Clinical Characteristics and Therapeutic Management of Osteogenesis Imperfecta in Iraqi Children

Abstract

Background: Osteogenesis imperfecta (OI) is a rare congenital condition that results in bone fragility, recurrent fractures, and various extra-skeletal manifestations. Currently, intravenous bisphosphonate is the mainstay of medical treatment in OI. Objective: To identify the effect of current management strategies on Iraqi children diagnosed with OI. Methods: A retrospective study enrolled OI patients who were registered in Central Child Teaching Hospital, Baghdad, Iraq, from January 2015 to December 2022. We enrolled confirmed OI cases (either clinically and/or radiologically) who received cyclic pamidronate therapy for at least 3 cycles. They neither received other types of bisphosphonates nor underwent surgical intervention. Results: A total of 52 cases of OI had been identified, but only 36 patients were eligible for the current study. The mean age of the patients was 6.64±4.22 years. A statistically significant drop in the annual fracture rate in OI patients who received intravenous pamidronate cycles was seen, along with a significant rise in weight for age Z-score, lumbar spine bone mineral density DEXA Z-score, and alkaline phosphate levels. No significant improvement was documented in height for age Z-score in OI patients. Conclusions: Intravenous pamidronate cycles for Iraqi children with osteogenesis imperfecta work to lower their risk of breaking bones every year and raise their weight, lumbar spine bone mineral density, and alkaline phosphate levels. Pamidronate did not result in an improvement in the height of OI children.