Protein Labelling Accuracy for UK Patients with PKU Following a Low Protein Diet

Kraleva, Dilyana; Evans, Sharon; Pinto, Alex; Daly, Anne; Ashmore, Catherine; Pointon-Bell, Kiri; Rocha, Júlio César; MacDonald, Anita

doi:10.3390/nu12113440

Cited by 5 publications

(6 citation statements)

References 11 publications

(14 reference statements)

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“…Several respondents were frustrated that some potentially suitable instant dessert mixes and dried cereals had a protein content given on the food analysis after manufacturers had assumed they would be reconstituted/prepared with added cow’s milk or egg, rendering the products unsuitable for people with PKU; no data were provided about the protein content of the dry products as purchased. There were many examples of ice creams that gave protein content for volume (in millilitres) rather than weight, and prepackaged foods that only gave a protein content of <0.5 g/100 g. Some commented that it would make a ‘massive difference’ if food labelling was clearer as there would be more foods that could be consumed, that the ‘confusing protein labelling made it very hard when choosing suitable foods in the supermarket’, and ‘the problems of interpreting protein labelling will not help my son become independent.’ These issues were also identified by Kravela et al 2020, who examined the accuracy of protein analysis from supermarket websites [ 5 ].…”

Section: Discussionmentioning

confidence: 99%

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The Challenges and Dilemmas of Interpreting Protein Labelling of Prepackaged Foods Encountered by the PKU Community

et al. 2022

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Phenylketonuria (PKU) can lead to severe intellectual impairment unless a phenylalanine-restricted diet starts early in life. It requires expert user knowledge about the protein content of foods. The ability of adults or caregivers of children with PKU to calculate protein exchanges from food labels on manufactured foods and any difficulties they encounter in interpreting food labels has not been studied systematically. Individuals with PKU or their caregivers residing in the UK were invited to complete a cross-sectional online survey that collected both qualitative and quantitative data about their experience when calculating protein exchanges from the food labelling on prepackaged foods. Data was available from 246 questionnaire respondents (152 caregivers of patients with PKU aged <18 years, 57 patients with PKU aged ≥18 years or their caregivers (n = 28), and 9 teenagers with PKU). Thirty-one per cent (n = 76/246) found it difficult to interpret food protein exchanges from food labels. The respondents listed that the main issues with protein labelling were the non-specification of whether the protein content was for the cooked or uncooked weight (64%, n = 158/246); labels stating foods contained 0 g protein but then included protein sources in the list of ingredients (56%, n = 137/246); the protein content being given after a product was prepared with regular milk rather than the dry weight of the product (55%, n = 135/246); and the non-clarity of whether the protein content was for the weight of prepared or unprepared food (in addition to non-specification of cooked or uncooked weights on food labelling) (54%, n = 133/246). Over 90% (n = 222/246) of respondents had experienced problems with food labelling in the previous six months. Misleading or confusing protein labelling of manufactured foods was common. The food industry and legislators have a duty to provide accurate and clear protein food labelling to protect populations requiring low protein diets.

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Section: Discussionmentioning

confidence: 99%

“…The questionnaire was divided into 3 sections. Section 1 collected information on patient age, sex, type of supermarket they commonly shopped at, and ease of calculating protein exchanges from food analysis labels for known problems previously identified [ 5 ].…”

Section: Methodsmentioning

confidence: 99%

The Challenges and Dilemmas of Interpreting Protein Labelling of Prepackaged Foods Encountered by the PKU Community

et al. 2022

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“…Some protein substitutes such as CGMP-AA contain Phe which may complicate gaining acceptable blood Phe control in children [ 7 ]. Persistent consumer pressure from the food industry and busy working adult lives has led to increased dependence on processed foods which are commonly not low in Phe or may have unreliable protein labelling information [ 25 ]. In addition, societal efforts to reduce the sugar content of foods has also led to sugar replacement by artificial sweeteners such as aspartame, another unquantified source of Phe [ 26 ].…”

Section: Discussionmentioning

confidence: 99%

Metabolic Control of Patients with Phenylketonuria in a Portuguese Metabolic Centre Comparing Three Different Recommendations

et al. 2021

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Blood phenylalanine (Phe) is used as the primary marker to evaluate metabolic control. Our study aimed to describe the metabolic control of patients with phenylketonuria (PKU) comparing three different treatment recommendations (European guidelines/US guidelines/Portuguese consensus). This was a retrospective, observational, single centre study in patients with PKU collecting data on blood Phe levels from 2017. Nutritional intake data and sapropterin (BH4) prescription were collected at the last appointment of 2017. The final sample studied included 87 patients (48% females) [13 hyperphenylalaninemia; 47 mild PKU; 27 classical PKU] with a median age of 18 y (range: 1–36 y). The median number of blood Phe measurements for patients was 21 (range: 6–89). In patients aged < 12 y, the median blood Phe level was 300 μmol/L (range 168–480) and 474 μmol/L (range 156–1194) for patients ≥ 12 y. Overall, a median of 83% of blood Phe levels were within the European PKU guidelines target range. In patients aged ≥ 12 years, there was a higher median % of blood Phe levels within the European PKU guidelines target range (≥12 y: 84% vs. <12 y: 56%). In children < 12 y with classical PKU (n = 2), only 34% of blood Phe levels were within target range for all 3 guidelines and 49% with mild PKU (n = 11). Girls had better control than boys (89% vs. 66% median Phe levels within European Guidelines). Although it is clear that 50% or more patients were unable to achieve acceptable metabolic control on current treatment options, a globally agreed upper Phe target associated with optimal outcomes for age groups is necessary. More studies need to examine how clinics with dissimilar resources, different therapeutic Phe targets and frequency of monitoring relate to metabolic control.

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“…Consequently, incomplete information may lead to the miscalculation of natural protein intake. Previous studies have also shown a high rate (>50%) of protein-labelling errors (e.g., unclear, misleading, inaccurate, or incomplete information) affecting interpretation of protein content of packaged manufactured foods on supermarket websites [34]. Ninety per cent of patients with PKU or their caregivers reported that they experienced problems with food labelling e.g., difficulties with interpreting food protein exchanges from food labels or labels containing misleading or confusing information [35].…”

Section: Introductionmentioning

confidence: 99%

Natural Protein Intake in Children with Phenylketonuria: Prescription vs. Actual Intakes

Pinto,

Daly,

Rocha

et al. 2023

Nutrients

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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 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.

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Protein Labelling Accuracy for UK Patients with PKU Following a Low Protein Diet

Cited by 5 publications

References 11 publications

The Challenges and Dilemmas of Interpreting Protein Labelling of Prepackaged Foods Encountered by the PKU Community

The Challenges and Dilemmas of Interpreting Protein Labelling of Prepackaged Foods Encountered by the PKU Community

Metabolic Control of Patients with Phenylketonuria in a Portuguese Metabolic Centre Comparing Three Different Recommendations

Natural Protein Intake in Children with Phenylketonuria: Prescription vs. Actual Intakes

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