Browsing by Author "Evans, S"
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- Early Feeding Practices in Infants with Phenylketonuria Across EuropePublication . 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; Bernabei, SM; Bontemps, C; Boyle, F; Bruni, G; Bueno-Delgado, M; Caine, G; Carvalho, R; Chrobot, A; Chyż, K; Cochrane, B; Correia, C; Corthouts, K; Daly, A; De Leo, S; Desloovere, A; De Meyer, A; De Theux, A; Didycz, B; Dijsselhof, ME; Dokoupil, K; Drabik, J; Dunlop, C; Eberle-Pelloth, W; Eftring, K; Ekengren, J; Errekalde, I; Evans, S; Foucart, A; Fokkema, L; François, L; French, M; Forssell, E; Gingell, C; Gonçalves, C; Gökmen Özel, H; Grimsley, A; Gugelmo, G; Gyüre, E; Heller, C; Hensler, R; Jardim, I; Joost, C; Jörg-Streller, M; Jouault, C; Jung, A; Kanthe, M; Koç, N; Kok, I L; Kozanoğlu, T; Kumru, B; Lang, F; Lang, K; Liegeois, I; Liguori, A; Lilje, R; Ļubina, O; Manta-Vogli, P; Mayr, D; Meneses, C; Newby, C; Meyer, U; Mexia, S; Nicol, C; Och, U; Olivas, SM; Pedrón-Giner, C; Pereira, R; Plutowska-Hoffmann, K; Purves, J; Re Dionigi, A; Reinson, K; Robert, M; Robertson, L; Rocha, JC; Rohde, C; Rosenbaum-Fabian, S; Rossi, A; Ruiz, M; Saligova, J; Gutiérrez-Sánchez, A; Schlune, A; Schulpis, K; Serrano-Nieto, J; Skarpalezou, A; Skeath, R; Slabbert, A; Straczek, K; Giżewska, M; Terry, A; Thom, R; Tooke, A; Tuokkola, J; van Dam, E; van den Hurk, TM; van der Ploeg, EC; Vande Kerckhove, K; Van Driessche, M; van Wegberg, AJ; van Wyk, K; Vasconcelos, C; Velez García, V; Wildgoose, J; Winkler, T; Żółkowska, J; Zuvadelli, J; MacDonald, 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.
- Natural Protein Intake in Children with Phenylketonuria: Prescription vs. Actual IntakesPublication . 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 the difference between the prescribed natural protein intake and their actual consumed intake, and to calculate the natural protein/Phe intake from foods given without measurement or restriction. Over a 6-month duration, three one-day diet diaries were collected every month by caregivers of children with PKU at the beginning of a follow-up study. Dietary intakes of Phe, as well as natural and total protein intakes, were calculated using Nutritics® (v5.09). Weekly blood Phe spots were collected by caregivers. The target blood Phe level was ≤360 μmol/L for ages up to 12 years and ≤600 μmol/L for ages ≥12 years. Sixteen early treated children (69% females) with PKU were recruited. The median age was 11 years (range: 9-13), and most had classical PKU (n = 14/16). A median of 18 (range 12-18) one-day diaries and 22 blood spots were analysed for each subject over 6 months. The median prescribed natural protein was 6 g/day (range: 3-27), but when calculated, the actual median intake from all foods consumed was 10 g/day (range: 4-37). The median prescribed Phe was 300 mg/day (range: 150-1350), but the actual median intake was 500 mg/day (range: 200-1850). The median difference between the prescribed and actual natural protein daily intakes was +4 g/day (range: -2.5 to +11.5), with a median percentage increase of 40% for natural protein/Phe intake (p < 0.001). The median blood Phe level was 250 μmol/L (range 20-750), with 91% of blood Phe levels within the target range. Only one patient (11 years) had less than 75% of their blood Phe levels within the target range. The UK Phe exchange system provides flexibility in the dietary management of PKU. With this method, the actual natural protein intake was 167% higher than the prescribed amount. Although this led to a variable daily protein intake, the majority of children (n = 15/16) experienced no deterioration in their metabolic control.
- Suitability and Allocation of Protein-Containing Foods According to Protein Tolerance in PKU: A 2022 UK National ConsensusPublication . 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; Firman, S; Ford, S; Freedman, F; French, M; Gaff, L; Gribben, J; Grimsley, A; Herlihy, I; Hill, M; Khan, F; McStravick, N; Millington, C; Moran, N; Newby, C; Nguyen, P; Purves, J; Pinto, A; César Rocha, J; Skeath, R; Skelton, A; Tapley, S; Woodall, A; Young, C; MacDonald, AIntroduction: 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 tolerate >10 g/day of protein. Methods: In January 2022, a 26-item food group questionnaire, listing a range of foods containing protein from 5 to >20 g/100 g, was sent to all British Inherited Metabolic Disease Group (BIMDG) dietitians (n = 80; 26 Inherited Metabolic Disease [IMD] centres). They were asked to consider within their IMD dietetic team when they would recommend introducing each of the 26 protein-containing food groups into a patient’s diet who tolerated >10 g to 60 g/day of protein. The patient protein tolerance for each food group that received the majority vote from IMD dietetic teams was chosen as its tolerance threshold for introduction. A virtual meeting was held using Delphi methodology in March 2022 to discuss and agree final consensus. Results: Responses were received from dietitians from 22/26 IMD centres (85%) (11 paediatric, 11 adult). For patients tolerating protein ≥15 g/day, the following foods were agreed for inclusion: gluten-free pastas, gluten-free flours, regular bread, cheese spreads, soft cheese, and lentils in brine; for protein tolerance ≥20 g/day: nuts, hard cheeses, regular flours, meat/fish, and plant-based alternative products (containing 5−10 g/100 g protein), regular pasta, seeds, eggs, dried legumes, and yeast extract spreads were added; for protein tolerance ≥30 g/day: meat/fish and plant-based alternative products (containing >10−20 g/100 g protein) were added; and for protein tolerance ≥40 g/day: meat/fish and plant-based alternatives (containing >20 g/100 g protein) were added. Conclusion: This UK consensus by IMD dietitians from 22 UK centres describes for the first time the suitability and allocation of higher protein foods according to individual patient protein tolerance. It provides valuable guidance for health professionals to enable them to standardize practice and give rational advice to patients.
