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However, neurocognitive outcome is suboptimal. This study aimed to investigate behavior problems and health-related quality of life (HR-QoL) in NTBC-dietary-treated TT1 and to relate this to phenylalanine and tyrosine concentrations.<\/jats:p>\n<\/jats:sec><jats:sec>\n<jats:title>Results<\/jats:title>\n<jats:p>Thirty-one TT1 patients (19 males; mean age 13.9\u2009\u00b1\u20095.3\u2009years) were included in this study. Emotional and behavioral problems, as measured by the Achenbach System of Empirically Based Assessment, were present in almost all domains. Attention and thought problems were particularly evident. HR-QoL was assessed by the TNO AZL Children\u2019s and Adults QoL questionnaires. Poorer HR-QoL as compared to reference populations was observed for the domains: independent daily functioning, cognitive functioning and school performance, social contacts, motor functioning, and vitality. Both internalizing and externalizing behavior problems were associated with low phenylalanine (and associated lower tyrosine) concentrations during the first year of life. In contrast, high tyrosine (and associated higher phenylalanine) concentrations during life and specifically the last year before testing were associated with more internalizing behavior and\/or HR-QoL problems.<\/jats:p>\n<\/jats:sec><jats:sec>\n<jats:title>Conclusions<\/jats:title>\n<jats:p>TT1 patients showed several behavior problems and a lower HR-QoL. Associations with metabolic control differed for different age periods. This suggests the need for continuous fine-tuning and monitoring of dietary treatment to keep phenylalanine and tyrosine concentrations within target ranges in NTBC-treated TT1 patients.<\/jats:p>\n<\/jats:sec>","DOI":"10.1186\/s13023-019-1259-2","type":"journal-article","created":{"date-parts":[[2019,12,4]],"date-time":"2019-12-04T20:03:03Z","timestamp":1575489783000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":21,"title":["Emotional and behavioral problems, quality of life and metabolic control in NTBC-treated Tyrosinemia type 1 patients"],"prefix":"10.1186","volume":"14","author":[{"given":"Kimber","family":"van Vliet","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Willem G.","family":"van Ginkel","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rianne","family":"Jahja","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anne","family":"Daly","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anita","family":"MacDonald","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Corinne","family":"De Laet","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Roshni","family":"Vara","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yusof","family":"Rahman","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David","family":"Cassiman","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francois","family":"Eyskens","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Corrie","family":"Timmer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nicky","family":"Mumford","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J\u00f6rgen","family":"Bierau","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peter M.","family":"van Hasselt","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paul","family":"Gissen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Philippe J.","family":"Goyens","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Patrick J.","family":"McKiernan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gisela","family":"Wilcox","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrew A. 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All patients and\/or parents gave informed consent to participate in this study.","order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval and consent to participate"}},{"value":"Not applicable.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}},{"value":"R. Jahja has received honoraria as a speaker and consultant for Merck Serono.A. Daly has received grants\/honoraria for research, teaching and courses from Nutricia Ltd., Vitaflo Int., First play dietary foods and Mavalia.A. MacDonald has received research funding and honoraria from Nutricia, Vitaflo, Biomarin and Lactalis International. She is a member of the following advisory boards: European Nutritionist Expert Panel (Biomarin), Sapropterin Advisory Board (Biomarin), ELEMENT (Danone-Nutricia), and Arla and Applied Pharma Research.P.J. McKiernan and A.A. Morris have contributed to advisory boards for SOBI.P. Gissen has previously performed consultancy work for SOBI but not in the past three years.C. Timmer has previously followed training that was (partly) payed for by Vitaflo and Nutricia, but not in the past three years.G. Wilcox has received travel grants from Genzyme, Biomarin, Alexion, Amicus and Shire, speaker honoraria from Vitaflo, Biomarin, Shire and Nutricia, research grants from the MPS society (UK), and advisory board membership with Biomarin, Medical Advisory Panel membership for the National Society for PKU (NSPKU) and consultancies for Dimension Therapeutics (Ultragenyx).S.C.J Huijbregts has participated in strategic advisory boards and received honoraria as a consultant and\/or speaker for Merck Serono SA, Biomarin and Nutricia.F.J. van Spronsen has received research grants, advisory board fees, and speaker\u2019s honoraria from Nutricia Research, Merck-Serono, Biomarin, Codexis, Alexion, Vitaflo, MendeliKABS, Promethera, SOBI, APR, and ARLA Foods Int.All other authors did not report any no conflicts of interest.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"285"}}