Influenceofbloodphenylalaninelevelvariationsonthedevelopmentofexecutivefunctionsandsocialcognitioninchildrenwithphenylketonuria

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dc.contributorSistema FMUSP-HC: Faculdade de Medicina da Universidade de São Paulo (FMUSP) e Hospital das Clínicas da FMUSP
dc.contributor.authorDUARTE, Cristiane Mendes de Almeida
dc.contributor.authorPIAZZON, Flavia Balbo
dc.contributor.authorROCCO, Isadora Salvador
dc.contributor.authorMELLO, Claudia Berlim de
dc.date.accessioned2024-04-05T19:36:29Z
dc.date.available2024-04-05T19:36:29Z
dc.date.issued2023
dc.description.abstractToinvestigatetheperformanceof27childrenwithphenylketonuria(PKU) intestsofExecutiveFunctions( EF) andSocialCognition(SC), andtheirassociationswithmetaboliccontrolinferredbyphenylalanine( Phe) levels. Methods: ThePKUgroupwasdichotomizedaccordingtobaselinePhe-levelsinto;""classicalPKU""( n= 14), withPhe-levelsabove1200mmol/L(> 20mg/dL); and""mildPKU""(n= 13) withPhebetween360and1200mmol/L(6-20mg/dL). TheneuropsychologicalassessmentfocusedontheEFandSCsubtestsoftheNEPSYIIbatteryandintellectualperformance. Childrenwerecomparedtoagematchedhealthyparticipants. Results: ParticipantswithPKUpresentedsignificantlylowerIntellectualQuotient(IQ) comparedtocontrols( p= 0.001). RegardingEFanalysisadjustedbyageandIQ, significantdifferencesbetweengroupswereobservedonlyintheexecutiveattentionsubtests( p= 0.029). TheSCsetofvariableswassignificantlydifferentbetweengroups( p= 0.003), asintheaffectiverecognitiontask(p< 0.001). InthePKUgroup, therelativevariationofPhe-achieved32.1 +/- 21.0%. RelativePhe-variationwascorrelatedonlywithmeasuresofWorkingMemory(p< 0.001), VerbalFluency( p= 0.004), InhibitoryControl(p= 0.035) andTheoryofMind(p= 0.003). Conclusions: PhonologicalVerbalFluency, WorkingMemory, InhibitoryControl, andTheoryofMindwereshowntobemostvulnerablewhenthereisnonidealmetaboliccontrol. VariationsinthelevelofPhemayhaveaselectivenegativeeffectonExecutiveFunctionsandSocialCogni- tion, butnotonintellectualperformance.eng
dc.description.indexMEDLINE
dc.description.indexPubMed
dc.description.indexScopus
dc.description.indexDimensions
dc.description.indexWoS
dc.description.indexScielo
dc.identifier.citationJORNAL DE PEDIATRIA, v.99, n.5, p.507-513, 2023
dc.identifier.doi10.1016/j.jped.2023.04.003
dc.identifier.eissn1678-4782
dc.identifier.issn0021-7557
dc.identifier.urihttps://observatorio.fm.usp.br/handle/OPI/59099
dc.language.isoeng
dc.publisherSOC BRASIL PEDIATRIAeng
dc.relation.ispartofJornal de Pediatria
dc.rightsopenAccesseng
dc.rights.holderCopyright SOC BRASIL PEDIATRIAeng
dc.subjectPhenylketonuriaeng
dc.subjectDiettherapyeng
dc.subjectCognitioneng
dc.subjectExecutivefunctionseng
dc.subjectTheoryofmindeng
dc.subject.otherearly-treated phenylketonuriaeng
dc.subject.otherphenylalanineeng
dc.subject.otherchildreneng
dc.subject.otherdiagnosiseng
dc.subject.otheradultseng
dc.subject.otherpkueng
dc.subject.wosPediatricseng
dc.titleInfluenceofbloodphenylalaninelevelvariationsonthedevelopmentofexecutivefunctionsandsocialcognitioninchildrenwithphenylketonuriaeng
dc.typearticleeng
dc.type.categoryoriginal articleeng
dc.type.versionpublishedVersioneng
dspace.entity.typePublication
hcfmusp.author.externalDUARTE, Cristiane Mendes de Almeida:Univ Fed Sao Paulo UNIFESP, Dept Psicobiol, Sao Paulo, SP, Brazil
hcfmusp.author.externalROCCO, Isadora Salvador:Univ Fed Sao Paulo UNIFESP, Programa Posgrad Cardiol, Sao Paulo, SP, Brazil; Dept Cirurgia Cardiaca, Sao Paulo, SP, Brazil
hcfmusp.author.externalMELLO, Claudia Berlim de:Univ Fed Sao Paulo UNIFESP, Dept Psicobiol, Sao Paulo, SP, Brazil
hcfmusp.citation.scopus0
hcfmusp.contributor.author-fmusphcFLAVIA BALBO PIAZZON
hcfmusp.description.beginpage507
hcfmusp.description.endpage513
hcfmusp.description.issue5
hcfmusp.description.volume99
hcfmusp.origemWOS
hcfmusp.origem.dimensionspub.1157909771
hcfmusp.origem.pubmed37178714
hcfmusp.origem.scieloSCIELO:S0021-75572023000500507
hcfmusp.origem.scopus2-s2.0-85160227887
hcfmusp.origem.wosWOS:001072978100001
hcfmusp.publisher.cityRIO DE JANEIRO, RJeng
hcfmusp.publisher.countryBRAZILeng
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