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Effect of Bisphosphonates on Function and Mobility Among Children With Osteogenesis Imperfecta: A Systematic Review
Christopher S Constantino1, Joseph J Krzak2,3, Alissa V Fial4
1Orthopedic and Rehabilitation Engineering Center, Marquette University & Medical College of Wisconsin Milwaukee Wisconsin USA.
Insights
Bisphosphonates (BP) are standard for severe Osteogenesis Imperfecta (OI). This review found mixed evidence on BP
Area of Science:
- Medical research
- Genetics
- Pharmacology
Background:
- Osteogenesis Imperfecta (OI) is a rare genetic disorder causing bone fragility.
- Bisphosphonates (BP) are a standard treatment for severe OI.
- Limited reviews exist on BP's impact on OI patient function and mobility.
Purpose of the Study:
- To systematically review the effects of bisphosphonates (BP) on function and mobility in Osteogenesis Imperfecta (OI) patients.
- To assess the quality of existing research and identify gaps in understanding BP's functional outcomes.
Main Methods:
- Systematic review of 26 studies involving 801 children with OI.
- Searched PubMed, CINAHL, Cochrane Library, Web of Science, and PEDro databases.
- Assessed study quality using the Cochrane Collaboration's risk of bias tool.
Main Results:
- Randomized controlled trials showed no significant improvement in function/mobility with oral BP.
- Non-randomized studies indicated objective improvements in function and mobility with oral and IV BP.
- Significant variability in BP administration protocols and outcome measures (e.g., Bleck score) was observed.
Conclusions:
- Evidence on bisphosphonates' effect on Osteogenesis Imperfecta patient function and mobility is mixed.
- Study design and outcome measurement variability complicate definitive conclusions.
- Further high-quality research is needed to clarify BP's role in improving OI patient function and mobility.
Abstract:
Osteogenesis imperfecta (OI) is a rare genetic connective tissue disorder that results in bone fragility and deformity. Management is multi-disciplinary. Although pharmacologic intervention with bisphosphonates (BP) is a standard of care for individuals with severe OI, no consensus or reviews were found that focus on the effects of bisphosphonates on function and mobility. PubMed, CINAHL, Cochrane Library, Web of Science, and PEDro databases were searched for eligible articles for this review. Methodological quality was assessed using the Cochrane Collaboration's tool for risk of bias. Twenty-six studies (801 children) were reviewed and five showed a low risk of bias. Included studies showed significant variability among clinical protocols for administering BP. Randomized controlled trials did not demonstrate a significant improvement in function and mobility with oral BP administration, while non-randomized open-label uncontrolled studies demonstrated that oral and intravenous BP administration objectively improved function and mobility. The most common outcome measure used by the studies included in this review was the Bleck score. Effect sizes (d = 0.28 - 4.5) varied among studies. This systematic review also summarized the apparent confounding variables affecting results of previous studies and provided suggestions to improve the quality of future studies.
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