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Effect of intravenous pamidronate treatment in children with osteogenesis imperfecta
Irum Atta1, Fauzia Iqbal1, Saira Waqar Lone1
1Department of Paediatrics, National Institute of Child Health, Karachi.
Insights
Pamidronate treatment significantly reduced fractures and improved bone mineral density, mobility, wellbeing, and pain in children with osteogenesis imperfecta (OI). This bisphosphonate therapy offers effective symptomatic relief for OI patients.
Area of Science:
- Pediatric Endocrinology
- Bone Metabolism
- Genetic Disorders
Background:
- Osteogenesis imperfecta (OI) is a genetic disorder characterized by fragile bones, leading to frequent fractures and significant morbidity in children.
- Current treatments aim to manage symptoms, reduce fracture risk, and improve quality of life.
Purpose of the Study:
- To evaluate the efficacy of intravenous pamidronate treatment in pediatric patients diagnosed with osteogenesis imperfecta.
- To assess the impact of pamidronate on fracture rates, bone mineral density, mobility, and overall wellbeing.
Main Methods:
- An experimental study conducted at the National Institute of Child Health, Karachi, Pakistan, from 2007 to 2011.
- Included children with OI experiencing over 3 fractures/year or platyspondyly, treated with pamidronate (1 mg/kg/day for 3 consecutive days, every 3 months for 2 years).
- Evaluated fracture rate, BMD, mobility, wellbeing, and pain episodes at baseline and after 2 years.
Main Results:
- The annual fracture rate decreased significantly from 5.8 to 0.6 (p < 0.001) in 72 participating children.
- Bone mineral density (BMD) Z-scores improved from -5.3 to -1.7 (p < 0.001).
- Mobility scores, wellbeing, and pain episodes showed significant improvements (p < 0.001), with 92% of patients showing a good response.
Conclusions:
- Intravenous pamidronate is an effective symptomatic treatment for children with osteogenesis imperfecta, regardless of mutation severity or clinical phenotype.
- Cyclical bisphosphonate therapy positively impacts fracture rate, BMD, mobility, wellbeing, and pain in pediatric OI patients.
Objective:
To assess the beneficial effect of intravenous pamidronate treatment in children with osteogenesis imperfecta (OI).
Study Design:
Experimental study.
Place And Duration Of Study:
Endocrine Unit at the National Institute of Child Health, Karachi, Pakistan, from January 2007 to December 2011.
Methodology:
All children diagnosed with osteogenesis imperfecta on the basis of repeated spontaneous fractures and typical radiological findings registered during the study period, were included in this study. Pamidronate therapy were offered to those with more than 3 fractures per year or had platyspondyly. Pamidronate disodium was diluted in isotonic saline and administered by slow ravenous infusion over 3 hours in a dosage 1 mg/kg/day for 3 consecutive days 3 monthly for 2 years. Fracture rate, bone mineral density (BMD), mobility score, wellbeing and pain episodes were evaluated at baseline and 2 years after the treatment. Good response was defined as less than 2 fractures per year or mobility score improvement and poor response as more than 2 fracture per year with mobility score less than 2.
Results:
Seventy two patients were included in this study. There were 40 boys and 32 girls with mean age of 3.64 ± 3.2 years. The annual fracture rate decreased overall from 5.8 ± 1.61 to 0.6 ± 0.93 (p < 0.001). BMD Z-score improved from -5.3 ± 1.74 to -1.7 ± 0.72 (p < 0.001). Mobility score was 0.94 ± 1.30 at baseline and 2.5 ± 1.02 at the end of the treatment (p < 0.001). Wellbeing gained from 3.63 ± 1.44 to 7.8 ± 1.18 (p < 0.001) and pain episode improved from 24.1 ± 8.15 to 2.7 ± 8.31 (p < 0.001). Good response was noted in 92% of patients and poor response in 8% patients.
Conclusion:
Bisphosphonate seems to be an effective symptomatic treatment for children with osteogenesis imperfecta irrespective of severity of mutation or clinical phenotype. Cyclical bisphosphonate therapy has a positive effect on fracture rate, BMD, mobility score, wellbeing and pain episode.
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