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Impact on Diet Quality and Burden of Care in Sapropterin Dihydrochloride Use in Children with Phenylketonuria: A 6 Month Follow-Up Report.
Gama, Maria Inês; Daly, Anne; Ashmore, Catherine; Evans, Sharon; Moreira-Rosário, André; Rocha, Júlio César; MacDonald, Anita.
Afiliación
  • Gama MI; Birmingham Children's Hospital, Steelhouse Lane, Birmingham B4 6NH, UK.
  • Daly A; Nutrition and Metabolism, NOVA Medical School, Faculdade de Ciências Médicas da Universidade NOVA de Lisboa, Campo dos Mártires da Pátria 130, 1169-056 Lisboa, Portugal.
  • Ashmore C; Birmingham Children's Hospital, Steelhouse Lane, Birmingham B4 6NH, UK.
  • Evans S; Birmingham Children's Hospital, Steelhouse Lane, Birmingham B4 6NH, UK.
  • Moreira-Rosário A; Birmingham Children's Hospital, Steelhouse Lane, Birmingham B4 6NH, UK.
  • Rocha JC; Nutrition and Metabolism, NOVA Medical School, Faculdade de Ciências Médicas da Universidade NOVA de Lisboa, Campo dos Mártires da Pátria 130, 1169-056 Lisboa, Portugal.
  • MacDonald A; CINTESIS@RISE, Nutrition and Metabolism, NOVA Medical School, Faculdade de Ciências Médicas, NMS, FCM, Universidade NOVA de Lisboa, 1169-056 Lisboa, Portugal.
Nutrients ; 15(16)2023 Aug 17.
Article en En | MEDLINE | ID: mdl-37630793
INTRODUCTION: In phenylketonuria (PKU) changes in dietary patterns and behaviors in sapropterin-responsive populations have not been widely reported. We aimed to assess changes in food quality, mental health and burden of care in a paediatric PKU sapropterin-responsive cohort. METHODS: In an observational, longitudinal study, patient questionnaires on food frequency, neophobia, anxiety and depression, impact on family and burden of care were applied at baseline, 3 and 6-months post successful sapropterin-responsiveness testing (defined as a 30% reduction in blood phenylalanine levels). RESULTS: 17 children (10.8 ± 4.2 years) completed 6-months follow-up. Patients body mass index (BMI) z-scores remained unchanged after sapropterin initiation. Blood phenylalanine was stable. Natural protein increased (p < 0.001) and protein substitute intake decreased (p = 0.002). There were increases in regular cow's milk (p = 0.001), meat/fish, eggs (p = 0.005), bread (p = 0.01) and pasta (p = 0.011) intakes but special low-protein foods intake decreased. Anxiety (p = 0.016) and depression (p = 0.022) decreased in caregivers. The impact-on-family, familial-social impact (p = 0.002) and personal strain (p = 0.001) lessened. After sapropterin, caregivers spent less time on PKU tasks, the majority ate meals outside the home more regularly and fewer caregivers had to deny food choices to their children. CONCLUSION: There were significant positive changes in food patterns, behaviors and burden of care in children with PKU and their families after 6-months on sapropterin treatment.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Fenilcetonurias / Dieta Tipo de estudio: Observational_studies / Prognostic_studies Límite: Animals Idioma: En Revista: Nutrients Año: 2023 Tipo del documento: Article Pais de publicación: Suiza
Texto completo
Añadir a Mi BVS
Imprimir
XML
PubMed Links
Buscar en Google

Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Fenilcetonurias / Dieta Tipo de estudio: Observational_studies / Prognostic_studies Límite: Animals Idioma: En Revista: Nutrients Año: 2023 Tipo del documento: Article Pais de publicación: Suiza