Phenylalanine Tolerance over Time in Phenylketonuria

Bibliographic Details
Main Author: Pinto, Alex
Publication Date: 2023
Other Authors: Ilgaz, Fatma, Evans, Sharon, van Dam, Esther, Rocha, Júlio César, Karabulut, Erdem, Hickson, Mary, Daly, Anne, MacDonald, Anita
Format: Other
Language: eng
Source: Repositórios Científicos de Acesso Aberto de Portugal (RCAAP)
Download full: http://hdl.handle.net/10362/157771
Summary: 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. 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 speaker from APR, Merck Serono, Biomarin, Nutricia, Vitaflo, Cambrooke, PIAM and Lifediet. A.M. has received research funding and honoraria from Danone Nutricia, Vitaflo International, Biomarin, MetaX, Applied Pharma Research, and Merck Serono; she is a member of the advisory board for Danone Nutricia, Arla, and Applied Pharma Research. The remaining authors declare no conflicts of interest. ® Publisher Copyright: © 2023 by the authors.
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spelling Phenylalanine Tolerance over Time in PhenylketonuriaA Systematic Review and Meta-Analysisamino acidsmedical formulanatural proteinphenylalaninephenylalanine tolerancephenylketonuriaPKUproteinprotein substituteprotein toleranceFood 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. 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 speaker from APR, Merck Serono, Biomarin, Nutricia, Vitaflo, Cambrooke, PIAM and Lifediet. A.M. has received research funding and honoraria from Danone Nutricia, Vitaflo International, Biomarin, MetaX, Applied Pharma Research, and Merck Serono; she is a member of the advisory board for Danone Nutricia, Arla, and Applied Pharma Research. The remaining authors declare no conflicts of interest. ® Publisher Copyright: © 2023 by the authors.In phenylketonuria (PKU), natural protein tolerance is defined as the maximum natural protein intake maintaining a blood phenylalanine (Phe) concentration within a target therapeutic range. Tolerance is affected by several factors, and it may differ throughout a person’s lifespan. Data on lifelong Phe/natural protein tolerance are limited and mostly reported in studies with low subject numbers. This systematic review aimed to investigate how Phe/natural protein tolerance changes from birth to adulthood in well-controlled patients with PKU on a Phe-restricted diet. Five electronic databases were searched for articles published until July 2020. From a total of 1334 results, 37 articles met the eligibility criteria (n = 2464 patients), and 18 were included in the meta-analysis. The mean Phe (mg/day) and natural protein (g/day) intake gradually increased from birth until 6 y (at the age of 6 months, the mean Phe intake was 267 mg/day, and natural protein intake was 5.4 g/day; at the age of 5 y, the mean Phe intake was 377 mg/day, and the natural protein intake was 8.9 g/day). However, an increase in Phe/natural protein tolerance was more apparent at the beginning of late childhood and was >1.5-fold that of the Phe tolerance in early childhood. During the pubertal growth spurt, the mean natural protein/Phe tolerance was approximately three times higher than in the first year of life, reaching a mean Phe intake of 709 mg/day and a mean natural protein intake of 18 g/day. Post adolescence, a pooled analysis could only be performed for natural protein intake. The mean natural protein tolerance reached its highest (32.4 g/day) point at the age of 17 y and remained consistent (31.6 g/day) in adulthood, but limited data were available. The results of the meta-analysis showed that Phe/natural protein tolerance (expressed as mg or g per day) increases with age, particularly at the beginning of puberty, and reaches its highest level at the end of adolescence. This needs to be interpreted with caution as limited data were available in adult patients. There