scholarly journals Effects of Exercise Training on Functional Capacity and Quality of Life in a Patient with Late-Onset Pompe Disease Receiving Enzyme Replacement Therapy

2015 ◽  
Vol 2 (s1) ◽  
pp. S33-S33
Author(s):  
Rosângela M. Silva ◽  
Carmen S.C. Mendes ◽  
Carolina C. Aranda ◽  
Marco A. Curiati ◽  
Maret H. Rand ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Exercise Training ◽  
Enzyme Replacement Therapy ◽  
Replacement Therapy ◽  
Functional Capacity ◽  
Pompe Disease ◽  
Late Onset ◽  
Enzyme Replacement

Effect of Aerobic and Resistance Exercise Training on Late-Onset Pompe Disease Patients Receiving Enzyme Replacement Therapy

2011 ◽  
Vol 33 (6) ◽  
pp. S40 ◽  
Author(s):  
Gerasimos Terzis ◽  
Georgios K. Papadimas ◽  
Filippos Dimopoulos ◽  
Costas Papadopoulos ◽  
Konstantinos Spengos ◽  
...  
Keyword(s):  
Exercise Training ◽  
Enzyme Replacement Therapy ◽  
Resistance Exercise ◽  
Replacement Therapy ◽  
Pompe Disease ◽  
Late Onset ◽  
Resistance Exercise Training ◽  
Enzyme Replacement

scholarly journals A Systematic Review and Meta-Analysis of Enzyme Replacement Therapy in Late-Onset Pompe Disease

2021 ◽  
Vol 10 (21) ◽  
pp. 4828
Author(s):  
Alícia Dorneles Dornelles ◽  
Ana Paula Pedroso Junges ◽  
Tiago Veiga Pereira ◽  
Bárbara Corrêa Krug ◽  
Candice Beatriz Treter Gonçalves ◽  
...  
Keyword(s):  
Enzyme Replacement Therapy ◽  
Replacement Therapy ◽  
Pompe Disease ◽  
Late Onset ◽  
Meta Analysis ◽  
Glycogen Storage ◽  
Enzyme Replacement ◽  
Sf 36 ◽  
Glycogen Storage Disorder

Pompe disease (PD) is a glycogen storage disorder caused by deficient activity of acid alpha-glucosidase (GAA). We sought to review the latest available evidence on the safety and efficacy of recombinant human GAA enzyme replacement therapy (ERT) for late-onset PD (LOPD). Methods: We systematically searched the MEDLINE (via PubMed), Embase, and Cochrane databases for prospective clinical studies evaluating ERT for LOPD on pre-specified outcomes. A meta-analysis was also performed. Results: Of 1601 articles identified, 22 were included. Studies were heterogeneous and with very low certainty of evidence for most outcomes. The following outcomes showed improvements associated with GAA ERT, over a mean follow-up of 32.5 months: distance walked in the 6-min walking test (6MWT) (mean change 35.7 m (95% confidence interval [CI] 7.78, 63.75)), physical domain of the SF-36 quality of life (QOL) questionnaire (mean change 1.96 (95% CI 0.33, 3.59)), and time on ventilation (TOV) (mean change −2.64 h (95% CI −5.28, 0.00)). There were no differences between the pre- and post-ERT period for functional vital capacity (FVC), Walton and Gardner-Medwin Scale score, upper-limb strength, or total SF-36 QOL score. Adverse events (AEs) after ERT were mild in most cases. Conclusion: Considering the limitations imposed by the rarity of PD, our data suggest that GAA ERT improves 6MWT, physical QOL, and TOV in LOPD patients. ERT was safe in the studied population. PROSPERO register: 135102.

scholarly journals Quality of life and participation in daily life of adults with Pompe disease receiving enzyme replacement therapy: 10 years of international follow-up

2015 ◽  
Vol 39 (2) ◽  
pp. 253-260 ◽  
Author(s):  
Deniz Güngör ◽  
Michelle E. Kruijshaar ◽  
Iris Plug ◽  
Dimitris Rizopoulos ◽  
Tim A. Kanters ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Enzyme Replacement Therapy ◽  
Replacement Therapy ◽  
Pompe Disease ◽  
Daily Life ◽  
Enzyme Replacement

Effect of aerobic and resistance exercise training on late-onset Pompe disease patients receiving enzyme replacement therapy

2011 ◽  
Vol 104 (3) ◽  
pp. 279-283 ◽  
Author(s):  
Gerasimos Terzis ◽  
Filippos Dimopoulos ◽  
George K. Papadimas ◽  
Constantinos Papadopoulos ◽  
Konstantinos Spengos ◽  
...  
Keyword(s):  
Exercise Training ◽  
Enzyme Replacement Therapy ◽  
Resistance Exercise ◽  
Replacement Therapy ◽  
Pompe Disease ◽  
Late Onset ◽  
Resistance Exercise Training ◽  
Enzyme Replacement

scholarly journals Effects of Aerobic Exercise Training in Late-Onset Pompe Disease Before and 1 Month after Enzyme Replacement Therapy

2015 ◽  
Vol 2 (s1) ◽  
pp. S32-S32
Author(s):  
Rosângela M. Silva ◽  
Carmen S.C. Mendes ◽  
Carolina C. Aranda ◽  
Marco A. Curiati ◽  
Maret H. Rand ◽  
...  
Keyword(s):  
Exercise Training ◽  
Enzyme Replacement Therapy ◽  
Aerobic Exercise ◽  
Replacement Therapy ◽  
Pompe Disease ◽  
Late Onset ◽  
Aerobic Exercise Training ◽  
Enzyme Replacement

Effects of exercise training during infusion on late-onset Pompe disease patients receiving enzyme replacement therapy

2012 ◽  
Vol 107 (4) ◽  
pp. 669-673 ◽  
Author(s):  
Gerasimos Terzis ◽  
Argyro Krase ◽  
Giorgos Papadimas ◽  
Constantinos Papadopoulos ◽  
Stavros A. Kavouras ◽  
...  
Keyword(s):  
Exercise Training ◽  
Enzyme Replacement Therapy ◽  
Replacement Therapy ◽  
Pompe Disease ◽  
Late Onset ◽  
Enzyme Replacement
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