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Open AccessArticle

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

1
Dietetic Department, Birmingham Children’s Hospital, Steelhouse Lane, Birmingham B4 6NH, UK
2
Nutrition and Metabolism, NOVA Medical School, Faculdade de Ciências Médicas, Universidade Nova de Lisboa, 1169-056 Lisboa, Portugal
3
Centre for Health Technology and Services Research (CINTESIS), 4200-450 Porto, Portugal
*
Author to whom correspondence should be addressed.
Nutrients 2020, 12(10), 3153; https://doi.org/10.3390/nu12103153
Received: 19 September 2020 / Revised: 11 October 2020 / Accepted: 12 October 2020 / Published: 15 October 2020
(This article belongs to the Special Issue Diet Therapy and Nutritional Management of Phenylketonuria)
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. View Full-Text
Keywords: phenylketonuria; PKU; glycomacropeptide; special low protein foods; macronutrient intake; protein substitute phenylketonuria; PKU; glycomacropeptide; special low protein foods; macronutrient intake; protein substitute
MDPI and ACS Style

Daly, A.; Evans, S.; Pinto, A.; Ashmore, C.; Rocha, J.C.; MacDonald, A. 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 2020, 12, 3153. https://doi.org/10.3390/nu12103153

AMA Style

Daly A, Evans S, Pinto A, Ashmore C, Rocha JC, MacDonald A. 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. 2020; 12(10):3153. https://doi.org/10.3390/nu12103153

Chicago/Turabian Style

Daly, Anne; Evans, Sharon; Pinto, Alex; Ashmore, Catherine; Rocha, Júlio C.; MacDonald, Anita. 2020. "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 12, no. 10: 3153. https://doi.org/10.3390/nu12103153

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