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Effect of alendronate therapy in children with osteogenesis imperfecta
Václav Vyskocil1, Richard Pikner, Stepán Kutílek
1Bone Disease Centre, Charles University Hospital, Pilsen, Czech Republic.
Insights
Oral alendronate significantly improved bone mineral density, reduced fractures, and eased pain in children with osteogenesis imperfecta. This bisphosphonate therapy enhanced quality of life without adverse effects.
Area of Science:
- Pediatric Endocrinology
- Bone Metabolism
- Genetic Disorders
Background:
- Osteogenesis imperfecta (OI) is a rare genetic disorder characterized by fragile bones.
- Current treatments for OI aim to reduce fracture risk and improve bone quality.
- The efficacy of bisphosphonates in pediatric OI requires further investigation.
Purpose of the Study:
- To evaluate the therapeutic effect of orally administered alendronate in children diagnosed with osteogenesis imperfecta.
- To assess changes in bone mineral density, fracture rates, pain, and mobility.
- To monitor bone turnover markers and adverse events during treatment.
Main Methods:
- A cohort of 30 children (aged 4-16 years) with osteogenesis imperfecta types I, III, and IV received alendronate for 3 years.
- Dosage varied based on age: 5 mg/day for 4-10 years, 10 mg/day for >10 years.
- Bone mineral density, fracture incidence, pain, mobility, bone turnover markers, and adverse reactions were assessed.
Main Results:
- Significant increases in bone mineral density Z-scores and a substantial decrease in fracture rates were observed after 1 year.
- Chronic pain decreased, and ambulation/mobility improved significantly within the first year.
- Sustained improvements were noted at 3 years, with reduced bone turnover markers and no adverse reactions.
Conclusions:
- Oral alendronate therapy positively impacted the quality of life for pediatric patients with osteogenesis imperfecta.
- Bisphosphonate treatment for OI in children should be administered within a structured, well-defined therapeutic protocol.
- Alendronate demonstrates a favorable risk-benefit profile in this pediatric population.
Objective:
To evaluate the effect of orally administered alendronate in children with osteogenesis imperfecta.
Methods:
Thirty children (16 girls and 14 boys; mean age at baseline 10.7 +/- 6.0 years; range 4-16 years) with osteogenesis imperfecta type I (n = 22), III (n = 2), or IV (n = 6) were treated with alendronate (5 mg/day in patients aged 4-10 years and 10 mg/day in children >10 years of age) for 3 years.
Results:
After 1 year of alendronate therapy we observed a significant increase in areal and volumetric bone mineral density Z-scores (from -2.03 +/- 1.51 to -1.04 +/- 1.20, and from -1.91 +/- 1.38 to -1.33 +/- 1.30, respectively, P < 0.001), together with a significant drop in fracture rate (from 3.77 +/- 1.57 to 0.13 +/- 0.57, P < 0.000001), relief of chronic pain (from 3.83 +/- 1.44 days of pain/week to 0.73 +/- 0.77, P < 0.000001) and improvement in ambulation/mobility (P < 0.00002). After additional 2 years of therapy there were no further significant changes in these parameters, however the improvement was still remarkable in comparison to the pretreatment values (P < 0.003, P < 0.004, P < 0.000001, P < 0.000001 and P < 0.00001, respectively). A significant drop in markers of bone turnover (urinary deoxypyridinoline and serum osteocalcin) occurred after 3 years of therapy (P < 0.003 and 0.004, respectively). No adverse reactions were observed throughout the treatment.
Conclusions:
Alendronate has positively influenced quality of life in paediatric patients with osteogenesis imperfecta. Bisphosphonate therapy should be used only in the context of a well-defined protocol.
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