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Published on: December 10, 2010
Cyclic pamidronate therapy in children with osteogenesis imperfecta
1Department of Pediatric Endocrinology and Metabolism, Mofid Children's Hospital, Shaheed Beheshti University of Medical Sciences, Tehran, I.R. Iran. shadabshadab@yahoo.com
Summary
Pamidronate therapy significantly reduced fractures and improved bone density in children with severe osteogenesis imperfecta (OI). This treatment also eased chronic pain and fatigue without adverse effects, enhancing mobility.
Area of Science:
- Pediatric Endocrinology
- Bone Metabolism
- Pharmacology
Background:
- Severe osteogenesis imperfecta (OI) is a debilitating genetic disorder causing osteopenia and frequent fractures.
- Limited effective therapeutic options have historically been available for severe OI.
- Recent advancements aim to improve bone health and reduce fracture incidence in affected children.
Purpose of the Study:
- To evaluate the efficacy of pamidronate in enhancing bone mineralization in children with severe OI.
- To assess the safety profile of pamidronate treatment in this pediatric population.
- To determine the impact of pamidronate on fracture incidence and clinical outcomes.
Main Methods:
- Sixty-four children with severe OI received intravenous pamidronate (1 mg/kg/day for 3 days every 4 months) for 24-48 months.
- Regular assessments included clinical status, bone turnover markers, bone mineral density (BMD), and radiological changes.
- Treatment involved monitoring for adverse effects and evaluating improvements in mobility and pain.
Main Results:
- Fracture incidence decreased significantly from a median of 8 to 0 fractures per year (p <0.05).
- Bone mineral density (BMD) z-score of the lumbar spine improved significantly after 16 months (p <0.001).
- Serum alkaline phosphatase (ALP) levels decreased, indicating reduced bone turnover, with improved mobility, pain relief, and no adverse effects.
Conclusions:
- Cyclic administration of pamidronate is an effective treatment for improving bone mineralization in children with severe OI.
- Pamidronate therapy significantly reduces fracture incidence and alleviates chronic pain and fatigue.
- The treatment demonstrates a favorable safety profile in pediatric patients with osteogenesis imperfecta.
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