Background: Osteogenesis imperfecta (OI) is a heritable disease marked by a varying degree of low bone mass and increased incidence of fractures. Approximately 1 in 15 000 and 1 in 20 000 children are affected by this connective tissue disorder. Although, the mainstay of treatment for children and adolescents with OI is multidisciplinary, the role of bisphosphonates is rapidly becoming the standard of care. Bisphosphonates work by inhibiting osteoclast mediated bone resorption, therefore, allowing osteoblast more time for bone formation. Currently, very little is known about the effect of oral bisphosphonate treatment; despite it being under investigation in controlled trials. This can be attributed to the lack of blinding and relatively small study populations in previous reviewed studies. Therefore, the purpose of this systematic review is to determine the efficacy of bisphosphonates in increasing bone mineral density and decreasing the incidence of fractures in a larger study population with adequate blinding in randomized control trials.
Methods: An exhaustive literature search was performed in the following databases Medline-Ovid, CINAHL,Web of Science, and Medline-PubMed. The keywords include “osteogenesis imperfecta,” “children,” and “bisphosphonates.” Articles selected were limited to those studies in the English language, studies with a publication date in or after 2010, randomized control trials and a study population greater than 100 participants.
Results: Two articles met inclusion criteria. The first study included was a randomized, double-blind, placebo-controlled study with 143 participants who demonstrated that risendronate was effective in both decreasing the incidence of fractures and increasing bone mineral density. The second study was a prospective, randomized, double-blind, parallel- group,placebo- controlled study with 139 participants. This study concluded that alendronate increased bone mineral density but did not decrease the incidence of fractures.
Conclusion: Largely, this conflict in opinion is the reason bisphosphonates are in great debate for the treatment of OI. It is essential to come to a consensus on whether or not bisphosphonates remain an adequate and safe treatment option for children with OI. The evidence presented in this paper proves that bisphosphonates are useful in increasing BMD, but the evidence on decreasing incidence of fractures remains inconclusive, indicating further research is necessary.
Keywords: osteogenesis imperfecta, children, bisphosphonates
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