Selenium intake and nutritional status of children with phenylketonuria in Minas Gerais, Brazil

Abstract

Objective: To evaluate selenium dietary intake and nutritional status of patients with phenylketonuria. Methods:The study prospectively evaluated 54 children with phenylketonuria, from 4 to10 years old. The study was performed before and after the use of a selenium-supplemented amino acid mixture. The second phase of the study was performed after, at least, 90 days of use of the supplementation. Selenium nutritional status was assessed through the analysis of biochemical parameters: serum free thyroxin and selenium and glutathione peroxidase in erythrocytes. Selenium dietary intake was evaluated by the administration of the Food Frequency Questionnaire.Results: Mean age of the children was of 7.0±1.8 years, and 35.2% were female. Mean time of supplementation of selenium, on special formula, was 122.2±25.1 days. The selenium-supplemented amino acid mixture represented 72.9% of the daily supply of the mineral. Upon supplementation, mean concentrations of serum selenium and glutathione peroxidase in erythrocytes increased significantly (p < 0.05). The average daily intake of selenium increased significantly (p < 0.001), reaching the levels recommended by the Dietary Reference Intakes. The concentration of free thyroxin, in serum, presented significant reduction (p < 0.001) in all patients during the second phase of the study, and returned to normal limits in those who had changed levels. Conclusion:Selenium supplementation through protein replacement is effective to improve and adapt the nutritional status of selenium in patients with phenylketonuria.J Pediatr (Rio J). 2012;88(5):396-400: Phenylketonuria, selenium, glutathione peroxidase, thyroid hormones. ResumoObjetivo: Avaliar a ingestão alimentar e o estado nutricional em selênio em pacientes com fenilcetonúria. Métodos:Foram avaliados prospectivamente 54 crianças com fenilcetonúria, entre 4 e 10 anos de idade. O estudo foi realizado antes e após o uso de mistura de aminoácidos complementada com selênio. A segunda fase do estudo foi realizada com, no mínimo, 90 dias de utilização da mistura complementada. O estado nutricional em selênio foi avaliado por meio da análise de parâmetros bioquímicos: dosagens séricas de selênio e tiroxina livre e dosagem de glutationa peroxidase no eritrócito. A ingestão alimentar de selênio foi avaliada por aplicação de Questionário de Frequência Alimentar Quantitativo.Resultados: A idade média das crianças foi de 7,0±1,8 anos, e 35,2% eram do sexo feminino. O tempo médio de complementação de selênio, em fórmula especial, foi de 122,2±25,1 dias. A mistura de aminoácidos complementada com o mineral representou 72,9% da oferta diária de selênio. Após a complementação, as concentrações médias de selênio sérico e de glutationa peroxidase no eritrócito apresentaram aumento significativo (p < 0,05). A ingestão média diária de selênio aumentou significativamente (p < 0,001), alcançando o recomendado pela Ingestão Dietética de Referência. A concentração de tiroxina livre, no soro, apresentou redução significativa (p < 0,001) em tod...

Metabolic syndrome in children and adolescents with phenylketonuria

“…[21][22][23][24] É mais frequente o excesso de peso (e suas consequências). [21][22][23][24] É mais frequente o excesso de peso (e suas consequências).…”

Current Knowledge on the Importance of Selenium in Food for Living Organisms: A Review

“…Increasing deficiency of selenium in different parts of the world leads to the occurrence of many pathological disorders. Particularly vulnerable to the adverse effects of selenium deficiency are patients suffering from phenylketonuria [24] or individuals with diet-related diseases. In addition, individuals exposed to specialized chemotherapy and individuals who have already undergone radiotherapy are vulnerable to decreased levels of this microelement in the organism [25].…”

Supplementation of Micronutrient Selenium in Metabolic Diseases: Its Role as an Antioxidant

“…Using a special formula containing 31.5 μ g/d selenium and 98 mg/d L-carnitine reduced lipid peroxidation and protein oxidative damage and improved GPX activity in phenylketonuric patients, indicating that selenium supplementation was important for the amelioration of neurological symptoms of phenylketonuria via regulating oxidative stress pathways [ 110 ]. Alves observed in a clinical study of phenylketonuric children that selenium supplementation could significantly increase serum selenium and GPX in erythrocytes, which in turn reduced serum concentration of free thyroxin and improved patient conditions [ 111 ].…”