = 0.0007) and body mass index (BMI) SDS (p < 0.0001). None of the children developed obesity. Although not statistically significant, the median height-SDS increased into the normal range (from -2.29 to -1.85). Weight-SDS positively correlated with the percentage of energy requirements from feeds (p = 0.02), and the BMI-SDS correlated with the percentage of total energy intake as fat (p < 0.001). CONCLUSION: GT feeding improves weight-SDS and BMI-SDS without leading to obesity. GT feeding improved height-SDS but this did not reach statistical significance, suggesting that factors in addition to nutritional optimization need to be considered for statural growth.
RESUMO
BACKGROUND: Paediatric patients with recessive dystrophic epidermolysis bullosa (RDEB) are at risk of vitamin D deficiency, owing to lack of sunlight from reduced mobility and having large areas of skin being covered with dressings, and to impaired nutritional intake and status. AIM: To establish an appropriate level of vitamin D supplementation in paediatric patients with RDEB. METHODS: Patients with RDEB attending the EB tertiary multidisciplinary team clinic were enrolled. Serum levels of total 25(OH)D were retrospectively recorded for the study period 2012-2018. Data from clinical records on supplements, bone mineral density (BMD) Z scores, compliance, and use of enteral feeds and/or formula were also recorded. RESULTS: In total, 24 patients met the inclusion criteria: 20 with severe RDEB, 3 with RDEB inversa and 1 with intermediate RDEB. Of the 24 patients, 21 (88%) were advised to take a vitamin D3 supplement in line with Department of Health Guidelines (UK), with the remaining 3 patients receiving sufficient intake from formula or enteral feeds. Thirteen of the 24 (54%) had vitamin D deficiency or insufficiency despite advice to supplement; 9 of these 13 (69%) subsequently started or increased the dosage of vitamin D supplements and levels became sufficient (> 50 nmol/L), while the remaining 4 patients (31%) continued to have persistent insufficient levels due to noncompliance with supplements. Reasons for noncompliance were palatability, cost and forgetting to take the tablets. The dose required to maintain sufficient serum levels increased with age, up to 300% of the reference nutrient intake (RNI). CONCLUSION: All patients with RDEB require a supplement or a formula or enteral/sip feed containing vitamin D to maintain sufficient serum vitamin D. The dose required increases with age and can be up to three times higher than the RNI for the normal population. Compliance may improve using a once-weekly loading dose of vitamin D3. Vitamin D deficiency was not solely causative of a low BMD Z score.
