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Longitudinal Dietary Intake Data in Patients with Phenylketonuria from Europe

Bibliographic Details
Main Author: Pinto, Alex
Publication Date: 2024
Other Authors: Ahring, Kirsten, Almeida, Manuela Ferreira, Ashmore, Catherine, Bélanger-Quintana, Amaya, Burlina, Alberto, Coşkun, Turgay, Daly, Anne, van Dam, Esther, Dursun, Ali, Evans, Sharon, Feillet, François, Giżewska, Maria, Gökmen-Özel, Hulya, Hickson, Mary, Hoekstra, Yteke, Ilgaz, Fatma, Jackson, Richard, Leśniak, Alicja, Loro, Christian, Malicka, Katarzyna, Patalan, Michał, Rocha, Júlio César, Sivri, Serap, Rodenburg, Iris, van Spronsen, Francjan, Strączek, Kamilla, Tokatli, Ayşegül, MacDonald, Anita
Format: Article
Language: eng
Source: Repositórios Científicos de Acesso Aberto de Portugal (RCAAP)
Download full: http://hdl.handle.net/10362/172743
Summary: Funding Information: A.P. received an educational grant from Cambrooke Therapeutics and Biomarin and grants from Vitaflo International, Nutricia, Merck Serono, Biomarin, Mevalia, Galen, PIAM, and Applied Pharma Research to attend scientific meetings. This project is also part of A.P.\u2019s 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) and 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 and financial support to attend study days and conferences from both Nutricia and Vitaflo International. C.A. received honoraria from Nutricia and Vitaflo International to attend study days and conferences. A.M. received research funding and honoraria from Nutricia, Vitaflo International, and Biomarin. She is a member of the Advisory Board Element (Danone-Nutricia). F.F. was a member of the Advisory Board for Vitaflo, Biomarin, Chiesi, Sanofi-Genzyme, and Nutricia and received honoraria as a speaker from Recordati, Biomarin, Merck Serono, Vitaflo, Travere Therapeutics, PTC Therapeutics, Lucane, and Alexion. M.G. received financial support and honoraria as a speaker from Biomarin, Nutricia, Vitaflo, Nestl\u00E9 Polska, Mead-Johnson, PTC Therapeutics International, and APR Applied Pharma Research. The remaining authors do not have any conflicts of interest. The funders had no role in the design of this study; in the collection, analyses, or interpretation of the data; in the writing of the manuscript; or in the decision to publish the results. Publisher Copyright: © 2024 by the authors.
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spelling Longitudinal Dietary Intake Data in Patients with Phenylketonuria from EuropeThe Impact of Age and Phenylketonuria Severitynatural protein intakephenylalanine intakephenylketonuriaprotein equivalent from protein substitute intaketotal protein intakeFood ScienceNutrition and DieteticsFunding Information: A.P. received an educational grant from Cambrooke Therapeutics and Biomarin and grants from Vitaflo International, Nutricia, Merck Serono, Biomarin, Mevalia, Galen, PIAM, and Applied Pharma Research to attend scientific meetings. This project is also part of A.P.\u2019s 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) and 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 and financial support to attend study days and conferences from both Nutricia and Vitaflo International. C.A. received honoraria from Nutricia and Vitaflo International to attend study days and conferences. A.M. received research funding and honoraria from Nutricia, Vitaflo International, and Biomarin. She is a member of the Advisory Board Element (Danone-Nutricia). F.F. was a member of the Advisory Board for Vitaflo, Biomarin, Chiesi, Sanofi-Genzyme, and Nutricia and received honoraria as a speaker from Recordati, Biomarin, Merck Serono, Vitaflo, Travere Therapeutics, PTC Therapeutics, Lucane, and Alexion. M.G. received financial support and honoraria as a speaker from Biomarin, Nutricia, Vitaflo, Nestl\u00E9 Polska, Mead-Johnson, PTC Therapeutics International, and APR Applied Pharma Research. The remaining authors do not have any conflicts of interest. The funders had no role in the design of this study; in the collection, analyses, or interpretation