The Experts below are selected from a list of 13134 Experts worldwide ranked by ideXlab platform
Anita Macdonald - One of the best experts on this subject based on the ideXlab platform.
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a 3 year longitudinal prospective review examining the dietary profile and contribution made by special Low Protein Foods to energy and macronutrient intake in children with phenylketonuria
Nutrients, 2020Co-Authors: A. Daly, S. Evans, Catherine Ashmore, Alex Pinto, J C Rocha, Anita MacdonaldAbstract:The nutritional composition of special Low Protein Foods (SLPFs) is controlled under EU legislation for ‘Foods for Special Medical Purposes (FSMP)’. They are designed to meet the energy needs of patients unable to eat a normal Protein containing diet. In phenylketonuria (PKU), the macronutrient contribution of SLPFs has been inadequately examined. Aim: A 3-year longitudinal prospective study investigating the contribution of SLPFs to the macronutrient intake of children with early treated PKU. Methods: 48 children (27 boys) with a mean recruitment age of 9.3 y were studied. Semi-quantitative dietary assessments and food frequency questionnaires (FFQ) were collected three to four times/year for 3 years. Results: The mean energy intake provided by SLPFs was 33% (SD ± 8), and this figure was 42% (SD ± 13) for normal food and 21% (SD ± 5) for Protein substitutes (PS). SLPFs supplied a mean intake of 40% carbohydrate (SD ± 10), 51% starch (SD ± 18), 21% sugar (SD ± 8), and 38% fat (SD ± 13). Fibre intake met 83% of the Scientific Advisory Committee on Nutrition (SACN) reference value, with 50% coming from SLPFs with added gums and hydrocolloids. Low Protein bread, pasta and milk provided the highest energy contribution, and the intake of sweet SLPFs (e.g., biscuits, cakes, and chocolate) was minimal. Children averaged three portions fruit/vegetable daily, and children aged ≥ 12 y had irregular meal patterns. Conclusion: SLPFs provide essential energy in phenylalanine restricted diets. Optimising the nutritional quality of SLPFs deserves more attention.
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how does feeding development and progression onto solid Foods in pku compare with non pku children during weaning
Nutrients, 2019Co-Authors: S. Evans, A. Daly, J Wildgoose, Barbara Cochrane, S Chahal, Catherine Ashmore, Nik Loveridge, Anita MacdonaldAbstract:Weaning is complex for children with phenylketonuria (PKU). Breastmilk/infant formula and phenylalanine (Phe)-free infant Protein-substitute (PS) are gradually replaced with equivalent amounts of Phe-containing food, a semi-solid/spoonable weaning PS and special Low-Protein Foods. In PKU, feeding patterns/practices during weaning in PKU have not been formally evaluated. In this longitudinal, prospective, case-control study (n = 20) infants with PKU transitioning to a second-stage PS, were recruited at weaning (4–6 months) for a comparison of feeding practices and development with non-PKU infants. Subjects were monitored monthly to 12 months and at age 15 months, 18 months and 24 months for: feeding progression; food textures; motor skill development and self-feeding; feeding environment; gastrointestinal symptoms; and negative feeding behaviours. Children with PKU had comparable weaning progression to non-PKU infants including texture acceptance, infant formula volume and self-feeding skills. However, children with PKU had more prolonged Phe-free infant formula bottle-feeding and parental spoon feeding than controls; fewer meals/snacks per day; and experienced more flatulence (p = 0.0005), burping (p = 0.001), retching (p = 0.03); and less regurgitation (p = 0.003). Negative behaviours associated with PS at age 10–18 months, coincided with the age of teething. Use of semi-solid PS in PKU supports normal weaning development/progression but parents require support to manage the complexity of feeding and to normalise the social inclusivity of their child’s family food environment. Further study regarding parental anxiety associated with mealtimes is required.
