The aim of this study was to examine muscular strength differences in patients with Mucopolysaccharidosis (MPS) I, II, or VI versus age- and sex-matched healthy controls. Isokinetic knee extension and flexion strength was measured at 90, 120, and 180 degrees/second (d/s) using a dynamometer in 30 subjects with MPS and 42 controls (9-16 years). MPS-IH (neuropathic), MPS-II, and MPS-VI exhibited significant strength decrements when compared to controls. MPS-IA (non-neuropathic) was not significantly different than controls. MPS-IH and MPS-II had significantly lower dominant leg extension average peak torque (DLEAPT) than controls at 90, 120, and 180d/s. MPS-VI had significantly lower DLEAPT than controls at 90 and 120d/s. At 180d/s, MPS-IH DLEAPT was significantly lower than that of MPS-IA. MPS-IA DLEAPT was not significantly different than controls at any angular velocity. Children with MPS-IH, II and VI have decreased skeletal muscle strength compared to children with MPS-IA.
University of Miinesota M.S. thesis. May 2013. Major: Kinesiology. Advisor: Donald R. Dengel, Ph.D. 1 computer file (PDF); v, 42 pages, appendix p. 36-42.
Taylor, Natalie Ebsch.
Isokinetic strength differences in patients with mucopolysaccharidosis I, II, and VI.
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