Influenceofbloodphenylalaninelevelvariationsonthedevelopmentofexecutivefunctionsandsocialcognitioninchildrenwithphenylketonuria

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Tipo de produção
article
Data de publicação
2023
DOI
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Scopus
Web of Science
PubMed
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Título da Revista
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Título do Volume
Editora
SOC BRASIL PEDIATRIA
Autores
DUARTE, Cristiane Mendes de Almeida
ROCCO, Isadora Salvador
MELLO, Claudia Berlim de
Citação
JORNAL DE PEDIATRIA, v.99, n.5, p.507-513, 2023
Projetos de Pesquisa
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Resumo
Toinvestigatetheperformanceof27childrenwithphenylketonuria(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.
Palavras-chave
Phenylketonuria, Diettherapy, Cognition, Executivefunctions, Theoryofmind
URI
https://observatorio.fm.usp.br/handle/OPI/59099
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Artigos e Materiais de Revistas Científicas - FM/Outros