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Special Low Protein Foods for phenylketonuria: availability in Europe and an examination of their nutritional profile
Orphanet Journal of Rare Diseases, 2015Co-Authors: Maria João Pena, Manuela Ferreira Almeida, Esther Van Dam, Katharina Dokoupil, Hulya Gokmen-ozel, Anna Maria Lammardo, Kirsten Ahring, Amaya Belanger-quintana, Anita Macdonald, Martine RobertAbstract:BACKGROUND: Special Low Protein Foods (SLPF) are essential in the nutritional management of patients with phenylketonuria (PKU). The study objectives were to: 1) identify the number of SLPF available for use in eight European countries and Turkey and 2) analyse the nutritional composition of SLPF available in one of these countries.\n\nMETHODS: European Nutritionist Expert Panel on PKU (ENEP) members (Portugal, Spain, Belgium, Italy, Germany, Netherlands, UK, Denmark and Turkey) provided data on SPLF available in each country. The nutritional composition of Portuguese SLPF was compared with regular food products.\n\nRESULTS: The number of different SLPF available in each country varied widely with a median of 107 [ranging from 73 (Portugal) and 256 (Italy)]. Food analysis of SLPF available from a single country (Portugal) indicated that the mean phenylalanine content was higher in Low Protein baby cereals (mean 48 mg/100 g) and chocolate/energy bars/jelly (mean 41 mg/100 g). The energy content of different Foods from a sub-group of SLPF (cookies) varied widely between 23 and 96 kcal/cookie. Low Protein bread had a high fat content [mean 5.8 g/100 g (range 3.7 to 10)] compared with 1.6 g/100 g in regular bread. Seven of the 12 SLPF sub-groups (58 %) did not declare any vitamin content, and only 4 (33 %) identified a limited number of minerals.\n\nCONCLUSIONS: Whilst equal and free access to all SLPF is desirable, the widely variable nutritional composition requires careful nutritional knowledge of all products when prescribed for individual patients with PKU. There is a need for more specific nutritional standards for special Low Protein Foods.
Dalia Malkova - One of the best experts on this subject based on the ideXlab platform.
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impact of phenylketonuria type meal on appetite thermic effect of feeding and postprandial fat oxidation
Clinical Nutrition, 2017Co-Authors: Hani Alfheeaid, Barbara Cochrane, Konstantinos Gerasimidis, Anamaria Năstase, Mie Elhauge, Dalia MalkovaAbstract:Summary Background Dietary management of phenylketonuria (PKU) requires the replacement of natural Protein-containing Foods with special Low Protein Foods. The effect of a PKU type diet on factors contributing to energy balance requires investigation. Objective To investigate the impact of a PKU type meal on appetite ratings, gut appetite hormones, thermic effect of feeding (TEF) and fat oxidation. Methods Twenty-three healthy adults (mean ± SD age: 24.3 ± 5.1 years; BMI: 22.4 ± 2.5 kg/m 2 ) participated in a randomized, crossover design study. Each participant conducted two (PKU and Control) experimental trials which involved consumption of a PKU type meal and Protein substitute drink or an isocaloric and weight matched ordinary meal and Protein-enriched milk. Appetite, metabolic rate, fat oxidation measurements and blood collections were conducted for the duration of 300 min. On the completion of the measurements ad libitum buffet dinner was served. Results Responses of appetite ratings, plasma concentrations of GLP-1 and PYY ( P > 0.05, trial effect, two-way ANOVA) and energy intake during ad libitum buffet dinner ( P > 0.05, paired t -test) were not significantly different between the two trials. The TEF (PKU, 10.2 ± 1.5%; Control, 13.2 ± 1.0%) and the total amount of fat oxidized (PKU, 18.90 ± 1.10 g; Control, 22.10 ± 1.10 g) were significantly ( P t -tests) Lower in the PKU than in the Control trial. The differences in TEF and fat oxidation were significant ( P t -tests) for the post-meal period. Conclusions Consumption of a meal composed of special Low Protein Foods has no detrimental impact on appetite and appetite hormones but produces a Lower TEF and postprandial fat oxidation than an ordinary meal. These metabolic alterations may contribute to the increased prevalence of obesity reported in patients with PKU on contemporary dietary management. Clinical trial registration : The trial has been registered in ClinicalTrials as NCT02440932 .
