Do Bisphosphonates Alter the Skeletal Response to Mechanical Stimulation in Children With Osteogenesis Imperfecta?
Status:
Completed
Trial end date:
2017-11-02
Target enrollment:
Participant gender:
Summary
Osteogenesis Imperfecta(OI) is an inherited disorder characterised by extreme fragility of
the bones. Bones often break from little or no apparent cause.
Current available medicine can increase bone strength by making bones wider and "filling in"
the holes in the bone walls that weaken it. These medicines are bisphosphonates, given either
by a drip intravenously (eg pamidronate), or taken by mouth (eg risedronate). Their major
action is to prevent bone breakdown by stopping the normal process of removing and then
replacing old bone tissue, so in some parts of the bone, new bone formation is actually
reduced. Most studies of bisphosphonates in children with OI have shown increased bone
mineral density and improved exercise tolerance that could positively affect new bone
formation; some have shown reduced fracture rate. Bone is highly responsive to mechanical
stimulation. Whole body vibration (WBV) is a form of mechanical stimulation that has been
shown to improve bone mineral density in some individuals with narrow bones.
Little is known whether bisphosphonates affect the response of the skeleton to mechanical
stimulation. We will determine the response to mechanical stimulation in children with OI by
looking at bone turnover markers following WBV in those who are and are not treated with
bisphosphonates.
The results from this study will help us to understand whether skeleton in children with OI
is normally responsive to mechanical stimulation, and whether bisphosphonates alter that
responsiveness in a way that is either beneficial or not for increasing bone strength.