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Preliminary Data on Free Use of Fruits and Vegetables Containing Phenylalanine 76–100 mg/100 g of Food in 16 Children with Phenylketonuria

2023-07Artigo científico Acesso aberto
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dc.contributor.authorPinto, Alex
dc.contributor.authorDaly, Anne
dc.contributor.authorRocha, Júlio César
dc.contributor.authorAshmore, Catherine
dc.contributor.authorEvans, Sharon
dc.contributor.authorJackson, Richard
dc.contributor.authorHickson, Mary
dc.contributor.authorMacDonald, Anita
dc.contributor.institutionNOVA Medical School|Faculdade de Ciências Médicas (NMS|FCM)
dc.contributor.institutionCentro de Investigação em Tecnologias e Serviços de Saúde (CINTESIS)
dc.contributor.pblMDPI AG
dc.date.accessioned2023-07-24T22:16:08Z
dc.date.available2023-07-24T22:16:08Z
dc.date.issued2023-07
dc.descriptionFunding Information: A.P. received an educational grant from Cambrooke Therapeutics and grants from Vitaflo International, Nutricia, Merck Serono, Biomarin, Mevalia and Applied Pharma Research to attend scientific meetings. This project is also part of A.P.’s. PhD, which is funded by Vitaflo International. A.D. received research funding from Vitaflo International and financial support from Nutricia, Mevalia and Vitaflo International to attend study days and conferences. J.C.R. was a member of the European Nutritionist Expert Panel (Biomarin), the Advisory Board for Applied Pharma Research, Vitaflo, Synlogic, Biomarin and Nutricia and received honoraria as a speaker from APR, Merck Serono, Biomarin, Nutricia, Vitaflo, Cambrooke, PIAM and Lifediet. S.E. received research funding from Nutricia and financial support from Nutricia and Vitaflo International to attend study days and conferences. C.A. received honoraria from Nutricia and Vitaflo International to attend study days and conferences. A.M. has received research funding and honoraria from Nutricia, Vitaflo International and Biomarin. She is a member of the Advisory Board Element (Danone-Nutricia). The remaining authors do not have any conflicts of interest. The funders had no role in the design of the study; in the collection, analyses, or interpretation of data; in the writing of the manuscript; or in the decision to publish the results. ® Funding Information: We received a National Society for Phenylketonuria (NSPKU) grant for GBP 10,000 to support the costs of this study. Publisher Copyright: © 2023 by the authors.
dc.description.abstractIn phenylketonuria (PKU), a previous intervention study assessing the patients ability to tolerate fruits and vegetables containing phenylalanine 76–100 mg/100 g without limit or measurement, found that an extra 50 mg/day phenylalanine, but not 100 mg/day, was tolerated from these fruits and vegetables. In a further 6-month extension study, we examined the effect of the ‘free’ use of this group of fruits and vegetables on blood phenylalanine control. For 6 months, the patients ate fruits and vegetables containing phenylalanine 76–100 mg/100 g without limit or measurement. Three-day diet diaries and the patients’ weights were collected monthly. Blood phenylalanine spots were collected weekly aiming for blood phenylalanine levels <360 μmol/L. Retrospective blood phenylalanine was collected 6 months pre-trial. All 16 patients (69% females) from the intervention study took part in the extension study. Most of the patients (n = 14/16) had classical PKU with a median age of 10.5 years (range: 6–13). There was no statistically significant difference in the median blood phenylalanine pre-study (270, range: 50–760 μmol/L) compared to the 6-month extension study (250, range: 20–750 μmol/L) (p= 0.4867). The patients had a median of 21 and 22 bloodspots, pre- and post-trial, respectively. In the extension study, the patients had an actual mean intake of 11 g/day (4–37) natural protein and 65 g/day (60–80) protein equivalent from a protein substitute. The mean phenylalanine intake was 563 mg/day (200–1850) with only 19 mg/day (0–146) phenylalanine from fruits and vegetables containing phenylalanine 76–100 mg/100 g. The weight z-scores remained unchanged (1.52 vs. 1.60, p = 0.4715). There was no adverse impact on blood phenylalanine control when fruits and vegetables containing phenylalanine 76–100 mg/100 g were eaten without limit or measurement. However, the fruits and vegetable portion sizes eaten were small (60 g/week). Further longitudinal work is necessary to examine the ‘free’ use of fruits and vegetables containing phenylalanine 76–100 mg/100 g on metabolic control in patients with PKU.en
dc.description.versionpublishersversion
dc.description.versionpublished
dc.format.extent983276
dc.identifier.doi10.3390/nu15133046
dc.identifier.issn1422-8599
dc.identifier.otherPURE: 67081289
dc.identifier.otherPURE UUID: 01f81558-2389-4122-ba4e-900a79486ee7
dc.identifier.otherScopus: 85164739307
dc.identifier.otherPubMed: 37447372
dc.identifier.urihttp://hdl.handle.net/10362/155784
dc.identifier.urlhttps://www.scopus.com/pages/publications/85164739307
dc.language.isoeng
dc.peerreviewedyes
dc.subjectfruits
dc.subjectmetabolic control
dc.subjectphenylalanine
dc.subjectphenylketonuria
dc.subjectvegetables
dc.subjectFood Science
dc.subjectNutrition and Dietetics
dc.titlePreliminary Data on Free Use of Fruits and Vegetables Containing Phenylalanine 76–100 mg/100 g of Food in 16 Children with Phenylketonuriaen
dc.title.subtitle6 Months Follow-Upen
dc.typejournal article
degois.publication.issue13
degois.publication.titleNutrients
degois.publication.volume15
dspace.entity.typePublication
rcaap.rightsopenAccess

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