A. Daly - One of the best experts on this subject based on the ideXlab platform.
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a 3 year longitudinal prospective review examining the dietary profile and contribution made by special Low Protein Foods to energy and macronutrient intake in children with phenylketonuria
Nutrients, 2020Co-Authors: A. Daly, S. Evans, Catherine Ashmore, Alex Pinto, J C Rocha, Anita MacdonaldAbstract:The nutritional composition of special Low Protein Foods (SLPFs) is controlled under EU legislation for ‘Foods for Special Medical Purposes (FSMP)’. They are designed to meet the energy needs of patients unable to eat a normal Protein containing diet. In phenylketonuria (PKU), the macronutrient contribution of SLPFs has been inadequately examined. Aim: A 3-year longitudinal prospective study investigating the contribution of SLPFs to the macronutrient intake of children with early treated PKU. Methods: 48 children (27 boys) with a mean recruitment age of 9.3 y were studied. Semi-quantitative dietary assessments and food frequency questionnaires (FFQ) were collected three to four times/year for 3 years. Results: The mean energy intake provided by SLPFs was 33% (SD ± 8), and this figure was 42% (SD ± 13) for normal food and 21% (SD ± 5) for Protein substitutes (PS). SLPFs supplied a mean intake of 40% carbohydrate (SD ± 10), 51% starch (SD ± 18), 21% sugar (SD ± 8), and 38% fat (SD ± 13). Fibre intake met 83% of the Scientific Advisory Committee on Nutrition (SACN) reference value, with 50% coming from SLPFs with added gums and hydrocolloids. Low Protein bread, pasta and milk provided the highest energy contribution, and the intake of sweet SLPFs (e.g., biscuits, cakes, and chocolate) was minimal. Children averaged three portions fruit/vegetable daily, and children aged ≥ 12 y had irregular meal patterns. Conclusion: SLPFs provide essential energy in phenylalanine restricted diets. Optimising the nutritional quality of SLPFs deserves more attention.
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how does feeding development and progression onto solid Foods in pku compare with non pku children during weaning
Nutrients, 2019Co-Authors: S. Evans, A. Daly, J Wildgoose, Barbara Cochrane, S Chahal, Catherine Ashmore, Nik Loveridge, Anita MacdonaldAbstract:Weaning is complex for children with phenylketonuria (PKU). Breastmilk/infant formula and phenylalanine (Phe)-free infant Protein-substitute (PS) are gradually replaced with equivalent amounts of Phe-containing food, a semi-solid/spoonable weaning PS and special Low-Protein Foods. In PKU, feeding patterns/practices during weaning in PKU have not been formally evaluated. In this longitudinal, prospective, case-control study (n = 20) infants with PKU transitioning to a second-stage PS, were recruited at weaning (4–6 months) for a comparison of feeding practices and development with non-PKU infants. Subjects were monitored monthly to 12 months and at age 15 months, 18 months and 24 months for: feeding progression; food textures; motor skill development and self-feeding; feeding environment; gastrointestinal symptoms; and negative feeding behaviours. Children with PKU had comparable weaning progression to non-PKU infants including texture acceptance, infant formula volume and self-feeding skills. However, children with PKU had more prolonged Phe-free infant formula bottle-feeding and parental spoon feeding than controls; fewer meals/snacks per day; and experienced more flatulence (p = 0.0005), burping (p = 0.001), retching (p = 0.03); and less regurgitation (p = 0.003). Negative behaviours associated with PS at age 10–18 months, coincided with the age of teething. Use of semi-solid PS in PKU supports normal weaning development/progression but parents require support to manage the complexity of feeding and to normalise the social inclusivity of their child’s family food environment. Further study regarding parental anxiety associated with mealtimes is required.
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Home delivery service of Low Protein Foods in inherited metabolic disorders: Does it help?
