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Long-term benefits of physical activity in adult patients with late onset Pompe disease: a retrospective cohort study with 10 years of follow-up.
Ismailova, Gamida; Wagenmakers, Margreet A E M; Brusse, Esther; van der Ploeg, Ans T; Favejee, Marein M; van der Beek, Nadine A M E; van den Berg, Linda E M.
Afiliación
  • Ismailova G; Department of Pediatrics, Center for Lysosomal and Metabolic Diseases, Erasmus Medical Center, Sophia Children's Hospital, Mailbox 2060, 3000 CB, Rotterdam, The Netherlands.
  • Wagenmakers MAEM; Department of Internal Medicine, Center for Lysosomal and Metabolic Diseases, Erasmus Medical Center, Mailbox 2040, 3000 CA, Rotterdam, The Netherlands.
  • Brusse E; Department of Neurology, Center for Lysosomal and Metabolic Diseases, Erasmus Medical Center, Mailbox 2040, 3000 CA, Rotterdam, The Netherlands.
  • van der Ploeg AT; Department of Pediatrics, Center for Lysosomal and Metabolic Diseases, Erasmus Medical Center, Sophia Children's Hospital, Mailbox 2060, 3000 CB, Rotterdam, The Netherlands.
  • Favejee MM; Department of Physical Therapy, Erasmus Medical Center, Mailbox 2040, 3000 CA, Rotterdam, The Netherlands.
  • van der Beek NAME; Department of Neurology, Center for Lysosomal and Metabolic Diseases, Erasmus Medical Center, Mailbox 2040, 3000 CA, Rotterdam, The Netherlands.
  • van den Berg LEM; Department of Orthopedics and Sports Medicine, Center for Lysosomal and Metabolic Diseases, Erasmus Medical Center, Sophia Children's Hospital, Mailbox 2060, 3000 CB, Rotterdam, The Netherlands. l.e.m.vandenberg@erasmusmc.nl.
Orphanet J Rare Dis ; 18(1): 319, 2023 10 11.
Article en En | MEDLINE | ID: mdl-37821981
BACKGROUND: In 2011 a 12 weeks personalized exercise training program in 23 mildly affected adult late onset Pompe patients (age 19.6-70.5 years) improved endurance, muscle strength and function. Data on long-term effects of this program or of other physical activity in Pompe disease are absent. This retrospective cohort study aimed to explore effects of long-term healthy physical activity according to the WHO norm and the former exercise training program on the disease course. RESULTS: A total of 29 adult late onset Pompe patients were included: 19 former exercise training program participants and 10 comparable control patients. Patients, who based on interviews, met the 2010 WHO healthy physical activity norm (active, n = 16) performed better on endurance (maximal cardiopulmonary exercise test), muscle strength and function compared to patients not meeting this norm (inactive, n = 13) (p < 0.05). Majority of the outcomes, including endurance and manually tested muscle strength, tended to be higher in the active patients of the 2011 training cohort who continued the program compared to active control patients (p > 0.05). CONCLUSION: In Pompe disease long-term healthy physical activity according to the 2010 WHO norm leads to physical benefits and a personalized exercise training program may have additional favorable effects and both should be recommended as standard of care.
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Enfermedad del Almacenamiento de Glucógeno Tipo II / Terapia por Ejercicio Tipo de estudio: Etiology_studies / Observational_studies Límite: Adult / Aged / Humans / Middle aged Idioma: En Revista: Orphanet J Rare Dis Asunto de la revista: MEDICINA Año: 2023 Tipo del documento: Article País de afiliación: Países Bajos Pais de publicación: Reino Unido
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Texto completo: 1 Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Enfermedad del Almacenamiento de Glucógeno Tipo II / Terapia por Ejercicio Tipo de estudio: Etiology_studies / Observational_studies Límite: Adult / Aged / Humans / Middle aged Idioma: En Revista: Orphanet J Rare Dis Asunto de la revista: MEDICINA Año: 2023 Tipo del documento: Article País de afiliación: Países Bajos Pais de publicación: Reino Unido