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

Detalhes bibliográficos
Autor(a) principal: Pinto, Alex
Data de Publicação: 2023
Outros Autores: Daly, Anne, Rocha, Júlio César, Ashmore, Catherine, Evans, Sharon, Jackson, Richard, Hickson, Mary, MacDonald, Anita
Tipo de documento: Artigo
Idioma: eng
Título da fonte: Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
Texto Completo: http://hdl.handle.net/10362/155784
Resumo: Funding 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.
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spelling Preliminary Data on Free Use of Fruits and Vegetables Containing Phenylalanine 76–100 mg/100 g of Food in 16 Children with Phenylketonuria6 Months Follow-Upfruitsmetabolic controlphenylalaninephenylketonuriavegetablesFood ScienceNutrition and DieteticsFunding 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.In 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.NOVA Medical School|Faculdade de Ciências Médicas (NMS|FCM)Centro de Investigação em Tecnologias e Serviços de Saúde (CINTESIS)RUNPinto, AlexDaly, AnneRocha, Júlio CésarAshmore, CatherineEvans, SharonJackson, RichardHickson, MaryMacDonald, Anita2023-07-24T22:16:08Z2023-072023-07-01T00:00:00Zinfo:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/articleapplication/pdfhttp://hdl.handle.net/10362/155784eng1422-8599PURE: 67081289https://doi.org/10.3390/nu15133046info:eu-repo/semantics/openAccessreponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãoinstacron:RCAAP2024-03-11T05:38:22Zoai:run.unl.pt:10362/155784Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireopendoar:71602024-03-20T03:56:12.801160Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãofalse
dc.title.none.fl_str_mv Preliminary Data on Free Use of Fruits and Vegetables Containing Phenylalanine 76–100 mg/100 g of Food in 16 Children with Phenylketonuria
6 Months Follow-Up
title Preliminary Data on Free Use of Fruits and Vegetables Containing Phenylalanine 76–100 mg/100 g of Food in 16 Children with Phenylketonuria
spellingShingle Preliminary Data on Free Use of Fruits and Vegetables Containing Phenylalanine 76–100 mg/100 g of Food in 16 Children with Phenylketonuria
Pinto, Alex
fruits
metabolic control
phenylalanine
phenylketonuria
vegetables
Food Science
Nutrition and Dietetics
title_short Preliminary Data on Free Use of Fruits and Vegetables Containing Phenylalanine 76–100 mg/100 g of Food in 16 Children with Phenylketonuria
title_full Preliminary Data on Free Use of Fruits and Vegetables Containing Phenylalanine 76–100 mg/100 g of Food in 16 Children with Phenylketonuria
title_fullStr Preliminary Data on Free Use of Fruits and Vegetables Containing Phenylalanine 76–100 mg/100 g of Food in 16 Children with Phenylketonuria
title_full_unstemmed Preliminary Data on Free Use of Fruits and Vegetables Containing Phenylalanine 76–100 mg/100 g of Food in 16 Children with Phenylketonuria
title_sort Preliminary Data on Free Use of Fruits and Vegetables Containing Phenylalanine 76–100 mg/100 g of Food in 16 Children with Phenylketonuria
author Pinto, Alex
author_facet Pinto, Alex
Daly, Anne
Rocha, Júlio César
Ashmore, Catherine
Evans, Sharon
Jackson, Richard
Hickson, Mary
MacDonald, Anita
author_role author
author2 Daly, Anne
Rocha, Júlio César
Ashmore, Catherine
Evans, Sharon
Jackson, Richard
Hickson, Mary
MacDonald, Anita
author2_role author
author
author
author
author
author
author
dc.contributor.none.fl_str_mv NOVA Medical School|Faculdade de Ciências Médicas (NMS|FCM)
Centro de Investigação em Tecnologias e Serviços de Saúde (CINTESIS)
RUN
dc.contributor.author.fl_str_mv Pinto, Alex
Daly, Anne
Rocha, Júlio César
Ashmore, Catherine
Evans, Sharon
Jackson, Richard
Hickson, Mary
MacDonald, Anita
dc.subject.por.fl_str_mv fruits
metabolic control
phenylalanine
phenylketonuria
vegetables
Food Science
Nutrition and Dietetics
topic fruits
metabolic control
phenylalanine
phenylketonuria
vegetables
Food Science
Nutrition and Dietetics
description Funding 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.
publishDate 2023
dc.date.none.fl_str_mv 2023-07-24T22:16:08Z
2023-07
2023-07-01T00:00:00Z
dc.type.status.fl_str_mv info:eu-repo/semantics/publishedVersion
dc.type.driver.fl_str_mv info:eu-repo/semantics/article
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status_str publishedVersion
dc.identifier.uri.fl_str_mv http://hdl.handle.net/10362/155784
url http://hdl.handle.net/10362/155784
dc.language.iso.fl_str_mv eng
language eng
dc.relation.none.fl_str_mv 1422-8599
PURE: 67081289
https://doi.org/10.3390/nu15133046
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eu_rights_str_mv openAccess
dc.format.none.fl_str_mv application/pdf
dc.source.none.fl_str_mv reponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
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