'Elsevier BV', 2019Co-Authors: A. Macdonald, A. Pinto, S. Evans, C. Ashmore, J. Macdonald, A. DalyAbstract:Background: In the UK, the customary method of obtaining special Low Protein (LP) Foods was by dispensing through a pharmacist (until 2010) for patients with inherited metabolic disorders (IMD) requiring LP diets. Recently, different home delivery services have been introduced to support patient access of Low Protein Foods, but the effectiveness of these services is unclear. Aim: A prospective, longitudinal, observational study to examine the effectiveness and safety of patient home delivery services for LP Foods over 12 months in IMD patients requiring a LP diet. Methods: IMD patients/caregivers had the choice of 2 home delivery services (Homeward® and Vitaflo at Home®) as well as access to primary care pharmacy services. Both home delivery services provided a limited range of LP Foods. Over a 12-month period, a member of the IMD dietetic team conducted 4 home visits to IMD patients on LP diets using home delivery services for Low Protein Foods. At each visit, caregivers completed a questionnaire consisting of 20 multiple choice and open questions about their prescription experience with special LP Foods. The researchers also completed stock checks, assessed ‘use by dates’ and adequacy of home storage for LP Foods. Results: In total, 58 patients participated in this study. Over 12 months, 95% (n = 55/58) of caregivers used their local pharmacy, 93% (n = 54/58) Homeward® and 78% (n = 45/58) Vitaflo at Home® to access LP Foods. Two home delivery services were used by 41 (71%) caregivers and the remaining 17 (29%) only used one of the home delivery service companies. Each patient only stored a median of 6 (range 0–22) different LP Foods at home. Overall, 45% (n = 26/58) of caregivers reported problems with their GP prescriptions. 30% (n = 16/53) of caregivers received at least one incorrect prescription when using their pharmacy (e.g. gluten-free Foods instead of LP, incorrect product or incorrect product amount), 6% errors (n = 3/53) with Homeward® and 2% (n = 1/48) with Vitaflo at Home®. 49% (n = 26/53) of caregivers said they experienced delayed receipt of LP Foods from their pharmacy, compared with 11% (n = 6/55) from Homeward® and 8% (n = 4/48) Vitaflo at Home®. Conclusions: Although home delivery services for special LP Foods are associated with less errors and delay compared with pharmacies, inaccuracies and inefficiencies still occur and the overall system is complex. We suggest a new, simpler, less fragmented system whereby metabolic dietitians prescribe LP Foods. This is likely to result in less burden on NHS resources and ensure a better treatment delivered to IMD patients. Keywords: Low Protein Foods, Home delivery service, Prescriptions, IM
S. Evans - One of the best experts on this subject based on the ideXlab platform.
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a 3 year longitudinal prospective review examining the dietary profile and contribution made by special Low Protein Foods to energy and macronutrient intake in children with phenylketonuria
Nutrients, 2020Co-Authors: A. Daly, S. Evans, Catherine Ashmore, Alex Pinto, J C Rocha, Anita MacdonaldAbstract:The nutritional composition of special Low Protein Foods (SLPFs) is controlled under EU legislation for ‘Foods for Special Medical Purposes (FSMP)’. They are designed to meet the energy needs of patients unable to eat a normal Protein containing diet. In phenylketonuria (PKU), the macronutrient contribution of SLPFs has been inadequately examined. Aim: A 3-year longitudinal prospective study investigating the contribution of SLPFs to the macronutrient intake of children with early treated PKU. Methods: 48 children (27 boys) with a mean recruitment age of 9.3 y were studied. Semi-quantitative dietary assessments and food frequency questionnaires (FFQ) were collected three to four times/year for 3 years. Results: The mean energy intake provided by SLPFs was 33% (SD ± 8), and this figure was 42% (SD ± 13) for normal food and 21% (SD ± 5) for Protein substitutes (PS). SLPFs supplied a mean intake of 40% carbohydrate (SD ± 10), 51% starch (SD ± 18), 21% sugar (SD ± 8), and 38% fat (SD ± 13). Fibre intake met 83% of the Scientific Advisory Committee on Nutrition (SACN) reference value, with 50% coming from SLPFs with added gums and hydrocolloids. Low Protein bread, pasta and milk provided the highest energy contribution, and the intake of sweet SLPFs (e.g., biscuits, cakes, and chocolate) was minimal. Children averaged three portions fruit/vegetable daily, and children aged ≥ 12 y had irregular meal patterns. Conclusion: SLPFs provide essential energy in phenylalanine restricted diets. Optimising the nutritional quality of SLPFs deserves more attention.
