Abstract
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.
Highlights
In phenylketonuria (PKU), deficiency or reduced activity of the phenylalanine hydroxylase enzyme (PAH) limits the conversion of phenylalanine to tyrosine
Nutrients 2020, 12, 3153 phenylalanine levels to within a strict target range [5]. Alternative treatments, such as sapropterin and pegvaliase (PEGylated recombinant Anabaena variabilis phenylalanine ammonia lyase (PAL)), have been licensed, they are only suitable for subsections of the PKU population, and access is restricted in some countries
Thereby, outcome in PKU is dependent on the early introduction of dietary treatment and the quality of lifelong blood phenylalanine control, which in turn is determined by the ability to adhere to dietary treatment
Summary
In phenylketonuria (PKU), deficiency or reduced activity of the phenylalanine hydroxylase enzyme (PAH) limits the conversion of phenylalanine to tyrosine. High levels of brain phenylalanine are probably the main cause of neurotoxicity [1] by interfering with cerebral protein synthesis [2], increasing myelin turnover and inhibiting neurotransmitter synthesis [3,4]. In children with classical PKU, their only effective management option is a severely restricted low protein/phenylalanine diet that aims to lower blood. Nutrients 2020, 12, 3153 phenylalanine levels to within a strict target range [5]. Alternative treatments, such as sapropterin and pegvaliase (PEGylated recombinant Anabaena variabilis phenylalanine ammonia lyase (PAL)), have been licensed, they are only suitable for subsections of the PKU population, and access is restricted in some countries. Thereby, outcome in PKU is dependent on the early introduction of dietary treatment and the quality of lifelong blood phenylalanine control, which in turn is determined by the ability to adhere to dietary treatment
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