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Effects of bisphosphonates in children with osteogenesis imperfecta: an AACPDM systematic review
Heidi Castillo1, Lisa Samson-Fang,
1Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.
Insights
Bisphosphonate treatment for osteogenesis imperfecta in children improves bone density and may reduce fractures. Optimal dosing, duration, and long-term effects require further investigation.
Area of Science:
- Pediatric Orthopedics
- Pharmacology
- Genetics
Background:
- Osteogenesis imperfecta (OI) is a genetic disorder characterized by fragile bones.
- Bisphosphonates are commonly used to treat OI in children, aiming to improve bone strength.
- Existing literature on bisphosphonate efficacy in pediatric OI is extensive but varies in quality.
Purpose of the Study:
- To systematically review the effects of bisphosphonate treatment in children with osteogenesis imperfecta.
- To assess the impact of bisphosphonates on bone density, fracture rates, growth, and other clinical outcomes.
- To identify gaps in the current research regarding optimal treatment strategies and long-term effects.
Main Methods:
- Systematic review adhering to the American Academy for Cerebral Palsy and Developmental Medicine methodology (Revision 1.1, 2004).
- Inclusion of eight studies with high internal validity to ensure reliable findings.
- Analysis of published literature on bisphosphonate therapy in pediatric osteogenesis imperfecta.
Main Results:
- Confirmed improvements in bone density across included studies.
- Observed reduction in fracture rates and enhanced growth in many, but not all, studies.
- Limited evaluation of broader impacts like deformity, surgery needs, pain, function, or quality of life; short-term side effects were minimal.
Conclusions:
- Bisphosphonates show promise in improving bone density and potentially reducing fractures in pediatric osteogenesis imperfecta.
- Significant gaps remain regarding optimal medication, dosage, treatment duration, and long-term outcomes.
- Further high-quality research, including larger controlled trials, is needed to address these gaps and evaluate bisphosphonates in diverse OI subtypes and age groups.
Abstract:
This systematic review of the effects of bisphosphonate treatment in children with osteogenesis imperfecta was conducted using the American Academy for Cerebral Palsy and Developmental Medicine methodology for developing systematic reviews of treatment interventions (Revision 1.1) 2004. Despite a large body of published literature, there have been only eight studies with a sufficiently high level of internal validity to be truly informative. These studies confirm improvement in bone density. Many, but not all studies, demonstrate reduction in fracture rate and enhanced growth. There has been extremely limited evaluation of broader treatment impacts such as deformity, need for orthopedic surgery, pain, functioning, or quality of life. Short-term side effects were minimal. Which medication and dosing regimen is optimal and how long patients should be treated are unclear. This body of evidence would be strengthened by a larger controlled trial, because many studies lacked adequate power to evaluate stated outcomes. These studies do not address the impacts of bisphosphonates in children with milder forms of osteogenesis imperfecta and severe forms that are not due to mutations in the type I pro-collagen gene (e.g. types VII and VIII). Additional research is needed into treatment of infants. More studies evaluating medication choices, optimal dosing, duration of treatment, post-treatment impacts, and long-term side effects are necessary.
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