Bisphosphonates have been widely administered to children with OI based on observational trials. A randomized controlled trial of q3m intravenous pamidronate in children with types III and IV OI yielded positive vertebral changes in DXA and geometry after 1 year of treatment, but no further significant improvement during extended treatment. The treated group did not experience significantly decreased pain or long bone fractures or have increased motor function or muscle strength.Introduction: Bisphosphonates, antiresorptive drugs for osteoporosis, are widely administered to children with osteogenesis imperfecta (OI). Uncontrolled pamidronate trials in OI reported increased BMD, vertebral coronal area, and mobility, and decreased pain. We conducted a randomized controlled trial of pamidronate in children with types III and IV OI. Materials and Methods: This randomized trial included 18 children (4-13 years of age) with types III and IV OI. The first study year was controlled; 9 children received pamidronate (10 mg/m
The purpose of this study was to evaluate the effects of an 8‐week program of hippotherapy on energy expenditure during walking; on the gait dimensions of stride length, velocity, and cadence; and on performance on the Gross Motor Function Measure (GMFM) in five children with spastic cerebral palsy (CP). A repeated‐measures within‐subjects design was used consisting of two baseline measurements taken 8 weeks apart, followed by an 8‐week intervention period, then a posttest. After hippotherapy, all five children showed a significant decrease (Xr2;=7.6, P<0.05) in energy expenditure during walking and a significant increase (Xr2=7.6, P<0.05) in scores on Dimension E (Walking, Running, and Jumping) of the GMFM. A trend toward increased stride length and decreased cadence was observed. This study suggests that hippotherapy may improve energy expenditure during walking and gross motor function in children with CP.
Objective
To describe the distribution and severity of muscle weakness using manual muscle testing (MMT) in 172 patients with polymyositis (PM), dermatomyositis (DM) and juvenile dermatomyositis (JDM). The secondary objectives included characterizing individual muscle group weakness and determining associations of weakness with functional status and myositis characteristics in this large cohort of patients with myositis.
Methods
Strength was assessed for 13 muscle groups using the 10-point MMT and expressed as a total score, subscores based on functional and anatomical regions, and grades for individual muscle groups. Patient characteristics and secondary outcomes such as clinical course, muscle enzymes, corticosteroid dosage, and functional status were evaluated for association with strength using univariate and multivariate analyses.
Results
A gradient of proximal weakness was seen, with PM weakest, DM intermediate and JDM strongest among the three myositis clinical groups (p ≤ 0.05). Hip flexors, hip extensors, hip abductors, neck flexors, and shoulder abductors were the muscle groups with the greatest weakness among all three clinical groups. Muscle groups were affected symmetrically.
Conclusions
Axial and proximal muscle impairment was reflected in the five weakest muscles shared by our cohort of myositis patients. However, differences in the pattern of weakness were observed among all three clinical groups. Our findings suggest a greater severity of proximal weakness in PM in comparison to DM.
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