Bone Abstracts

In children with spinal muscular atrophy (SMA): i) describe bone mineral density (BMD) measured by dual-energy x-ray absorptiometry (DXA); ii) examine bone health factors; iii) examine change in BMD over time and pamidronate effect.DXA results, medications, ambulation, 25-OH-Vit D levels, and fracture history were retrospectively reviewed in 14 children (five girls) with SMA. Sites scanned included whole body (WB), lumbar spine (LS), and lateral distal f...

Objective: In girls with Rett syndromeDescribe bone mineral density (BMD) and contributing factors in a cross-section of subjects.Examine serial DXA measures.Examine effect of pamidronate on BMD over time.Methods: We reviewed the clinical course, medications, level of ambulation, 25-OH-Vit D levels, fracture history and DXA results in 13 girls with Rett syndrome. Eight subjects had more...

Objectives: i) Describe bone mineral density (BMD) of children with MPS IV (Morquio disease), a rare genetic disorder which produces skeletal deformity, small stature and results in physical limitations such as the ability to walk.ii) Examine fracture history and factors affecting bone health in Morquio.iii) Describe technical issues encountered in assessing BMD by DXA in Morquio.Methods: In this prospective ...

Objectives: Examine the effects of a ramped dosage schedule of pamidronate on BMD, fracture rate and location compared to a uniform 5-course regimen. The ramping regimen is intended to alter the tendency for post-treatment fractures to occur at the juncture of pamidronate bands where stress-riser related fractures have been described.Methods: Ten non-ambulatory children (seven females) with neuromuscular disabilities who received IV pamidronate with a ta...