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

Terzis, Gerasimos; Dimopoulos, Filippos; Papadimas, George K.; Papadopoulos, Constantinos; Spengos, Konstantinos; Fatouros, Ioannis G.; Kavouras, Stavros A.; Manta, Panagiota

doi:10.1016/j.ymgme.2011.05.013

Cited by 49 publications

(38 citation statements)

References 17 publications

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“…There was a significant increase in muscle strength of 15-50%, based on the particular muscle group and better performance on the 6MWT, from 203.8 to 248 meters. 167 However, changes in muscle strength did not correlate with performance of the 6MWT, and there was no change in body composition. Therefore, this functional benefit could have been due to respiratory or cardiac improvement.…”

Section: Overview Of Exercise Studies In Nmdmentioning

confidence: 86%

Exercise in neuromuscular disease

Anziska

Sternberg

2013

Muscle and Nerve

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In this review, we present an overview of the role of exercise in neuromuscular disease (NMD). We demonstrate that despite the different pathologies in NMDs, exercise is beneficial, whether aerobic/endurance or strength/resistive training, and we explore whether this benefit has a similar mechanism to that of healthy subjects. We discuss further areas for study, incorporating imaginative and novel approaches to training and its assessment in NMD. We conclude by suggesting ways to improve future trials by avoiding previous methodological flaws and drawbacks in this field.

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Section: Overview Of Exercise Studies In Nmdmentioning

confidence: 86%

Exercise in neuromuscular disease

Anziska

Sternberg

2013

Muscle and Nerve

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“…Thus, it cannot be excluded that the beneficial effects described in the present study after ERT can be attributed, at least in part, to the increased level of physical activity. Recently, evidence has been provided that ERT and exercise training could have additive positive effects on these patients’ exercise tolerance and, ultimately, on their quality of life [2].…”

Section: Discussionmentioning

confidence: 99%

“…Until recently, no effective therapy for GSDII patients was available, even if physical activity alone [2] or in parallel with an high-protein and low-carbohydrate dietary regime has been demonstrated to improve quality of life and motor function [3]. Enzyme replacement therapy (ERT) with recombinant human α-glucosidase became available in 2000 [4], and currently a number of studies have been published on the efficacy and safety of ERT in GSDII disease.…”

Section: Introductionmentioning

confidence: 99%

Exercise testing in late-onset glycogen storage disease type II patients undergoing enzyme replacement therapy

Marzorati

Porcelli

Bellistri

et al. 2012

Neuromuscular Disorders

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Enzyme replacement therapy (ERT) has recently became available for patients with glycogen storage disease type II. Previous studies have demonstrated clinical efficacy of enzyme replacement therapy, however, data on physiological variables related to exercise tolerance are scarce. Four glycogen storage disease type II late-onset patients (45 ± 6 years) performed an incremental exercise on a cycle ergometer, up to voluntary exhaustion, before (BEFORE) and after 12 months of ERT (AFTER). Peak workload, oxygen uptake, heart rate, cardiac output (by impedance cardiography) and vastus lateralis oxygenation indices (by continuous-wave near-infrared spectroscopy, NIRS) were determined. Peak workload and oxygen uptake values significantly increased during ERT (54 ± 30 vs. 63 ± 31 watt, and 17.2 ± 4.4 vs. 19.7 ± 3.5 ml/kg/min, respectively, in BEFORE vs. AFTER). On the other hand, for both peak cardiac output (12.3 ± 5.3 vs. 14.8 ± 4.5 L/min) and the NIRS-determined peak skeletal muscle fractional O2 extraction, expressed as a percentage of the maximal values during a transient limb ischemia (30 ± 39% vs. 38 ± 28%), the observed increases were not statistically significant. Our findings suggest that in glycogen storage disease type II patients enzyme replacement therapy is associated with a mild improvement of exercise tolerance. The findings need to be validated during a longer follow-up on a larger group of patients.

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“…Another study investigated the effect of exercise training on muscular strength and body composition in five patients with LOPD receiving ERT who underwent a 20-week program of supervised aerobic and progressive resistance exercise training. Exercise training resulted in a significant increase in muscular strength and 6-min walking distance despite no change in total and lower extremity lean body mass, highlighting the benefits of exercise training in patients with LOPD on ERT [ 86 ].…”

Section: Management Of Components Of Lopdmentioning

confidence: 99%

Diagnosis and treatment of late-onset Pompe disease in the Middle East and North Africa region: consensus recommendations from an expert group

et al. 2015

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BackgroundPompe disease is a rare autosomal recessive disorder caused by a deficiency of the lysosomal enzyme alpha-glucosidase responsible for degrading glycogen. Late-onset Pompe disease has a complex multisystem phenotype characterized by a range of symptoms.MethodsAn expert panel from the Middle East and North Africa (MENA) region met to create consensus-based guidelines for the diagnosis and treatment of late-onset Pompe disease for the MENA region, where the relative prevalence of Pompe disease is thought to be high but there is a lack of awareness and diagnostic facilities.ResultsThese guidelines set out practical recommendations and include algorithms for the diagnosis and treatment of late-onset Pompe disease. They detail the ideal diagnostic workup, indicate the patients in whom enzyme replacement therapy should be initiated, and provide guidance on appropriate patient monitoring.ConclusionsThese guidelines will serve to increase awareness of the condition, optimize patient diagnosis and treatment, reduce disease burden, and improve patient outcomes.

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

Cited by 49 publications

References 17 publications

Exercise in neuromuscular disease

Exercise in neuromuscular disease

Exercise testing in late-onset glycogen storage disease type II patients undergoing enzyme replacement therapy

Diagnosis and treatment of late-onset Pompe disease in the Middle East and North Africa region: consensus recommendations from an expert group

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