of the data; in the writing of the manuscript; or in the decision to publish the results. Publisher Copyright: © 2024 by the authors.In phenylketonuria (PKU), natural protein intake is thought to increase with age, particularly during childhood and adolescence. Longitudinal dietary intake data are scarce and lifelong phenylalanine tolerance remains unknown. Nine centres managing PKU in Europe and Turkey participated in a retrospective study. Data were collected from dietetic records between 2012 and 2018 on phenylalanine (Phe), natural protein, and protein substitute intake. A total of 1323 patients (age range: 1–57 y; 51% male) participated. Dietary intake data were available on 1163 (88%) patients. Patient numbers ranged from 59 to 320 in each centre. A total of 625 (47%) had classical PKU (cPKU), n = 357 (27%) had mild PKU (mPKU), n = 325 (25%) had hyperphenylalaninemia (HPA), and n = 16 (1%) were unknown. The mean percentage of blood Phe levels within target ranged from 65 ± 54% to 88 ± 49%. When intake was expressed as g/day, the mean Phe/natural protein and protein equivalent from protein substitute gradually increased during childhood, reaching a peak in adolescence, and then remained consistent during adulthood. When intake was expressed per kg body weight (g/kg/day), there was a decline in Phe/natural protein, protein equivalent from protein substitute, and total protein with increasing age. Overall, the mean daily intake (kg/day) was as follows: Phe, 904 mg ± 761 (22 ± 23 mg/kg/day), natural protein 19 g ± 16 (0.5 g/kg/day ± 0.5), protein equivalent from protein substitute 39 g ± 22 (1.1 g/kg/day ± 0.6), and total protein 59 g ± 21 (1.7 g/kg/day ± 0.6). Natural protein tolerance was similar between males and females. Patients with mPKU tolerated around 50% less Phe/natural protein than HPA, but 50% more than cPKU. Higher intakes of natural protein were observed in Southern Europe, with a higher prevalence of HPA and mPKU compared with patients from Northern European centres. Natural protein intake doubled with sapropterin usage. In sapropterin-responsive patients, 31% no longer used protein substitutes. Close monitoring and optimisation of protein intake prescriptions are needed, along with future guidelines specifically for different age groups and severities.NOVA Medical School|Faculdade de Ciências Médicas (NMS|FCM)Centro de Investigação em Tecnologias e Serviços de Saúde (CINTESIS)Comprehensive Health Research Centre (CHRC) - pólo NMSRUNPinto, AlexAhring, KirstenAlmeida, Manuela FerreiraAshmore, CatherineBélanger-Quintana, AmayaBurlina, AlbertoCoşkun, TurgayDaly, Annevan Dam, EstherDursun, AliEvans, SharonFeillet, FrançoisGiżewska, MariaGökmen-Özel, HulyaHickson, MaryHoekstra, YtekeIlgaz, FatmaJackson, RichardLeśniak, AlicjaLoro, ChristianMalicka, KatarzynaPatalan, MichałRocha, Júlio CésarSivri, SerapRodenburg, Irisvan Spronsen, FrancjanStrączek, KamillaTokatli, AyşegülMacDonald, Anita2024-09-30T22:32:35Z2024-092024-09-01T00:00:00Zinfo:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/articleapplication/pdfhttp://hdl.handle.net/10362/172743eng1422-8599PURE: 99898799https://doi.org/10.3390/nu16172909info: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:RCAAP2025-02-10T01:36:46Zoai:run.unl.pt:10362/172743Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireinfo@rcaap.ptopendoar:https://opendoar.ac.uk/repository/71602025-05-28T18:55:20.381661Repositórios 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 Longitudinal Dietary Intake Data in Patients with Phenylketonuria from Europe
The Impact of Age and Phenylketonuria Severity
title Longitudinal Dietary Intake Data in Patients with Phenylketonuria from Europe
spellingShingle Longitudinal Dietary Intake Data in Patients with Phenylketonuria from Europe
Pinto, Alex
natural protein intake
phenylalanine intake
phenylketonuria
protein equivalent from protein substitute intake
total protein intake
Food Science
Nutrition and Dietetics
title_short Longitudinal Dietary Intake Data in Patients with Phenylketonuria from Europe