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how does feeding development and progression onto solid Foods in pku compare with non pku children during weaning
Nutrients, 2019Co-Authors: S. Evans, A. Daly, J Wildgoose, Barbara Cochrane, S Chahal, Catherine Ashmore, Nik Loveridge, Anita MacdonaldAbstract:Weaning is complex for children with phenylketonuria (PKU). Breastmilk/infant formula and phenylalanine (Phe)-free infant Protein-substitute (PS) are gradually replaced with equivalent amounts of Phe-containing food, a semi-solid/spoonable weaning PS and special Low-Protein Foods. In PKU, feeding patterns/practices during weaning in PKU have not been formally evaluated. In this longitudinal, prospective, case-control study (n = 20) infants with PKU transitioning to a second-stage PS, were recruited at weaning (4–6 months) for a comparison of feeding practices and development with non-PKU infants. Subjects were monitored monthly to 12 months and at age 15 months, 18 months and 24 months for: feeding progression; food textures; motor skill development and self-feeding; feeding environment; gastrointestinal symptoms; and negative feeding behaviours. Children with PKU had comparable weaning progression to non-PKU infants including texture acceptance, infant formula volume and self-feeding skills. However, children with PKU had more prolonged Phe-free infant formula bottle-feeding and parental spoon feeding than controls; fewer meals/snacks per day; and experienced more flatulence (p = 0.0005), burping (p = 0.001), retching (p = 0.03); and less regurgitation (p = 0.003). Negative behaviours associated with PS at age 10–18 months, coincided with the age of teething. Use of semi-solid PS in PKU supports normal weaning development/progression but parents require support to manage the complexity of feeding and to normalise the social inclusivity of their child’s family food environment. Further study regarding parental anxiety associated with mealtimes is required.
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Home delivery service of Low Protein Foods in inherited metabolic disorders: Does it help?
'Elsevier BV', 2019Co-Authors: A. Macdonald, A. Pinto, S. Evans, C. Ashmore, J. Macdonald, A. DalyAbstract:Background: In the UK, the customary method of obtaining special Low Protein (LP) Foods was by dispensing through a pharmacist (until 2010) for patients with inherited metabolic disorders (IMD) requiring LP diets. Recently, different home delivery services have been introduced to support patient access of Low Protein Foods, but the effectiveness of these services is unclear. Aim: A prospective, longitudinal, observational study to examine the effectiveness and safety of patient home delivery services for LP Foods over 12 months in IMD patients requiring a LP diet. Methods: IMD patients/caregivers had the choice of 2 home delivery services (Homeward® and Vitaflo at Home®) as well as access to primary care pharmacy services. Both home delivery services provided a limited range of LP Foods. Over a 12-month period, a member of the IMD dietetic team conducted 4 home visits to IMD patients on LP diets using home delivery services for Low Protein Foods. At each visit, caregivers completed a questionnaire consisting of 20 multiple choice and open questions about their prescription experience with special LP Foods. The researchers also completed stock checks, assessed ‘use by dates’ and adequacy of home storage for LP Foods. Results: In total, 58 patients participated in this study. Over 12 months, 95% (n = 55/58) of caregivers used their local pharmacy, 93% (n = 54/58) Homeward® and 78% (n = 45/58) Vitaflo at Home® to access LP Foods. Two home delivery services were used by 41 (71%) caregivers and the remaining 17 (29%) only used one of the home delivery service companies. Each patient only stored a median of 6 (range 0–22) different LP Foods at home. Overall, 45% (n = 26/58) of caregivers reported problems with their GP prescriptions. 30% (n = 16/53) of caregivers received at least one incorrect prescription when using their pharmacy (e.g. gluten-free Foods instead of LP, incorrect product or incorrect product amount), 6% errors (n = 3/53) with Homeward® and 2% (n = 1/48) with Vitaflo at Home®. 49% (n = 26/53) of caregivers said they experienced delayed receipt of LP Foods from their pharmacy, compared with 11% (n = 6/55) from Homeward® and 8% (n = 4/48) Vitaflo at Home®. Conclusions: Although home delivery services for special LP Foods are associated with less errors and delay compared with pharmacies, inaccuracies and inefficiencies still occur and the overall system is complex. We suggest a new, simpler, less fragmented system whereby metabolic dietitians prescribe LP Foods. This is likely to result in less burden on NHS resources and ensure a better treatment delivered to IMD patients. Keywords: Low Protein Foods, Home delivery service, Prescriptions, IM
Barbara Cochrane - One of the best experts on this subject based on the ideXlab platform.
