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Longitudinal Dietary Intake Data in Patients with Phenylketonuria from Europe

2024-09Artigo científico Acesso aberto
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dc.contributor.authorPinto, Alex
dc.contributor.authorAhring, Kirsten
dc.contributor.authorAlmeida, Manuela Ferreira
dc.contributor.authorAshmore, Catherine
dc.contributor.authorBélanger-Quintana, Amaya
dc.contributor.authorBurlina, Alberto
dc.contributor.authorCoşkun, Turgay
dc.contributor.authorDaly, Anne
dc.contributor.authorvan Dam, Esther
dc.contributor.authorDursun, Ali
dc.contributor.authorEvans, Sharon
dc.contributor.authorFeillet, François
dc.contributor.authorGiżewska, Maria
dc.contributor.authorGökmen-Özel, Hulya
dc.contributor.authorHickson, Mary
dc.contributor.authorHoekstra, Yteke
dc.contributor.authorIlgaz, Fatma
dc.contributor.authorJackson, Richard
dc.contributor.authorLeśniak, Alicja
dc.contributor.authorLoro, Christian
dc.contributor.authorMalicka, Katarzyna
dc.contributor.authorPatalan, Michał
dc.contributor.authorRocha, Júlio César
dc.contributor.authorSivri, Serap
dc.contributor.authorRodenburg, Iris
dc.contributor.authorvan Spronsen, Francjan
dc.contributor.authorStrączek, Kamilla
dc.contributor.authorTokatli, Ayşegül
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.institutionComprehensive Health Research Centre (CHRC) - pólo NMS
dc.contributor.pblMDPI AG
dc.date.accessioned2024-09-30T22:32:35Z
dc.date.available2024-09-30T22:32:35Z
dc.date.issued2024-09
dc.descriptionFunding 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.
dc.description.abstractIn 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.en
dc.description.versionpublishersversion
dc.description.versionpublished
dc.format.extent342072
dc.identifier.doi10.3390/nu16172909
dc.identifier.issn1422-8599
dc.identifier.otherPURE: 99898799
dc.identifier.otherPURE UUID: 5323d2da-776c-4e6f-b3d7-67d77f662829
dc.identifier.otherScopus: 85204105610
dc.identifier.otherPubMed: 39275225
dc.identifier.urihttp://hdl.handle.net/10362/172743
dc.identifier.urlhttps://www.scopus.com/pages/publications/85204105610
dc.language.isoeng
dc.peerreviewedyes
dc.subjectnatural protein intake
dc.subjectphenylalanine intake
dc.subjectphenylketonuria
dc.subjectprotein equivalent from protein substitute intake
dc.subjecttotal protein intake
dc.subjectFood Science
dc.subjectNutrition and Dietetics
dc.titleLongitudinal Dietary Intake Data in Patients with Phenylketonuria from Europeen
dc.title.subtitleThe Impact of Age and Phenylketonuria Severityen
dc.typejournal article
degois.publication.issue17
degois.publication.titleNutrients
degois.publication.volume16
dspace.entity.typePublication
rcaap.rightsopenAccess

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