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

Daly, Anne; Evans, Sharon; Chahal, Satnam; Surplice, I. M.; Vijay, Suresh; Santra, Saikat; MacDonald, Anita

doi:10.1111/jhn.12354

Cited by 2 publications

(3 citation statements)

References 13 publications

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“…Raw food diet Raw vegetables, fruits and seeds, milk, and eggs Greater risk than vegan diet Fruit diet Fresh and dried fruits, seeds, and some vegetables Greater risk than vegan diet Ketogenic diet Limited amount of carbohydrates Vitamin B, vitamin C, vitamin D, calcium, selenium, and magnesium [42,43] poor growth. On the contrary, position papers from North America state that well-planned vegetarian and vegan diets, when appropriately supplemented, are suitable for all life stages [32].…”

Section: Greater Risk Than Vegan Diet [31 • ]mentioning

confidence: 99%

“…The restriction of specific diet components potentially reduces micronutrient status; hence, a comprehensive vitamin and mineral supplement should be provided and becomes an essential adjunct to dietary treatment. Moreover, feeding differences are common in children with inherited metabolic disorders, with meals complicated by poor appetite, limited food variety, and lengthy mealtimes [41][42][43]. Phenylketonuria (PKU) is the most common inherited disorder of amino acid metabolism.…”

Section: Inherited Metabolic Diseasesmentioning

confidence: 99%

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Potential Micronutrient Deficiencies in the First 1000 Days of Life: The Pediatrician on the Side of the Weakest

Panzeri,

Pecoraro,

Dianin

et al. 2024

Curr Obes Rep

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Purpose of Review This study is to examine potential micronutrient deficiencies and any need for supplementation in children following specific diet plans in the first 1000 days of life. Recent Findings Optimal nutrition in the first 1000 days of life has a lifelong positive impact on child development. Specific intrauterine and perinatal factors, pathological conditions, and dietary restrictions can represent potential risk factors for micronutrient deficiencies in the first 1000 days of life, which can have negative systemic consequences. Preterm and low-birth-weight infants are intrinsically at risk because of immature body systems. Children affected by cystic fibrosis are prone to malnutrition because of intestinal malabsorption. The risk of micronutrient deficiency can increase in various situations, including but not limited to children following selective dietary regimens (vegetarian and vegan diets and children affected by specific neuropsychiatric conditions) or specific dietary therapies (children affected by food allergies or specific metabolic disorders and children following restricted diet as a part of therapeutic approach, i.e., ketogenic diet for epilepsy). In light of this situation, the micronutrient status in these categories of children should be investigated in order to tailor strategies specific to the individual’s metabolic needs, with a particular focus on deficiencies which can impair or delay the physical and cognitive development of children, namely, vitamin B12, vitamin D and folic acid, as well as oligo-elements such as iron, zinc, calcium, sodium, magnesium, and phosphorus, and essential fatty acids such as omega-3. Summary Identification of micronutrient deficiency in the first 1000 days of life and timely supplementation proves essential to prevent their long-term consequences. Graphical Abstract

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Section: Greater Risk Than Vegan Diet [31 • ]mentioning

confidence: 99%

Section: Inherited Metabolic Diseasesmentioning

confidence: 99%

Potential Micronutrient Deficiencies in the First 1000 Days of Life: The Pediatrician on the Side of the Weakest

Panzeri,

Pecoraro,

Dianin

et al. 2024

Curr Obes Rep

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“…Vitamins and minerals deficiencies were reported in those groups of patients which can rapidly impair growth [7,26]. That could be due to the insufficient adherence to the synthetic amino-acid based formula provided or as consequences of the multiple dietary modifications that may place the patients at risk of macro and micronutrient deficiency [27,28]. Zinc, selenium, vitamin B12 and other deficiencies were reported long time ago mainly in PKU patients and other IMD [17,20,[29], [30], [31], [32], [33], [34], [35], [36]].…”

Section: Introductionmentioning

confidence: 99%

The effects of low protein products availability on growth parameters and metabolic control in selected amino acid metabolism disorders patients

Handoom¹,

Megdad²,

Al-Qasabi³

et al. 2018

International Journal of Pediatrics and Adolescent Medicine

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BackgroundIn Saudi Arabia, a diet for life policy has been adopted in the management of amino acid metabolism disorders for years. However, the specially designed low protein products/medical foods - which are one of the important treatment tools - were not available up until several years ago in Saudi Arabia (SA). Our aim was to measure the compliance and quality of life in patients affected with these disorders followed in the metabolic nutrition clinic at King Faisal Specialist Hospital & Research Centre (KFSH&RC), Riyadh, SA.MethodologyWe used a non-randomized retrospective/prospective study which utilized the growth parameters, biochemical data of patients plus questionnaires collected from patients and their family/caregivers. A total of n = 182 patients affected with selected amino acid metabolism disorders were enrolled. Some were excluded n = 84 for various reasons. Sample analyzed were: Phenylketonuria (PKU) (44), Maple Syrup Urine Disease (MSUD) (30), Tyrosinemia (TYR) (17) and Homocystinuria (HCU) (7). Tandem Mass Spectrometry (TMS) used to quantitate plasma amino acid concentrations. Data was obtained using (COMPLE) Microsoft-Access which was designed by the metabolic nutrition clinic at KFSH&RC-Riyadh. Student's paired t-test was used to investigate relationship between variables.ResultsThe main findings were the improvement of selected amino acid levels pre and post the usage of medical foods. In PKU patients, the TMS Phenylalanine (PHE) levels post usage was significantly decreased (P value < .0001). This was also the case in MSUD patients with significant decrease in Leucine & Isoleucine levels (P value .0008) but not in Valine levels (P value .1148) as 36.7% of them received Valine supplements while enrolled in the study.ConclusionLow protein products availability was successful in improving outcomes for selected amino acid metabolic disorders. However, due to compliance issues and impracticality of the diet, the results were not significant in all enrolled patients.

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

Cited by 2 publications

References 13 publications

Potential Micronutrient Deficiencies in the First 1000 Days of Life: The Pediatrician on the Side of the Weakest

Potential Micronutrient Deficiencies in the First 1000 Days of Life: The Pediatrician on the Side of the Weakest

The effects of low protein products availability on growth parameters and metabolic control in selected amino acid metabolism disorders patients

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