vendex and Mucopolysaccharidosis-I

To determine muscular strength differences in patients with MPS-I, II, and VI versus age- and sex-matched healthy controls.. Dominant leg isokinetic knee extension strength was measured at 90 and 120 degrees per second (d/s) using a dynamometer in 30 subjects with MPS and 42 controls (5-16 yrs). MPS-I was further divided into MPS-IA (attenuated) and MPS-IH (severe). Strength measures analyzed were peak torque (PkT), peak torque per unit body weight (PkT/BW) and per unit lean body mass (PkT/LBM), and average power (AP).. Following adjusting strength measures for age, MPS-IH and MPS-II had significantly lower strength measures for all variables at both angular velocities. MPS-VI had significantly lower PkT, PkT/LBM, and AP compared to controls at 90 and 120d/s. In contrast, MPS-IA was not significantly different from controls for any strength variable at either angular velocity.. The results of this study suggest that decrements in skeletal muscle strength depend on MPS diagnosis and severity of disease. Children with MPS-IH demonstrate the greatest difference in muscular strength compared to healthy controls.

Topics: Adolescent; Case-Control Studies; Child; Child, Preschool; Cohort Studies; Female; Humans; Knee Joint; Male; Mucopolysaccharidosis I; Mucopolysaccharidosis II; Mucopolysaccharidosis VI; Muscle Strength; Muscle, Skeletal; Severity of Illness Index; Torque