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The Effect of Glycomacropeptide versus Amino Acids on Phenylalanine and Tyrosin...

Daly, Anne; Evans, Sharon; Chahal, Satnam; Santra, Saikat; Pinto, Alex; Gingell, Cerys; Rocha, Júlio César; van Spronsen, Francjan; Jackson, Richard

Introduction: In phenylketonuria (PKU), evidence suggests that casein glycomacropeptide supplemented with rate-limiting amino acids (CGMP-AA) is associated with better protein utilisation and less blood phenylalanine (Phe) variability. Aim: To study the impact of CGMP-AA on blood Phe variability using 3 different dietary regimens in children with PKU. Methods: This was a 6-week randomised controlled cross-over ...


Over Restriction of Dietary Protein Allowance: The Importance of Ongoing Reasse...

Pinto, Alex; Almeida, Manuela Ferreira; MacDonald, Anita; Ramos, Paula Cristina; Rocha, Sara; Guimas, Arlindo; Ribeiro, Rosa; Martins, Esmeralda

Phenylalanine (Phe) tolerance is highly variable in phenylketonuria (PKU) and rarely described in patients aged ≥12 years. Patients ≥12 years of age with PKU were systematically challenged with additional natural protein (NP) if blood Phe levels remained below 480 µmol/L (i.e., upper target blood Phe level for patients aged ≥12 years using Portuguese PKU guidelines). In PKU patients, NP tolerance was calculated...


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