Quality of life and adherence to treatment in early-treated Brazilian phenylketonuria pediatric patients
dc.contributor.author | Vieira Neto, Eduardo | pt_BR |
dc.contributor.author | Maia Filho, Heber de Souza | pt_BR |
dc.contributor.author | Monteiro, Cláudia Braga | pt_BR |
dc.contributor.author | Carvalho, Lilian Mattos | pt_BR |
dc.contributor.author | Tonon, Tássia | pt_BR |
dc.contributor.author | Vanz, Ana Paula | pt_BR |
dc.contributor.author | Schwartz, Ida Vanessa Doederlein | pt_BR |
dc.contributor.author | Ribeiro, Márcia Gonçalves | pt_BR |
dc.date.accessioned | 2018-10-23T02:41:31Z | pt_BR |
dc.date.issued | 2018 | pt_BR |
dc.identifier.issn | 0100-879X | pt_BR |
dc.identifier.uri | http://hdl.handle.net/10183/183909 | pt_BR |
dc.description.abstract | Early dietary treatment of phenylketonuria (PKU), an inborn error of phenylalanine (Phe) metabolism, results in normal cognitive development. Although health-related quality of life (HRQoL) of PKU patients has been reported as unaffected in high-income countries, there are scarce data concerning HRQoL and adherence to treatment of PKU children and adolescents from Brazil. The present study compared HRQoL scores in core dimensions of Brazilian early-treated PKU pediatric patients with those of a reference population, and explored possible relationships between adherence to treatment and HRQoL. Early-treated PKU pediatric patient HRQoL was evaluated by self- and parent-proxy reports of the Pediatric Quality of Life Inventory (PedsQL) core scales. Adherence to treatment was evaluated by median Phe levels and percentage of results within the therapeutic target range in two periods. Means for total and core scales scores of PedsQL self- and parent proxy-reports of PKU patients were significantly lower than their respective means for controls. Adequacy of median Phe concentrations and the mean percentage of values in the target range fell substantially from the first year of life to the last year of this study. There was no significant difference in mean total and core scale scores for self- and parent proxy-reports between patients with adequate and those with inadequate median Phe concentrations. The harmful consequences for intellectual capacity caused by poor adherence to dietary treatment could explain the observed decrease in all HRQoL scales, especially in school functioning. Healthcare system and financial difficulties may also have influenced negatively all HRQoL dimensions. | en |
dc.format.mimetype | application/pdf | pt_BR |
dc.language.iso | eng | pt_BR |
dc.relation.ispartof | Brazilian journal of medical and biological research = Revista brasileira de pesquisas médicas e biológicas. Ribeirão Preto. Vol. 51, no. 2 (Feb. 2018), p. 1-10 | pt_BR |
dc.rights | Open Access | en |
dc.subject | Fenilcetonuria | pt_BR |
dc.subject | Phenylketonuria | en |
dc.subject | Fenilalanina | pt_BR |
dc.subject | Quality of life | en |
dc.subject | Metabolismo | pt_BR |
dc.subject | Questionnaires | en |
dc.subject | Patient compliance | en |
dc.subject | Diet therapy | en |
dc.title | Quality of life and adherence to treatment in early-treated Brazilian phenylketonuria pediatric patients | pt_BR |
dc.type | Artigo de periódico | pt_BR |
dc.identifier.nrb | 001073081 | pt_BR |
dc.type.origin | Nacional | pt_BR |
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