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Zoledronic acid in children with osteogenesis imperfecta and Bruck syndrome: a 2-year prospective observational study
G A Otaify1, M S Aglan2, M M Ibrahim3
1Department of Clinical Genetics, Human Genetics and Genome Research Division, National Research Centre, Cairo, Egypt. ghadaotaify@yahoo.com.
Insights
Zoledronic acid (ZA) treatment in children with osteogenesis imperfecta (OI) and Bruck syndrome (BS) significantly reduced fractures and pain while improving bone density and motor skills. This study highlights ZA
Area of Science:
- Pediatric Endocrinology
- Bone Metabolism
- Genetic Skeletal Disorders
Background:
- Osteogenesis imperfecta (OI) and Bruck syndrome (BS) are genetic disorders characterized by bone fragility and low bone mineral density (BMD).
- Limited literature exists on the efficacy and safety of zoledronic acid (ZA) in pediatric populations with OI and BS.
- This study investigates the response of Egyptian children with OI and BS to ZA treatment over two years.
Purpose of the Study:
- To assess the efficacy and safety of zoledronic acid (ZA) in children diagnosed with osteogenesis imperfecta (OI) and Bruck syndrome (BS).
- To evaluate changes in bone mineral density (BMD), fracture rates, pain levels, and motor development following ZA treatment.
- To compare treatment outcomes between autosomal-dominant (AD) and autosomal-recessive (AR) forms of OI.
Main Methods:
- A cohort of 33 children with OI and 5 with BS received intravenous zoledronic acid (ZA) at 0.1 mg/kg every six months for two years.
- Bone mineral density (BMD) Z-scores, fracture rates, pain, ambulation, and a clinical severity score (CSS) were assessed at baseline and after two years.
- Outcomes were compared between AD-OI and AR-OI patient groups.
Main Results:
- Zoledronic acid (ZA) treatment led to significant improvements in BMD Z-scores in both spine and hip within six months (P < 0.003).
- A significant reduction in fracture rates (P < 0.001), pain (P < 0.001), and improved ambulation (P < 0.001) were observed.
- Clinical severity scores (CSS) significantly decreased after two years, with Autosomal-Recessive OI patients showing greater improvement.
Conclusions:
- Zoledronic acid (ZA) is a safe and effective treatment for pediatric osteogenesis imperfecta (OI) and Bruck syndrome (BS).
- The biannual infusion schedule was well-tolerated and convenient for pediatric patients.
- Treatment benefits correlated positively with disease severity, and CSS proved valuable for assessing severity and treatment response.
Unlabelled:
Treatment with zoledronic acid (ZA) over 2 years, among 33 children with osteogenesis imperfecta (OI) and five Bruck syndrome cases, showed reduction in fracture rates, pain, and improvement in bone mineral density (BMD) and motor milestones of development. This is the first study reporting the use of bisphosphonates in patients with Bruck syndrome (BS).
Introduction:
OI and BS are genetic disorders that result in bone fragility and reduced BMD. There is little literature describing the efficacy and safety of ZA in this population. In this study, we assess the response to treatment with ZA at six monthly intervals in Egyptian children with OI and BS for a period of 2 years.
Methods:
Thirty-three patients with OI and five patients with BS were treated with 0.1 mg/kg ZA intravenously every 6 months for 2 years during which they were followed up using different parameters. A clinical severity score (CSS) was applied to the patients before and 2 years after the start of therapy. Comparison of disease severity and response to ZA treatment between autosomal-dominant (AD) and autosomal-recessive (AR) OI patients was also done.
Results:
After 6 months of treatment, OI and BS patients showed a significant increase in BMD Z-scores (P < 0.003 in the spine and P < 0.004 in the hip), together with a significant drop in fracture rate (P < 0.001), relief of pain (P < 0.001), and improvement in ambulation (P < 0.001). CSS was significantly reduced after 2 years of treatment in both OI and BS patients. AR-OI patients were more severely affected than AD-OI patients and showed more significant improvement.
Conclusion:
Zoledronic acid proved to be safe and effective in the treatment of OI and BS. The biannual infusion protocol was convenient to patients. There was a positive correlation between disease severity and benefits of the treatment. The use of the CSS proved to be of value in the assessment of the degree of severity in OI, and with some modifications, it was a valuable tool for the assessment of response to treatment.
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