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Over Restriction of Dietary Protein Allowance: The Importance of Ongoing Reassessment of Natural Protein Tolerance in Phenylketonuria

2019-04-30Journal article Open access
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dc.contributor.authorPinto, Alex
dc.contributor.authorAlmeida, Manuela Ferreira
dc.contributor.authorMacDonald, Anita
dc.contributor.authorRamos, Paula Cristina
dc.contributor.authorRocha, Sara
dc.contributor.authorGuimas, Arlindo
dc.contributor.authorRibeiro, Rosa
dc.contributor.authorMartins, Esmeralda
dc.contributor.authorBandeira, Anabela
dc.contributor.authorJackson, Richard
dc.contributor.authorvan Spronsen, Francjan
dc.contributor.authorPayne, Anne
dc.contributor.authorRocha, Júlio César
dc.date.accessioned2020-07-14T14:49:24Z
dc.date.available2020-07-14T14:49:24Z
dc.date.issued2019-04-30
dc.description.abstractPhenylalanine (Phe) tolerance is highly variable in phenylketonuria (PKU) and rarely described in patients aged ≥12 years. Patients ≥12 years of age with PKU were systematically challenged with additional natural protein (NP) if blood Phe levels remained below 480 µmol/L (i.e., upper target blood Phe level for patients aged ≥12 years using Portuguese PKU guidelines). In PKU patients, NP tolerance was calculated at baseline and a median of 6 months after systematic challenge with NP whilst patients were maintaining a blood Phe ≤480 μmol/L. Anthropometry was assessed at both times. Routine blood Phe levels were collected. We studied 40 well-controlled PKU patients (10 hyperphenylalaninemia (HPA), 23 mild and 7 classic PKU), on a low-Phe diet with a mean age of 17 years (12-29 years). Median daily NP intake significantly increased between assessments (35 vs. 40 g/day, p = 0.01). Twenty-six patients (65%) were able to increase their median NP intake by a median 12 g/day (2-42 g)/day and still maintain blood Phe within target range. Out of the previous 26 patients, 20 (77%) (8 HPA, 11 mild and 1 classical PKU) increased NP from animal sources (e.g. dairy products, fish and meat) and 6 patients (23%) (3 mild and 3 classical PKU) from plant foods (bread, pasta, potatoes). Median protein equivalent intake from Phe-free/low-Phe protein substitute decreased (0.82 vs. 0.75 g/kg, p = 0.01), while median blood Phe levels remained unchanged (279 vs. 288 μmol/L, p = 0.06). Almost two-thirds of patients with PKU tolerated additional NP when challenged and still maintained blood Phe within the national target range. This suggests that some patients with PKU treated by a low-Phe diet only may over restrict their NP intake. In order to minimise the burden of treatment and optimise NP intake, it is important to challenge with additional NP at periodic intervals.pt_PT
dc.description.sponsorshipThis article was supported by National Funds through FCT-Fundação para a Ciência e a Tecnologia within CINTESIS, R&D Unit (reference UID/IC/4255/2019)pt_PT
dc.description.versioninfo:eu-repo/semantics/publishedVersionpt_PT
dc.identifier.citationPinto A, Almeida MF, MacDonald A, et al. Over Restriction of Dietary Protein Allowance: The Importance of Ongoing Reassessment of Natural Protein Tolerance in Phenylketonuria. Nutrients. 2019;11(5):995. Published 2019 Apr 30. doi:10.3390/nu11050995pt_PT
dc.identifier.doi10.3390/nu11050995pt_PT
dc.identifier.issn2072-6643
dc.identifier.urihttp://hdl.handle.net/10400.16/2427
dc.language.isoengpt_PT
dc.peerreviewedyespt_PT
dc.publisherMDPIpt_PT
dc.relation.publisherversionhttps://www.mdpi.com/2072-6643/11/5/995pt_PT
dc.rights.urihttp://creativecommons.org/licenses/by/4.0/pt_PT
dc.subjectBH4pt_PT
dc.subjectnatural protein tolerancept_PT
dc.subjectphenylketonuriapt_PT
dc.subjectphenylalaninept_PT
dc.titleOver Restriction of Dietary Protein Allowance: The Importance of Ongoing Reassessment of Natural Protein Tolerance in Phenylketonuriapt_PT
dc.typejournal article
dspace.entity.typePublication
oaire.citation.conferencePlaceSwitzerlandpt_PT
oaire.citation.issue5pt_PT
oaire.citation.startPage995pt_PT
oaire.citation.titleNutrientspt_PT
oaire.citation.volume11pt_PT
person.familyNameRocha
person.givenNameJúlio César
person.identifier.ciencia-idED11-107D-ABFB
person.identifier.orcid0000-0002-4977-8345
person.identifier.ridK-5399-2013
person.identifier.scopus-author-id35196853000
rcaap.rightsopenAccesspt_PT
rcaap.typearticlept_PT
relation.isAuthorOfPublication2d020da8-ada9-4090-bef1-0ceae77f3fda
relation.isAuthorOfPublication.latestForDiscovery2d020da8-ada9-4090-bef1-0ceae77f3fda

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