- Weaning Practices in Phenylketonuria Vary Between Health Professionals in EuropePublication . 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; Bernabei, S M; Bontemps, C; Boyle, F; Bruni, G; Bueno-Delgado, M; Caine, G; Carvalho, R; Chrobot, A; Chyż, K; Cochrane, B; Correia, C; Corthouts, K; Daly, A; De Leo, S; Desloovere, A; De Meyer, A; De Theux, A; Didycz, B; Dijsselhof, M E; Dokoupil, K; Drabik, J; Dunlop, C; Eberle-Pelloth, W; Eftring, K; Ekengren, J; Errekalde, I; Evans, S; Foucart, A; Fokkema, L; François, L; French, M; Forssell, E; Gingell, C; Gonçalves, C; Gökmen Özel, H; Grimsley, A; Gugelmo, G; Gyüre, E; Heller, C; Hensler, R; Jardim, I; Joost, C; Jörg-Streller, M; Jouault, C; Jung, A; Kanthe, M; Koç, N; Kok, I L; Kozanoğlu, T; Kumru, B; Lang, F; Lang, K; Liegeois, I; Liguori, A; Lilje, R; Ļubina, O; Manta-Vogli, P; Mayr, D; Meneses, C; Newby, C; Meyer, U; Mexia, S; Nicol, C; Och, U; Olivas, S M; Pedrón-Giner, C; Pereira, R; Plutowska-Hoffmann, K; Purves, J; Re Dionigi, A; Reinson, K; Robert, M; Robertson, L; Rocha, J C; Rohde, C; Rosenbaum-Fabian, S; Rossi, A; Ruiz, M; Saligova, J; Gutiérrez-Sánchez, A; Schlune, A; Schulpis, K; Serrano-Nieto, J; Skarpalezou, A; Skeath, R; Slabbert, A; Straczek, K; Giżewska, M; Terry, A; Thom, R; Tooke, A; Tuokkola, J; van Dam, E; van den Hurk, T A M; van der Ploeg, E M C; Vande Kerckhove, K; Van Driessche, M; van Wegberg, A M J; van Wyk, K; Vasconcelos, C; Velez García, V; Wildgoose, J; Winkler, T; Żółkowska, J; Zuvadelli, J; MacDonald, 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. Our aim was to assess different weaning approaches used by health professionals across Europe.
- Weaning Practices in Phenylketonuria Vary Between Health Professionals in EuropePublication . 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; Bernabei, SM; Bontemps, C; Boyle, F; Bruni, G; Bueno-Delgado, M; Caine, G; Carvalho, R; Chrobot, A; Chyż, K; Cochrane, B; Correia, C; Corthouts, K; Daly, A; De Leo, S; Desloovere, A; De Meyer, A; De Theux, A; Didycz, B; Dijsselhof, ME; Dokoupil, K; Drabik, J; Dunlop, C; Eberle-Pelloth, W; Eftring, K; Ekengren, J; Errekalde, I; Evans, S; Foucart, A; Fokkema, L; François, L; French, M; Forssell, E; Gingell, C; Gonçalves, C; Gökmen Özel, H; Grimsley, A; Gugelmo, G; Gyüre, E; Heller, C; Hensler, R; Jardim, I; Joost, C; Jörg-Streller, M; Jouault, C; Jung, A; Kanthe, M; Koç, N; Kok, I L; Kozanoğlu, T; Kumru, B; Lang, F; Lang, K; Liegeois, I; Liguori, A; Lilje, R; Ļubina, O; Manta-Vogli, P; Mayr, D; Meneses, C; Newby, C; Meyer, U; Mexia, S; Nicol, C; Och, U; Olivas, SM; Pedrón-Giner, C; Pereira, R; Plutowska-Hoffmann, K; Purves, J; Re Dionigi, A; Reinson, K; Robert, M; Robertson, L; Rocha, JC; Rohde, C; Rosenbaum-Fabian, S; Rossi, A; Ruiz, M; Saligova, J; Gutiérrez-Sánchez, A; Schlune, A; Schulpis, K; Serrano-Nieto, J; Skarpalezou, A; Skeath, R; Slabbert, A; Straczek, K; Giżewska, M; Terry, A; Thom, R; Tooke, A; Tuokkola, J; van Dam, E; van den Hurk, TAM; van der Ploeg, E C; Vande Kerckhove, K; Van Driessche, M; van Wegberg, AMJ; van Wyk, K; Vasconcelos, C; Velez García, V; Wildgoose, J; Winkler, T; Żółkowska, J; Zuvadelli, J; MacDonald, 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. Our aim was to assess different weaning approaches used by health professionals across Europe.