Background: Osteogenesis imperfecta (OI) is an inherited clinically heterogenous disorder of bone metabolism characterized by bone and skeletal fragility and an increased risk of fractures. Pamidronate infusion was the standard treatment, but zoledronic acid is increasingly used to treat children with OI.

Objectives: We conducted a systematic literature review to evaluate the efficacy and safety of intravenous zoledronic acid in the treatment of OI in pediatric patients.

Methods: A systematic review of the published literature was performed according to the PRISMA guidelines. Eligible articles were clinical trials and observational studies including pediatric patients (< 16 years) with OI treated with zoledronic acid. We selected articles published during the 20 past years. The selected languages were english and french. We included articles with a minimum sample size of five patients.

Results: Six articles fulfilled the selection criteria. Majority of patients were Chinese (58%).The predominant sex was male (65%), and the age of included patients ranged from 2.5 weeks to 16.8 years. For all patients, zoledronic infusions were administrated intravenously. The zoledronic treatment duration ranged from 1 to 3 years. Densitometry parameters before and after zoledronic treatment were evaluated and showed significant improvement both in LS-BMD Z-score and FN-BMD Z-scores. A significant decrease in fracture rate has also been noted both in vertebral and nonvertebral fracture incidence. The two most common side effects were fever and flu-like reactions. None of the patients presented severe adverse events.

Conclusion: zoledronic acid appeared to be safe and effective in the treatment of pediatric OI.

REFERENCES: NIL.

Acknowledgements: NIL.

Disclosure of Interests: None declared.