was also a high degree of heterogeneity between studies due to differences in sample size, the severity of PKU, and target therapeutic levels for blood Phe control.NOVA Medical School|Faculdade de Ciências Médicas (NMS|FCM)Centro de Investigação em Tecnologias e Serviços de Saúde (CINTESIS)RUNPinto, AlexIlgaz, FatmaEvans, Sharonvan Dam, EstherRocha, Júlio CésarKarabulut, ErdemHickson, MaryDaly, AnneMacDonald, Anita2023-09-13T22:18:52Z2023-082023-08-01T00:00:00Zinfo:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/otherapplication/pdfhttp://hdl.handle.net/10362/157771eng1422-8599PURE: 71099501https://doi.org/10.3390/nu15163506info:eu-repo/semantics/openAccessreponame:Repositórios Científicos de Acesso Aberto de Portugal (RCAAP)instname:FCCN, serviços digitais da FCT – Fundação para a Ciência e a Tecnologiainstacron:RCAAP2024-05-22T18:14:22Zoai:run.unl.pt:10362/157771Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireinfo@rcaap.ptopendoar:https://opendoar.ac.uk/repository/71602025-05-28T17:44:59.049110Repositórios Científicos de Acesso Aberto de Portugal (RCAAP) - FCCN, serviços digitais da FCT – Fundação para a Ciência e a Tecnologiafalse
dc.title.none.fl_str_mv Phenylalanine Tolerance over Time in Phenylketonuria
A Systematic Review and Meta-Analysis
title Phenylalanine Tolerance over Time in Phenylketonuria
spellingShingle Phenylalanine Tolerance over Time in Phenylketonuria
Pinto, Alex
amino acids
medical formula
natural protein
phenylalanine
phenylalanine tolerance
phenylketonuria
PKU
protein
protein substitute
protein tolerance
Food Science
Nutrition and Dietetics
title_short Phenylalanine Tolerance over Time in Phenylketonuria
title_full Phenylalanine Tolerance over Time in Phenylketonuria
title_fullStr Phenylalanine Tolerance over Time in Phenylketonuria
title_full_unstemmed Phenylalanine Tolerance over Time in Phenylketonuria
title_sort Phenylalanine Tolerance over Time in Phenylketonuria
author Pinto, Alex
author_facet Pinto, Alex
Ilgaz, Fatma
Evans, Sharon
van Dam, Esther
Rocha, Júlio César
Karabulut, Erdem
Hickson, Mary
Daly, Anne
MacDonald, Anita
author_role author
author2 Ilgaz, Fatma
Evans, Sharon
van Dam, Esther
Rocha, Júlio César
Karabulut, Erdem
Hickson, Mary
Daly, Anne
MacDonald, Anita
author2_role author
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
Ilgaz, Fatma
Evans, Sharon
van Dam, Esther
Rocha, Júlio César
Karabulut, Erdem
Hickson, Mary
Daly, Anne
MacDonald, Anita
dc.subject.por.fl_str_mv amino acids
medical formula
natural protein
phenylalanine
phenylalanine tolerance
phenylketonuria
PKU
protein
protein substitute
protein tolerance
Food Science
Nutrition and Dietetics
topic amino acids
medical formula
natural protein
phenylalanine
phenylalanine tolerance
phenylketonuria
PKU
protein
protein substitute
protein tolerance
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. 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 speaker from APR, Merck Serono, Biomarin, Nutricia, Vitaflo, Cambrooke, PIAM and Lifediet. A.M. has received research funding and honoraria from Danone Nutricia, Vitaflo International, Biomarin, MetaX, Applied Pharma Research, and Merck Serono; she is a member of the advisory board for Danone Nutricia, Arla, and Applied Pharma Research. The remaining authors declare no conflicts of interest. ® Publisher Copyright: © 2023 by the authors.
publishDate 2023
dc.date.none.fl_str_mv 2023-09-13T22:18:52Z
2023-08
2023-08-01T00:00:00Z
dc.type.status.fl_str_mv info:eu-repo/semantics/publishedVersion
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status_str publishedVersion
dc.identifier.uri.fl_str_mv http://hdl.handle.net/10362/157771
url http://hdl.handle.net/10362/157771
dc.language.iso.fl_str_mv eng
language eng
dc.relation.none.fl_str_mv 1422-8599
PURE: 71099501
https://doi.org/10.3390/nu15163506
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