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Implementing a Transition Program from Paediatric to Adult Services in Phenylketonuria: Results after Two Years of Follow-Up with an Adult Team

We aimed to report the implementation of a phenylketonuria (PKU) transition program and study the effects of follow-up with an adult team on metabolic control, adherence, and loss of follow-up. Fifty-five PKU patients were analysed in the study periods (SP): 2 years before (SP1) and after the beginn...

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Autores principales: Peres, Maria, Almeida, Manuela F., Pinto, Élia J., Carmona, Carla, Rocha, Sara, Guimas, Arlindo, Ribeiro, Rosa, Martins, Esmeralda, Bandeira, Anabela, MacDonald, Anita, Rocha, Júlio C.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8001271/
https://www.ncbi.nlm.nih.gov/pubmed/33671057
http://dx.doi.org/10.3390/nu13030799
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author Peres, Maria
Almeida, Manuela F.
Pinto, Élia J.
Carmona, Carla
Rocha, Sara
Guimas, Arlindo
Ribeiro, Rosa
Martins, Esmeralda
Bandeira, Anabela
MacDonald, Anita
Rocha, Júlio C.
author_facet Peres, Maria
Almeida, Manuela F.
Pinto, Élia J.
Carmona, Carla
Rocha, Sara
Guimas, Arlindo
Ribeiro, Rosa
Martins, Esmeralda
Bandeira, Anabela
MacDonald, Anita
Rocha, Júlio C.
author_sort Peres, Maria
collection PubMed
description We aimed to report the implementation of a phenylketonuria (PKU) transition program and study the effects of follow-up with an adult team on metabolic control, adherence, and loss of follow-up. Fifty-five PKU patients were analysed in the study periods (SP): 2 years before (SP1) and after the beginning of adult care (SP2). Retrospective data on metabolic control and number of clinic appointments were collected for each SP, and protein intakes were analysed. In SP2, three patients (6%) were lost to follow-up. There was a small but statistically significant increase in median number of annual blood spots from SP1 to SP2: 11 (7–15) vs. 14 (7–20); p = 0.002. Mean ± SD of median blood Phe remained stable (525 ± 248 µmol/L vs. 552 ± 225 µmol/L; p = 0.100); median % of blood Phe < 480 µmol/L decreased (51 (4–96)% vs. 37 (5–85)%; p = 0.041) and median number of clinic appointments increased from SP1 to SP2: (5 (4–6) vs. 11 (8–13); p < 0.001). No significant differences were found regarding any parameter of protein intake. Our results suggest that the implementation of an adult service was successful as impact on metabolic control was limited and attendance remained high. Continuous dietetic care likely contributed to these results by keeping patients in follow-up and committed to treatment.
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spelling pubmed-80012712021-03-28 Implementing a Transition Program from Paediatric to Adult Services in Phenylketonuria: Results after Two Years of Follow-Up with an Adult Team Peres, Maria Almeida, Manuela F. Pinto, Élia J. Carmona, Carla Rocha, Sara Guimas, Arlindo Ribeiro, Rosa Martins, Esmeralda Bandeira, Anabela MacDonald, Anita Rocha, Júlio C. Nutrients Article We aimed to report the implementation of a phenylketonuria (PKU) transition program and study the effects of follow-up with an adult team on metabolic control, adherence, and loss of follow-up. Fifty-five PKU patients were analysed in the study periods (SP): 2 years before (SP1) and after the beginning of adult care (SP2). Retrospective data on metabolic control and number of clinic appointments were collected for each SP, and protein intakes were analysed. In SP2, three patients (6%) were lost to follow-up. There was a small but statistically significant increase in median number of annual blood spots from SP1 to SP2: 11 (7–15) vs. 14 (7–20); p = 0.002. Mean ± SD of median blood Phe remained stable (525 ± 248 µmol/L vs. 552 ± 225 µmol/L; p = 0.100); median % of blood Phe < 480 µmol/L decreased (51 (4–96)% vs. 37 (5–85)%; p = 0.041) and median number of clinic appointments increased from SP1 to SP2: (5 (4–6) vs. 11 (8–13); p < 0.001). No significant differences were found regarding any parameter of protein intake. Our results suggest that the implementation of an adult service was successful as impact on metabolic control was limited and attendance remained high. Continuous dietetic care likely contributed to these results by keeping patients in follow-up and committed to treatment. MDPI 2021-02-28 /pmc/articles/PMC8001271/ /pubmed/33671057 http://dx.doi.org/10.3390/nu13030799 Text en © 2021 by the authors. https://creativecommons.org/licenses/by/4.0/Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (http://creativecommons.org/licenses/by/4.0/ (https://creativecommons.org/licenses/by/4.0/) ).
spellingShingle Article
Peres, Maria
Almeida, Manuela F.
Pinto, Élia J.
Carmona, Carla
Rocha, Sara
Guimas, Arlindo
Ribeiro, Rosa
Martins, Esmeralda
Bandeira, Anabela
MacDonald, Anita
Rocha, Júlio C.
Implementing a Transition Program from Paediatric to Adult Services in Phenylketonuria: Results after Two Years of Follow-Up with an Adult Team
title Implementing a Transition Program from Paediatric to Adult Services in Phenylketonuria: Results after Two Years of Follow-Up with an Adult Team
title_full Implementing a Transition Program from Paediatric to Adult Services in Phenylketonuria: Results after Two Years of Follow-Up with an Adult Team
title_fullStr Implementing a Transition Program from Paediatric to Adult Services in Phenylketonuria: Results after Two Years of Follow-Up with an Adult Team
title_full_unstemmed Implementing a Transition Program from Paediatric to Adult Services in Phenylketonuria: Results after Two Years of Follow-Up with an Adult Team
title_short Implementing a Transition Program from Paediatric to Adult Services in Phenylketonuria: Results after Two Years of Follow-Up with an Adult Team
title_sort implementing a transition program from paediatric to adult services in phenylketonuria: results after two years of follow-up with an adult team
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8001271/
https://www.ncbi.nlm.nih.gov/pubmed/33671057
http://dx.doi.org/10.3390/nu13030799
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