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ACTA Pharmaceutica Sciencia 2025 , Vol 63 , Num 2
Evolution of plasma trace element status in children treated with low-protein diets related to Maple Syrup Urine Disease (MSUD) and Urea Cycle Disorder (UCD)
Özlem ARAZ 1 Halit ÇAM 1
1 Istanbul University-Cerrahpasa, Cerrahpasa Faculty of Medicine, Istanbul, Türkiye
DOI : 10.23893/1307-2080.APS6325 Viewed : 3086 - Downloaded : 1219 The main treatment for Urea Cycle Disorder (UCD) and Maple syrup urine disease (MSUD) is low-protein diets and element deficiencies may develop related to dietary therapy. This study aimed to determine plasma trace element levels in patients with MSUD and UCD and to compare these results with those established in a healthy pediatric population not undergoing any dietary treatment. The study was conducted with 30 patients diagnosed with MSUD and UCD, and a control group of 30 health age-matched children. Dietary treatment involved a natural protein-restricted diet supplemented with a special formula, depending on the specific metabolic defect. Significant differences were observed between patients and control values for magnesium, selenium, and copper (p<0.05). These results reinforce that patients under dietary treatment should be regularly monitored for these elements. Furthermore, fortifying these elements in special formulas (as they are the sources of essential amino acids) should be considered. Keywords : trace element, children, MSUD, UCD, protein-restricted diet

Istanbul Medipol University