Expert Consensus on the Long-Term Effectiveness of Medical Nutrition Therapy and Its Impact on the Outcomes of Adults with Phenylketonuria
| Autor(a) principal: | |
|---|---|
| Data de Publicação: | 2023 |
| Outros Autores: | , , , , , , , , , , |
| Tipo de documento: | Artigo |
| Idioma: | eng |
| Título da fonte: | Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
| Texto Completo: | http://hdl.handle.net/10362/158695 |
Resumo: | Funding Information: The virtual meetings leading up to this manuscript as well as assistance in development of the current manuscript were supported by BioMarin Europe Ltd. and BioMarin Pharmaceutical Inc., but without involvement in the modified Delphi process leading up to the consensus statements. Publisher Copyright: © 2023 by the authors. |
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Expert Consensus on the Long-Term Effectiveness of Medical Nutrition Therapy and Its Impact on the Outcomes of Adults with Phenylketonuriaadultcomorbiditiesconsensus recommendationslow Phe dietmedical nutrition therapymodified DelphiphenylketonuriaPKUFood ScienceNutrition and DieteticsFunding Information: The virtual meetings leading up to this manuscript as well as assistance in development of the current manuscript were supported by BioMarin Europe Ltd. and BioMarin Pharmaceutical Inc., but without involvement in the modified Delphi process leading up to the consensus statements. Publisher Copyright: © 2023 by the authors.Many adults with phenylketonuria (PKU) rely on medical nutrition therapy (MNT; low phenylalanine (Phe) diet with protein substitutes/medical foods) to maintain blood Phe concentrations within recommended ranges and prevent PKU-associated comorbidities. Despite disease detection through newborn screening and introduction of MNT as early as birth, adherence to MNT often deteriorates from childhood onwards, complicating the assessment of its effectiveness in the long term. Via a modified Delphi process, consensus (≥70% agreement) was sought on 19 statements among an international, multidisciplinary 13-member expert panel. After three iterative voting rounds, the panel achieved consensus on 17 statements related to the limitations of the long-term effectiveness of MNT (7), the burden of long-term reliance on MNT (4), and its potential long-term detrimental health effects (6). According to the expert panel, the effectiveness of MNT is limited in the long term, is associated with a high treatment burden, and demonstrates that adults with PKU are often unable to achieve metabolic control through dietary management alone, creating an unmet need in the adult PKU population.NOVA Medical School|Faculdade de Ciências Médicas (NMS|FCM)Centro de Investigação em Tecnologias e Serviços de Saúde (CINTESIS)RUNRocha, Júlio CésarAhring, Kirsten K.Bausell, HeatherBilder, Deborah A.Harding, Cary O.Inwood, AnitaLongo, NicolaMuntau, Ania C.Pessoa, André L.SantosRohr, FranSivri, SerapHermida, Álvaro2023-10-04T22:19:05Z2023-092023-09-01T00:00:00Zinfo:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/articleapplication/pdfhttp://hdl.handle.net/10362/158695eng1422-8599PURE: 73136297https://doi.org/10.3390/nu15183940info:eu-repo/semantics/openAccessreponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãoinstacron:RCAAP2024-03-11T05:41:15Zoai:run.unl.pt:10362/158695Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireopendoar:71602024-03-20T03:57:16.700005Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãofalse |
| dc.title.none.fl_str_mv |
Expert Consensus on the Long-Term Effectiveness of Medical Nutrition Therapy and Its Impact on the Outcomes of Adults with Phenylketonuria |
| title |
Expert Consensus on the Long-Term Effectiveness of Medical Nutrition Therapy and Its Impact on the Outcomes of Adults with Phenylketonuria |
| spellingShingle |
Expert Consensus on the Long-Term Effectiveness of Medical Nutrition Therapy and Its Impact on the Outcomes of Adults with Phenylketonuria Rocha, Júlio César adult comorbidities consensus recommendations low Phe diet medical nutrition therapy modified Delphi phenylketonuria PKU Food Science Nutrition and Dietetics |
| title_short |
Expert Consensus on the Long-Term Effectiveness of Medical Nutrition Therapy and Its Impact on the Outcomes of Adults with Phenylketonuria |
| title_full |
Expert Consensus on the Long-Term Effectiveness of Medical Nutrition Therapy and Its Impact on the Outcomes of Adults with Phenylketonuria |
| title_fullStr |
Expert Consensus on the Long-Term Effectiveness of Medical Nutrition Therapy and Its Impact on the Outcomes of Adults with Phenylketonuria |
| title_full_unstemmed |
Expert Consensus on the Long-Term Effectiveness of Medical Nutrition Therapy and Its Impact on the Outcomes of Adults with Phenylketonuria |
| title_sort |
Expert Consensus on the Long-Term Effectiveness of Medical Nutrition Therapy and Its Impact on the Outcomes of Adults with Phenylketonuria |
| author |
Rocha, Júlio César |
| author_facet |
Rocha, Júlio César Ahring, Kirsten K. Bausell, Heather Bilder, Deborah A. Harding, Cary O. Inwood, Anita Longo, Nicola Muntau, Ania C. Pessoa, André L.Santos Rohr, Fran Sivri, Serap Hermida, Álvaro |
| author_role |
author |
| author2 |
Ahring, Kirsten K. Bausell, Heather Bilder, Deborah A. Harding, Cary O. Inwood, Anita Longo, Nicola Muntau, Ania C. Pessoa, André L.Santos Rohr, Fran Sivri, Serap Hermida, Álvaro |
| author2_role |
author author author author author author author author author author author |
| dc.contributor.none.fl_str_mv |
NOVA Medical School|Faculdade de Ciências Médicas (NMS|FCM) Centro de Investigação em Tecnologias e Serviços de Saúde (CINTESIS) RUN |
| dc.contributor.author.fl_str_mv |
Rocha, Júlio César Ahring, Kirsten K. Bausell, Heather Bilder, Deborah A. Harding, Cary O. Inwood, Anita Longo, Nicola Muntau, Ania C. Pessoa, André L.Santos Rohr, Fran Sivri, Serap Hermida, Álvaro |
| dc.subject.por.fl_str_mv |
adult comorbidities consensus recommendations low Phe diet medical nutrition therapy modified Delphi phenylketonuria PKU Food Science Nutrition and Dietetics |
| topic |
adult comorbidities consensus recommendations low Phe diet medical nutrition therapy modified Delphi phenylketonuria PKU Food Science Nutrition and Dietetics |
| description |
Funding Information: The virtual meetings leading up to this manuscript as well as assistance in development of the current manuscript were supported by BioMarin Europe Ltd. and BioMarin Pharmaceutical Inc., but without involvement in the modified Delphi process leading up to the consensus statements. Publisher Copyright: © 2023 by the authors. |
| publishDate |
2023 |
| dc.date.none.fl_str_mv |
2023-10-04T22:19:05Z 2023-09 2023-09-01T00:00:00Z |
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info:eu-repo/semantics/publishedVersion |
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info:eu-repo/semantics/article |
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article |
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publishedVersion |
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http://hdl.handle.net/10362/158695 |
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http://hdl.handle.net/10362/158695 |
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eng |
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eng |
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1422-8599 PURE: 73136297 https://doi.org/10.3390/nu15183940 |
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info:eu-repo/semantics/openAccess |
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openAccess |
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application/pdf |
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Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação |
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