Metabolic syndrome in children and adolescents with phenylketonuria.

Objective: This study aimed to identify markers of metabolic syndrome (MS) in patients with phenylketonuria (PKU).

Methods: This was a cross-sectional study consisting of 58 PKU patients (ages of 4-15 years): 29 patients with excess weight, and 29 with normal weight. The biochemical variables assessed were phenylalanine (phe), total cholesterol, HDL-c, triglycerides, glucose, and basal insulin.

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

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.

Breastfeeding in the treatment of children with phenylketonuria.

Objective: To evaluate the effect of breastmilk as a source of phenylalanine (phe) on levels of this amino acid and on growth in phenylketonuric infants.

Methods: The study recruited 35 breastfed phenylketonuric infants and compared their results with those of 35 infants fed on commercial, milk-based formula. The groups were paired for sex and age at weaning from breastfeeding.