Natural Protein Intake in 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, Ilgaz, Fatma, 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/10362/161520
Resumo: Funding Information: A.P. received an educational grant from Cambrooke Therapeutics and grants from Vitaflo International, Nutricia, Merck Serono, Biomarin, Mevalia, Galen, 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 ExpertPanel (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 both 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). ® Publisher Copyright: © 2023 by the authors.
id RCAP_a8c2c90c34116ac3bf0d33ed23f3089a
oai_identifier_str oai:run.unl.pt:10362/161520
network_acronym_str RCAP
network_name_str Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
repository_id_str 7160
spelling Natural Protein Intake in Children with PhenylketonuriaPrescription vs. Actual Intakesfruitsmetabolic controlphenylalaninephenylketonuriavegetablesFood ScienceNutrition and DieteticsFunding Information: A.P. received an educational grant from Cambrooke Therapeutics and grants from Vitaflo International, Nutricia, Merck Serono, Biomarin, Mevalia, Galen, 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 ExpertPanel (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 both 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). ® Publisher Copyright: © 2023 by the authors.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.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, SharonIlgaz, FatmaHickson, MaryMacDonald, Anita2023-12-20T23:54:35Z2023-122023-12-01T00:00:00Zinfo:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/articleapplication/pdfhttp://hdl.handle.net/10362/161520eng1422-8599PURE: 78405429https://doi.org/10.3390/nu15234903info: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:RCAAP2024-03-11T05:44:26Zoai:run.unl.pt:10362/161520Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireopendoar:71602024-03-20T03:58:33.993Repositório 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 Natural Protein Intake in Children with Phenylketonuria
Prescription vs. Actual Intakes
title Natural Protein Intake in Children with Phenylketonuria
spellingShingle Natural Protein Intake in Children with Phenylketonuria
Pinto, Alex
fruits
metabolic control
phenylalanine
phenylketonuria
vegetables
Food Science
Nutrition and Dietetics
title_short Natural Protein Intake in Children with Phenylketonuria
title_full Natural Protein Intake in Children with Phenylketonuria
title_fullStr Natural Protein Intake in Children with Phenylketonuria
title_full_unstemmed Natural Protein Intake in Children with Phenylketonuria
title_sort Natural Protein Intake in Children with Phenylketonuria
author Pinto, Alex
author_facet Pinto, Alex
Daly, Anne
Rocha, Júlio César
Ashmore, Catherine
Evans, Sharon
Ilgaz, Fatma
Hickson, Mary
MacDonald, Anita
author_role author
author2 Daly, Anne
Rocha, Júlio César
Ashmore, Catherine
Evans, Sharon
Ilgaz, Fatma
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
Ilgaz, Fatma
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, Galen, 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 ExpertPanel (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 both 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). ® Publisher Copyright: © 2023 by the authors.
publishDate 2023
dc.date.none.fl_str_mv 2023-12-20T23:54:35Z
2023-12
2023-12-01T00:00:00Z
dc.type.status.fl_str_mv info:eu-repo/semantics/publishedVersion
dc.type.driver.fl_str_mv info:eu-repo/semantics/article
format article
status_str publishedVersion
dc.identifier.uri.fl_str_mv http://hdl.handle.net/10362/161520
url http://hdl.handle.net/10362/161520
dc.language.iso.fl_str_mv eng
language eng
dc.relation.none.fl_str_mv 1422-8599
PURE: 78405429
https://doi.org/10.3390/nu15234903
dc.rights.driver.fl_str_mv info:eu-repo/semantics/openAccess
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)
instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação
instacron:RCAAP
instname_str Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação
instacron_str RCAAP
institution RCAAP
reponame_str Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
collection Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)
repository.name.fl_str_mv Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação
repository.mail.fl_str_mv
_version_ 1799138166676914176

Registros relacionados