9 documents found, page 1 of 1
Body Composition Evaluation and Clinical Markers of Cardiometabolic Risk in Pat...
Luengo-Pérez, LM; Fernández-Bueso, M; Ambrojo, A; Guijarro, M; Ferreira, AC; Pereira-da-Silva, L; Moreira-Rosário, A; Faria, A; Calhau, C; Daly, ACardiovascular diseases are the main cause of mortality worldwide. Patients with phenylketonuria (PKU) may be at increased cardiovascular risk. This review provides an overview of clinical and metabolic cardiovascular risk factors, explores the connections between body composition (including fat mass and ectopic fat) and cardiovascular risk, and examines various methods for evaluating body composition. It parti...
Natural Protein Intake in Children with Phenylketonuria: Prescription vs. Actua...
Pinto, A; Daly, A; César Rocha, J; Ashmore, C; Evans, S; Ilgaz, F; Hickson, M; MacDonald, AIn phenylketonuria (PKU), an important component of the UK dietary management system is a 50 mg phenylalanine (Phe)/1 g protein exchange system used to allocate the Phe/natural protein intakes according to individual patient tolerance. Any foods containing protein ≤ 0.5 g/100 g or fruits/vegetables containing Phe ≤ 75 mg/100 g are allowed without measurement or limit. In children with PKU, we aimed to assess th...
The Impact of the Quality of Nutrition and Lifestyle in the Reproductive Years ...
Gama, MI; Pinto, A; Daly, A; César Rocha, J; MacDonald, AA woman's nutritional status before and during pregnancy can affect the health of her progeny. Phenylketonuria (PKU), a rare disorder causing high blood and brain phenylalanine (Phe) concentrations, is associated with neurocognitive disability. Lifelong treatment is mainly dietetic with a Phe-restricted diet, supplemented with a low-Phe protein substitute. Treatment adherence commonly decreases in adolescence, ...
Suitability and Allocation of Protein-Containing Foods According to Protein Tol...
Gama, MI; Adam, S; Adams, S; Allen, H; Ashmore, C; Bailey, S; Cochrane, B; Dale, C; Daly, A; De Sousa, G; Donald, S; Dunlop, C; Ellerton, C; Evans, SIntroduction: There is little practical guidance about suitable food choices for higher natural protein tolerances in patients with phenylketonuria (PKU). This is particularly important to consider with the introduction of adjunct pharmaceutical treatments that may improve protein tolerance. Aim: To develop a set of guidelines for the introduction of higher protein foods into the diets of patients with PKU who ...
Glycomacropeptide: long-term use and impact on blood phenylalanine, growth and ...
Daly, A; Evans, S; Chahal, S; Santra, S; Pinto, A; Jackson, R; Gingell, C; Rocha, Júlio César; Van Spronsen, F J; MacDonald, AIn phenylketonuria, casein glycomacropeptide (CGMP) requires modification with the addition of some essential and semi essential amino acids to ensure suitability as a protein substitute. The optimal amount and ratio of additional amino acids is undefined. Aim: A longitudinal, parallel, controlled study over 12 months evaluating a CGMP (CGMP-AA2) formulation compared with phenylalanine-free L-amino acid supplem...
Weaning Practices in Phenylketonuria Vary Between Health Professionals in Europe
Pinto, A; Adams, S; Ahring, K; Allen, H; Almeida, MF; Garcia-Arenas, D; Arslan, N; Assoun, M; Atik Altınok, Y; Barrio-Carreras, D; Belanger Quintana, AIn phenylketonuria (PKU), weaning is considered more challenging when compared to feeding healthy infants. The primary aim of weaning is to gradually replace natural protein from breast milk or standard infant formula with solids containing equivalent phenylalanine (Phe). In addition, a Phe-free second stage L-amino acid supplement is usually recommended from around 6 months to replace Phe-free infant formula. ...
Weaning Practices in Phenylketonuria Vary Between Health Professionals in Europe
Pinto, A; Adams, S; Ahring, K; Allen, H; Almeida, M F; Garcia-Arenas, D; Arslan, N; Assoun, M; Atik Altınok, Y; Barrio-Carreras, D; Belanger Quintana, AIn phenylketonuria (PKU), weaning is considered more challenging when compared to feeding healthy infants. The primary aim of weaning is to gradually replace natural protein from breast milk or standard infant formula with solids containing equivalent phenylalanine (Phe). In addition, a Phe-free second stage L-amino acid supplement is usually recommended from around 6 months to replace Phe-free infant formula. ...
The Use of Glycomacropeptide in Patients with Phenylketonuria: A Systematic Rev...
Pena, MJ; Pinto, A; Daly, A; MacDonald, A; Azevedo L; rocha, jc; Borges, NunoIn phenylketonuria (PKU), synthetic protein derived from L-amino acids (AAs) is essential in a low-phenylalanine (Phe) diet. Glycomacropeptide (GMP), an intact protein, is very low in Phe in its native form. It has been modified and adapted for PKU to provide an alternative protein source through supplementation with rate-limiting amino acids (GMP-AAs), although it still contains residual Phe. This review aims ...
Early Feeding Practices in Infants with Phenylketonuria Across Europe
Pinto, A; Adams, S; Ahring, K; Allen, H; Almeida, MF; Garcia-Arenas, D; Arslan, N; Assoun, M; Atik Altınok, Y; Barrio-Carreras, D; Belanger Quintana, AIn infants with phenylketonuria (PKU), dietary management is based on lowering and titrating phenylalanine (Phe) intake from breast milk or standard infant formula in combination with a Phe-free infant formula in order to maintain blood Phe levels within target range. Professionals use different methods to feed infants with PKU and our survey aimed to document practices across Europe.