Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2015 Jul 19:10:87.
doi: 10.1186/s13023-015-0303-0.

Safety and efficacy of exercise training in adults with Pompe disease: evalution of endurance, muscle strength and core stability before and after a 12 week training program

Linda E M van den Berg  1 , Marein M Favejee  1   2 , Stephan C A Wens  3 , Michelle E Kruijshaar  1 , Stephan F E Praet  2 , Arnold J J Reuser  4 , Johannes B J Bussmann  2 , Pieter A van Doorn  3 , Ans T van der Ploeg  5
Affiliations

Safety and efficacy of exercise training in adults with Pompe disease: evalution of endurance, muscle strength and core stability before and after a 12 week training program

Linda E M van den Berg et al. Orphanet J Rare Dis. .

Abstract

Background: Pompe disease is a proximal myopathy. We investigated whether exercise training is a safe and useful adjuvant therapy for adult Pompe patients, receiving enzyme replacement therapy.

Methods: Training comprised 36 sessions of standardized aerobic, resistance and core stability exercises over 12 weeks. Before and after, the primary outcome measures safety, endurance (aerobic exercise capacity and distance walked on the 6 min walk test) and muscle strength, and secondary outcome measures core stability, muscle function and body composition, were evaluated.

Results: Of 25 patients enrolled, 23 successfully completed the training. Improvements in endurance were shown by increases in maximum workload capacity (110 W before to 122 W after training, [95 % CI of the difference 6 · 0 to 19 · 7]), maximal oxygen uptake capacity (69 · 4 % and 75 · 9 % of normal, [2 · 5 to 10 · 4]), and maximum walking distance (6 min walk test: 492 meters and 508, [-4 · 4 to 27 · 7] ). There were increases in muscle strength of the hip flexors (156 · 4 N to 180 · 7 N [1 · 6 to 13 · 6) and shoulder abductors (143 · 1 N to 150 · 7 N [13 · 2 to 35 · 2]). As an important finding in secondary outcome measures the number of patients who were able to perform the core stability exercises rose, as did the core stability balancing time (p < 0.05, for all four exercises). Functional tests showed small reductions in the time needed to climb four steps (2 · 4 sec to 2 · 1, [- 0 · 54 to -0 · 04 ]) and rise to standing position (5 · 8 sec to 4 · 8, [-2 · 0 to 0 · 0]), while time to run, the quick motor function test results and body composition remained unchanged.

Conclusions: Our study shows that a combination of aerobic, strength and core stability exercises is feasible, safe and beneficial to adults with Pompe disease.

PubMed Disclaimer

Figures

Fig. 1
Fig. 1
Flowchart for the standardized exercise-training regime combining aerobic, resistance and core stability exercises
Fig. 2
Fig. 2
Time patients (N = 23) were able to remain in balance for the four different core stability exercises before (white bars) and after training (black bars)
See this image and copyright information in PMC

References

    1. Engel AG, Hirschhorn R, Huie MI. Acid maltase deficiency. In: Engel AG, Franzini-Armstrong C, editors. Myology. New York: McGraw-Hill; 2004. pp. 1559–1586.
    1. van der Ploeg AT, Reuser AJ. Pompe's disease. Lancet. 2008;372(9646):1342–53. doi: 10.1016/S0140-6736(08)61555-X. - DOI - PubMed
    1. Engel AG. Acid maltase deficiency in adults: studies in four cases of a syndrome which may mimic muscular dystrophy or other myopathies. Brain. 1970;93(3):599–616. doi: 10.1093/brain/93.3.599. - DOI - PubMed
    1. Laforet P, Nicolino M, Eymard PB, Puech JP, Caillaud C, Poenaru L, et al. Juvenile and adult-onset acid maltase deficiency in France: genotype-phenotype correlation. Neurology. 2000;55(8):1122–8. - PubMed
    1. van der Beek NA, de Vries JM, Hagemans ML, Hop WC, Kroos MA, Wokke JH, et al. Clinical features and predictors for disease natural progression in adults with Pompe disease: a nationwide prospective observational study. Orphanet J Rare Dis. 2012;7:88. - PMC - PubMed

Publication types