title_full Longitudinal Dietary Intake Data in Patients with Phenylketonuria from Europe
title_fullStr Longitudinal Dietary Intake Data in Patients with Phenylketonuria from Europe
title_full_unstemmed Longitudinal Dietary Intake Data in Patients with Phenylketonuria from Europe
title_sort Longitudinal Dietary Intake Data in Patients with Phenylketonuria from Europe
author Pinto, Alex
author_facet Pinto, Alex
Ahring, Kirsten
Almeida, Manuela Ferreira
Ashmore, Catherine
Bélanger-Quintana, Amaya
Burlina, Alberto
Coşkun, Turgay
Daly, Anne
van Dam, Esther
Dursun, Ali
Evans, Sharon
Feillet, François
Giżewska, Maria
Gökmen-Özel, Hulya
Hickson, Mary
Hoekstra, Yteke
Ilgaz, Fatma
Jackson, Richard
Leśniak, Alicja
Loro, Christian
Malicka, Katarzyna
Patalan, Michał
Rocha, Júlio César
Sivri, Serap
Rodenburg, Iris
van Spronsen, Francjan
Strączek, Kamilla
Tokatli, Ayşegül
MacDonald, Anita
author_role author
author2 Ahring, Kirsten
Almeida, Manuela Ferreira
Ashmore, Catherine
Bélanger-Quintana, Amaya
Burlina, Alberto
Coşkun, Turgay
Daly, Anne
van Dam, Esther
Dursun, Ali
Evans, Sharon
Feillet, François
Giżewska, Maria
Gökmen-Özel, Hulya
Hickson, Mary
Hoekstra, Yteke
Ilgaz, Fatma
Jackson, Richard
Leśniak, Alicja
Loro, Christian
Malicka, Katarzyna
Patalan, Michał
Rocha, Júlio César
Sivri, Serap
Rodenburg, Iris
van Spronsen, Francjan
Strączek, Kamilla
Tokatli, Ayşegül
MacDonald, Anita
author2_role author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
author
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)
Comprehensive Health Research Centre (CHRC) - pólo NMS
RUN
dc.contributor.author.fl_str_mv Pinto, Alex
Ahring, Kirsten
Almeida, Manuela Ferreira
Ashmore, Catherine
Bélanger-Quintana, Amaya
Burlina, Alberto
Coşkun, Turgay
Daly, Anne
van Dam, Esther
Dursun, Ali
Evans, Sharon
Feillet, François
Giżewska, Maria
Gökmen-Özel, Hulya
Hickson, Mary
Hoekstra, Yteke
Ilgaz, Fatma
Jackson, Richard
Leśniak, Alicja
Loro, Christian
Malicka, Katarzyna
Patalan, Michał
Rocha, Júlio César
Sivri, Serap
Rodenburg, Iris
van Spronsen, Francjan
Strączek, Kamilla
Tokatli, Ayşegül
MacDonald, Anita
dc.subject.por.fl_str_mv natural protein intake
phenylalanine intake
phenylketonuria
protein equivalent from protein substitute intake
total protein intake
Food Science
Nutrition and Dietetics
topic natural protein intake
phenylalanine intake
phenylketonuria
protein equivalent from protein substitute intake
total protein intake
Food Science
Nutrition and Dietetics
description Funding Information: A.P. received an educational grant from Cambrooke Therapeutics and Biomarin and grants from Vitaflo International, Nutricia, Merck Serono, Biomarin, Mevalia, Galen, PIAM, and Applied Pharma Research to attend scientific meetings. This project is also part of A.P.\u2019s 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) and 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 and financial support to attend study days and conferences from both Nutricia and Vitaflo International. C.A. received honoraria from Nutricia and Vitaflo International to attend study days and conferences. A.M. received research funding and honoraria from Nutricia, Vitaflo International, and Biomarin. She is a member of the Advisory Board Element (Danone-Nutricia). F.F. was a member of the Advisory Board for Vitaflo, Biomarin, Chiesi, Sanofi-Genzyme, and Nutricia and received honoraria as a speaker from Recordati, Biomarin, Merck Serono, Vitaflo, Travere Therapeutics, PTC Therapeutics, Lucane, and Alexion. M.G. received financial support and honoraria as a speaker from Biomarin, Nutricia, Vitaflo, Nestl\u00E9 Polska, Mead-Johnson, PTC Therapeutics International, and APR Applied Pharma Research. The remaining authors do not have any conflicts of interest. The funders had no role in the design of this study; in the collection, analyses, or interpretation of the data; in the writing of the manuscript; or in the decision to publish the results. Publisher Copyright: © 2024 by the authors.
publishDate 2024
dc.date.none.fl_str_mv 2024-09-30T22:32:35Z
2024-09
2024-09-01T00:00:00Z
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