| Problem: Osteogenesis imperfecta (OI), more commonly known as "brittle bone disease" because of its resultant fragile bones and increased susceptibility to bone fracture, is the most common genetic metabolic bone disorder. Nearly 90% of OI cases are caused by heterozygosity of dominant mutations in one of the two Type I collagen genes, and clinical heterogeneity of the disease ranges from death in the perinatal period to marked short stature and severe bone deformity. Early and consistent therapies aim to increase bone strength and density while decreasing the occurrence and recurrence of fractures. CH greatly impacts our world; its immense burden on both local and global communities alike has a serious influence on global health. Methods: A search of PubMed and Medline was utilized to search for articles covering the use of bisphosphonate (BP) therapy for the treatment, management, and outcomes in patients with OI within the last 10 years. Key words were used such as "osteogenesis imperfecta", "bisphosphonate use and OI", "pharmaceutical management of OI", "metabolic bone disease", "therapies in O1" and "brittle bone disease". Articles were then retrieved and searched from the reference lists of those articles to find other potentially relevant articles. Results: The major finding in review of the literature yielded the benefits of using bisphosphonates for long-term treatment of the disease. When used appropriately and with early intervention, most bisphosphonate medications were found to reduce bone turnover, increase bone mineral density, and increase cortical width of bone. Conclusion: There is a clear benefit in the use of BP therapy for the treatment and management of OI. Since the disease is caused by an abnormality of collagen and bone strength, the increase in bone mass resulting from bisphosphonate therapy leads to decrease in the overall occurrence and recurrence of fractures. The impact of early and broad intervention, in combination with consistent therapies, has proven to greatly benefit patients afflicted with the disease. However, in communities of OI patients who are at a disadvantage of receiving early, broad, and consistent management, alternative methods must be established in treating those whom would otherwise benefit from the aggressive therapies available to patients in North America. |
