Clinical Profile and IV Bisphosphonate Therapy in Children with Osteogenesis Imperfecta in Cipto Mangunkusumo Hospital.

Introduction: Osteogenesis imperfecta (OI) is a rare genetic disease affecting type I collagen in bone and skin. There is currently no cure for OI; however, studies showed that bisphosphonate administration confers some benefits. We aimed to know the characteristics of OI patients and the correlation between bisphosphonate therapy and fracture incidence.
Methods: We conducted a retrospective cross-sectional study from the OI registry database in Cipto Mangunkusumo Hospital. Acquired data from the registry and medical records were analyzed.
Results: As many as 41 OI patients received intravenous (IV) bisphosphonate therapy. Twenty-five subjects had sufficient data for analysis. The fracture frequency and rate pre and post therapy decreased from 4 (1-20) to 1 (0-10) and from 1.2 (0.17-9) to 0.57(0-2), consecutively. Positive significant correlation were found between duration of therapy and fracture frequency post therapy (p= 0.04), duration of therapy and fracture frequency difference (p=0.028), frequency of therapy and fracture frequency post therapy (p=0.03), and frequency of therapy and fracture frequency difference between pre and post therapy. (p=0.046).
Conclusion: The fracture frequency difference pre and post therapy increased along with the frequency and duration of IV bisphosphonate administration. This conclusion supports the benefit of IV bisphosphonate therapy in OI children. However, the reduction of fracture incidence in pediatric OI receiving intravenous bisphosphonate therapy is still inconclusive.