Micronutrient deficiency is a risk in children with inborn errors of metabolism

The challenges of vitamin and mineral supplementation in children with inherited metabolic disorders: a prospective trial

Daly et al., JHND Early View


In order to achieve metabolic stability, dietary treatment of inborn errors of metabolism may require restriction of protein, fat or carbohydrate. Manipulation of dietary intake potentially reduces micronutrient status, and provision of a comprehensive vitamin and mineral supplement becomes an essential adjunct to dietary treatment.


To review the efficacy of a new complete vitamin and mineral supplement [Fruitivits, Vitaflo Ltd] in 14 subjects in an open prospective 26-week study.


All subjects had dietary restrictions: low protein diets (57%, n = 8), regular daytime cornstarch and overnight glucose polymer tube feeds (29%, n = 4), low fat diet (7%, n = 1) and modified Atkins diet (7%, n = 1). Plasma nutritional biochemistry, anthropometry and food frequency questionnaires were collected at week 0, 12 and 26 weeks respectively.


Five nutritional parameters showed a significant improvement from baseline (week 0) to study end (week 26): folate (P = 0.01), vitamin E (P= 0.04), plasma selenium (P = 0.002), whole blood selenium (P = 0.04) and total vitamin D (P = 0.008). All the other nutritional markers did not significantly change. Even with regular monitoring, 37% of the product remained unused.


Despite improvements in some nutritional markers, overall use of the vitamin and mineral supplement was less than prescribed. New methods are needed to guarantee delivery of micronutrients in children at risk of deficiencies as a result of an essential manipulation of diet in inborn disorders of metabolism.


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