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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, A

In 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...


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, S

Introduction: 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, A

In 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, A

In 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, A

In 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. ...


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, A

In 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.


The European Federation of Organisations for Medical Physics Policy Statement N...

Christofides, S; Isidoro, J; Pesznyak, C; Cremers, F; Figueira, R; van Swol, C; Evans, S; Torresin, A

Continuing Professional Development (CPD) is vital to the medical physics profession if it is to embrace the pace of change occurring in medical practice. As CPD is the planned acquisition of knowledge, experience and skills required for professional practice throughout one's working life it promotes excellence and protects the profession and public against incompetence. Furthermore, CPD is a recommended prereq...


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