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how does feeding development and progression onto solid Foods in pku compare with non pku children during weaning
Nutrients, 2019Co-Authors: S. Evans, A. Daly, J Wildgoose, Barbara Cochrane, S Chahal, Catherine Ashmore, Nik Loveridge, Anita MacdonaldAbstract:Weaning is complex for children with phenylketonuria (PKU). Breastmilk/infant formula and phenylalanine (Phe)-free infant Protein-substitute (PS) are gradually replaced with equivalent amounts of Phe-containing food, a semi-solid/spoonable weaning PS and special Low-Protein Foods. In PKU, feeding patterns/practices during weaning in PKU have not been formally evaluated. In this longitudinal, prospective, case-control study (n = 20) infants with PKU transitioning to a second-stage PS, were recruited at weaning (4–6 months) for a comparison of feeding practices and development with non-PKU infants. Subjects were monitored monthly to 12 months and at age 15 months, 18 months and 24 months for: feeding progression; food textures; motor skill development and self-feeding; feeding environment; gastrointestinal symptoms; and negative feeding behaviours. Children with PKU had comparable weaning progression to non-PKU infants including texture acceptance, infant formula volume and self-feeding skills. However, children with PKU had more prolonged Phe-free infant formula bottle-feeding and parental spoon feeding than controls; fewer meals/snacks per day; and experienced more flatulence (p = 0.0005), burping (p = 0.001), retching (p = 0.03); and less regurgitation (p = 0.003). Negative behaviours associated with PS at age 10–18 months, coincided with the age of teething. Use of semi-solid PS in PKU supports normal weaning development/progression but parents require support to manage the complexity of feeding and to normalise the social inclusivity of their child’s family food environment. Further study regarding parental anxiety associated with mealtimes is required.
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impact of phenylketonuria type meal on appetite thermic effect of feeding and postprandial fat oxidation
Clinical Nutrition, 2017Co-Authors: Hani Alfheeaid, Barbara Cochrane, Konstantinos Gerasimidis, Anamaria Năstase, Mie Elhauge, Dalia MalkovaAbstract:Summary Background Dietary management of phenylketonuria (PKU) requires the replacement of natural Protein-containing Foods with special Low Protein Foods. The effect of a PKU type diet on factors contributing to energy balance requires investigation. Objective To investigate the impact of a PKU type meal on appetite ratings, gut appetite hormones, thermic effect of feeding (TEF) and fat oxidation. Methods Twenty-three healthy adults (mean ± SD age: 24.3 ± 5.1 years; BMI: 22.4 ± 2.5 kg/m 2 ) participated in a randomized, crossover design study. Each participant conducted two (PKU and Control) experimental trials which involved consumption of a PKU type meal and Protein substitute drink or an isocaloric and weight matched ordinary meal and Protein-enriched milk. Appetite, metabolic rate, fat oxidation measurements and blood collections were conducted for the duration of 300 min. On the completion of the measurements ad libitum buffet dinner was served. Results Responses of appetite ratings, plasma concentrations of GLP-1 and PYY ( P > 0.05, trial effect, two-way ANOVA) and energy intake during ad libitum buffet dinner ( P > 0.05, paired t -test) were not significantly different between the two trials. The TEF (PKU, 10.2 ± 1.5%; Control, 13.2 ± 1.0%) and the total amount of fat oxidized (PKU, 18.90 ± 1.10 g; Control, 22.10 ± 1.10 g) were significantly ( P t -tests) Lower in the PKU than in the Control trial. The differences in TEF and fat oxidation were significant ( P t -tests) for the post-meal period. Conclusions Consumption of a meal composed of special Low Protein Foods has no detrimental impact on appetite and appetite hormones but produces a Lower TEF and postprandial fat oxidation than an ordinary meal. These metabolic alterations may contribute to the increased prevalence of obesity reported in patients with PKU on contemporary dietary management. Clinical trial registration : The trial has been registered in ClinicalTrials as NCT02440932 .
