RESUMO
Introduction: Patients with recessive dystrophic epidermolysis bullosa-severe subtype (RDEB-S) are at risk of vitamin K deficiency, potentially causing abnormal clotting, excessive bleeding, poor bone metabolism and abnormal vascular calcification. This study quantifies vitamin K deficiency prevalence in this cohort and identifies potential risk-factors to prevent deficiency. Methods: Patients with RDEB-S who attended the EB service between 2014 and 2020 were included. Serum vitamin K and PIVKAII were measured as part of the usual nutritional blood screen. Dietetic and medical notes were reviewed to establish: antibiotic use, enteral feed intake and micronutrient supplementation. Results: A total of 16/25 64% (10/16 female), of children aged 22-180 months, had serum vitamin K and PIVKAII analysed. Six of sixteen (37.5%) patients had vitamin K deficiency requiring supplementation. Two of six (33.3%) normalized serum vitamin K after 12 weeks supplementation with oral menadiol diphosphate. Four of six (66.6%) await retesting following supplementation. Six of six (100%) patients with vitamin K deficiency were not consuming a gastrostomy/sip feed. Nine of ten (90%) patients with sufficient vitamin K levels were consuming either; more than 200 ml prescribed sip feed or more than 400-800 ml gastrostomy feed daily (containing 5.9-11 µg/100 ml vitamin K). Patients who were consuming either more than 200 ml prescribed sip feed or more than 400-800 ml gastrostomy feed daily (containing 5.9-11 µg/100 ml vitamin K) were significantly less likely to suffer from vitamin K deficiency (0.08 odds ratio [(1/7)/(5/3)] with significance level p = 0.0342 [95% CI: 0.0074-0.8275]). Sixteen of sixteen (100%) received antibiotics (range 0-4 courses/year; median, 3; IQR, 3). Patients with the most frequent antibiotics (n = 4) had normal vitamin K and PIVKAII levels if they consumed a minimum of 200 ml prescribed sip feed or 400-800 ml gastrostomy feed daily. Sixteen of sixteen (100%) patients took a multivitamin/mineral supplement; none contained vitamin K. Summary: The prevalence of vitamin K deficiency is 37.5% in this cohort. Patients whom were not consuming gastrostomy/sip feeds of at least 200 ml daily were at greatest risk of vitamin K deficiency. Patients on a micronutrient supplement remain at risk of vitamin K deficiency, as most contain no vitamin K. Prescribing a vitamin/mineral supplement that contains vitamin K is recommended. Twelve-week supplementation of oral vitamin K (5 mg/day for 1-10 years and 10 mg/day for 12-17 years) adequately improved stores.
RESUMO
BACKGROUND: The mainstay of dietary management of food allergies remains the elimination diet. However, the removal of major food groups may predispose children to an inadequate nutrient intake. We therefore set out to establish growth status in food allergic children receiving dietetic input in the UK. METHODS: Dietitians were approached via the Food Allergy and Intolerance Specialist Group from the British Dietetic Association and asked to submit anthropometrical data for children with food allergies. Data collected related to the systems involved and number of foods excluded. Malnutrition was defined according to World Health Organization standards. RESULTS: Data from 13 different centres yielded 97 patients (51 male and 46 female) of which 66 excluded ≤2 foods and 31 excluded ≥3 foods. Data indicated that 8.5% had a weight for age ≤ -2 Z-score and, conversely, 8.5% were ≥2 Z-score. For height for age, 11.1% were ≤ -2 Z-score and, for weight for height, 3.7% were ≤ -2 Z-score and 7.5% ≥2 Z-score. Type of allergy, system involved and specific food elimination did not impact on the level of malnutrition. However, the elimination of ≥3 foods significantly impacted on weight for age (P = 0.044). CONCLUSIONS: The present study demonstrates that children with food allergies are more underweight than the general UK population, which appears to be linked to the number of foods excluded. However, the impact of the disease process itself should not be disregarded. Additionally, obesity can also occur in this population despite dietary elimination.
