An Observational Study Evaluating the Introduction of a Prolonged-Release Protein Substitute to the Dietary Management of Children with Phenylketonuria

MacDonald, Anita; Ashmore, Catherine; Daly, Anne; Pinto, Alex; Evans, Sharon

doi:10.3390/nu12092686

Cited by 12 publications

(7 citation statements)

References 31 publications

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“…This new technology suggests a physiological absorption of amino acids similar to natural protein [ 73 , 74 ]. In a short-term observational study using prolonged amino acids in subjects with PKU, it was well tolerated, with fewer gastrointestinal symptoms and no change in blood phenylalanine concentrations [ 75 ].…”

Section: Choice Of Protein Substitutesmentioning

confidence: 99%

Protein Substitutes in PKU; Their Historical Evolution

et al. 2021

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Protein substitutes developed for phenylketonuria (PKU) are a synthetic source of protein commonly based on L-amino acids. They are essential in the treatment of phenylketonuria (PKU) and other amino acid disorders, allowing the antagonistic amino acid to be removed but with the safe provision of all other amino acids necessary for maintaining normal physiological function. They were first formulated by a chemist and used experimentally on a 2-year-old girl with PKU and their nutritional formulations and design have improved over time. Since 2008, a bioactive macropeptide has been used as a base for protein substitutes in PKU, with potential benefits of improved bone and gut health, nitrogen retention, and blood phenylalanine control. In 2018, animal studies showed that physiomimic technology coating the amino acids with a polymer allows a slow release of amino acids with an improved physiological profile. History has shown that in PKU, the protein substitute’s efficacy is determined by its nutritional profile, amino acid composition, dose, timing, distribution, and an adequate energy intake. Protein substitutes are often given little importance, yet their pharmacological actions and clinical benefit are pivotal when managing PKU.

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Section: Choice Of Protein Substitutesmentioning

confidence: 99%

Protein Substitutes in PKU; Their Historical Evolution

et al. 2021

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“…The prolonged-release protein substitute designed with an ethyl cellulose and arginate coating masking the bitter taste, smell and reducing the osmolality of free amino acids are suitable for children with PKU. Most subjects, mixed the test protein substitute with food or fruit juice, had reduced blood phenylalanine levels and improved phenylalanine/tyrosine ratio (MacDonald et al, 2020).…”

Section: Introductionmentioning

confidence: 99%

Preparation and Evaluation of Some Bakery Products Very Low in Protein for Phenylketonuria Patients

Kamel

Zahran

Afify

2020

Egyptian Journal of Agricultural Sciences

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Potato flours and cornstarchare used to produce biscuits and flat bread for phenylketonuria(PKU) patients. The effect of thickening agents xanthan gum, Guar gum, carrageenan gum and mixturesofguar gum and carrageenan gum onphysical properties, hardness andsensory properties of biscuit and flat bread, were studied. The obtained results showed that the protein content of raw materials ranged between 0.10 to 6.12%, while total carbohydrates ranged from 88.28to 99.48%. The highest physical properties values (volume, thickness, diameter, spread ratio and specific volume), were in biscuit with xanthan gum. The hardness of biscuits decreased by adding gums. The thickening agents increased the sensory properties values of biscuits (taste and texture), whileit didnotaffect both color and odor of all biscuit samples compared with the control. The biscuit samples with xanthan gum and mixture of gums had the high acceptance. The highest moisture content was in xanthan gum of flat bread samples. The thickening agents caused increase in all samples of flat bread hardness compared with the control. The organoleptic results of flat bread showed that xanthan gum,guar gum, carrageenan gum and mixture of gums were more suitable to produce flat bread compared with the control samples. Potato flour and cornstarch,with thickening agents, can produce biscuits and flat bread low in protein for phenylketonuriapatients.

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“… 4 In practice, this dietary regimen involves restricting protein intake from food and instead providing the majority (up to 80%) of protein intake from phenylalanine-free or low-phenylalanine protein substitutes, consisting of either L-amino acids (L-AA) or casein glycomacropeptide (CGMP), 5 6 and more recently, protein substitutes using prolonged-release amino acid technology. 7–9 …”

Section: Introductionmentioning

confidence: 99%

Protein status of people with phenylketonuria: a scoping review protocol

et al. 2021

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IntroductionPhenylketonuria (PKU) is a disorder of protein metabolism resulting in an accumulation of phenylalanine in the body. Dietary management consists of altering the sources of ingested protein to limit phenylalanine intake. Current dietary protein guidelines for PKU are based on limited scientific evidence, thus it remains unclear whether current practice leads to optimal protein status in people with PKU. To date, no attempt has been made to systematically evaluate the protein status of people with PKU, using a combination of validated anthropometric, biochemical and functional measurement tools. Furthermore, factors known to influence protein status in the general population warrant consideration when determining protein status in individuals with PKU, alongside factors unique to PKU such as the type of protein substitute consumed. Understanding the impact of these variables on protein status is crucial to developing a personalised approach to protein recommendations for optimising health and functional outcomes in people with PKU. Therefore, the aim of this scoping review is to examine existing evidence regarding the protein status of people with PKU, and to investigate the nutritional and lifestyle variables that influence protein status.Methods and analysisThis review will be guided by Arksey and O’Malley’s framework, along with guidance from Levac et al, Pawliuk et al and the Joanna Briggs Institute. The following databases will be searched: MEDLINE (Ovid), Embase, CENTRAL, Web of Science and Scopus, alongside grey literature. Identified literature will be assessed by two independent reviewers for inclusion. Descriptive numerical analysis will be performed and a narrative summary will accompany the tabulated results describing how study findings relate to the review questions.Ethics and disseminationThis review protocol does not require ethical approval. Findings will be disseminated through peer-reviewed publication, presented at relevant conferences, and shared with a patient research advisory group to inform discussions on future research.

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An Observational Study Evaluating the Introduction of a Prolonged-Release Protein Substitute to the Dietary Management of Children with Phenylketonuria

Cited by 12 publications

References 31 publications

Protein Substitutes in PKU; Their Historical Evolution

Protein Substitutes in PKU; Their Historical Evolution

Preparation and Evaluation of Some Bakery Products Very Low in Protein for Phenylketonuria Patients

Protein status of people with phenylketonuria: a scoping review protocol

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