Exercise stress testing in children with metabolic or neuromuscular disorders

Tim Takken, Wim G Groen, Erik H Hulzebos, Cornelia G Ernsting, Peter M van Hasselt, Berthil H Prinsen, Paul J Helders, Gepke Visser, Tim Takken, Wim G Groen, Erik H Hulzebos, Cornelia G Ernsting, Peter M van Hasselt, Berthil H Prinsen, Paul J Helders, Gepke Visser

Abstract

The role of exercise as a diagnostic or therapeutic tool in patients with a metabolic disease (MD) or neuromuscular disorder (NMD) is relatively underresearched. In this paper we describe the metabolic profiles during exercise in 13 children (9 boys, 4 girls, age 5-15 yrs) with a diagnosed MD or NMD. Graded cardiopulmonary exercise tests and/or a 90-min prolonged submaximal exercise test were performed. During exercise, respiratory gas-exchange and heart rate were monitored; blood and urine samples were collected for biochemical analysis at set time points. Several characteristics in our patient group were observed, which reflected the differences in pathophysiology of the various disorders. Metabolic profiles during exercises CPET and PXT seem helpful in the evaluation of patients with a MD or NMD.

References

    1. Brooke MH, Carroll JE, Davis JE, Hagberg JM. The prolonged exercise test. Neurology. 1979;29(5):636–643.
    1. Carroll JE, Hagberg JM, Brooke MH, Shumate JB. Bicycle ergometry and gas exchange measurements in neuromuscular diseases. Archives of Neurology. 1979;36(8):457–461.
    1. Sockolov R, Irwin B, Dressendorfer RH, Bernauer EM. Exercise performance in 6 to 11 year old boys with Duchenne muscular dystrophy. Archives of Physical Medicine and Rehabilitation. 1977;58(5):195–201.
    1. Lewis SF, Haller RG. Skeletal muscle disorders and associated factors that limit exercise performance. Exercise and Sport Sciences Reviews. 1989;17:67–113.
    1. Haller RG, Lewis SF. Pathophysiology of exercise performance in muscle disease. Medicine and Science in Sports and Exercise. 1984;16(5):456–459.
    1. Flaherty KR, Weisman IM, Zeballos J, Martinez FJ. The role of cardiopulmonary exercise testing for patients with suspected metabolic myopathies and other neuromuscular disorders. Progress in Respiratory Research. 2002;32:242–253.
    1. Krivickas LS. Exercise in neuromuscular disease. Journal of Clinical Neuromuscular Disease. 2003;5(1):29–39.
    1. Takken T, Custers JWH, Visser G, Dorland L, Helders PJM, De Koning TJ. Prolonged exercise testing in two children with a mild Multiple Acyl-CoA-Dehydrogenase deficiency. Nutrition and Metabolism. 2005;2, article 12
    1. Godfrey S. Exercise Testing in Children. London, UK: W.B. Saunders; 1974.
    1. Binkhorst RA, van 't Hof MA, Saris WHM. Maximale Inspanning door Kinderen; Referentiewaarden voor 6–18 Jarige Meisjes en Jongens. Den-Haag, The Netherlands: Nederlandse Hartstichting; 1992.
    1. Van Leeuwen PB, Van Der Net J, Helders PJM, Takken T. Exercise parameters in healthy Dutch children. Geneeskunde en Sport. 2004;37(5):126–132.
    1. Takken T, Blank AC, Hulzebos EH, Van Brussel M, Groen WG, Helders PJ. Cardiopulmonary exercise testing in congenital heart disease: (contra)indications and interpretation. Netherlands Heart Journal. 2009;17(10):385–392.
    1. Eschenbacher WL, Mannina A. An algorithm for the interpretation of cardiopulmonary exercise tests. Chest. 1990;97(2):263–267.
    1. Strong WB, Malina RM, Blimkie CJR, et al. Evidence based physical activity for school-age youth. Journal of Pediatrics. 2005;146(6):732–737.
    1. Vissing J, Haller RG. A diagnostic cycle test for McArdle’s disease. Annals of Neurology. 2003;54(4):539–542.
    1. Pérez M, Ruiz JR, Fernández del Valle M, et al. The second wind phenomenon in very young McArdle’s patients. Neuromuscular Disorders. 2009;19(6):403–405.

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