Published online March 1, 2007
PEDIATRICS Vol. 119 Supplement March 2007, pp. S163-S165 (doi:10.1542/10.1542/peds.2006-2023I)
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SUPPLEMENT ARTICLE


Experience With Bisphosphonates in Osteogenesis Imperfecta

Francis H. Glorieux, OC, MD, PhD

Genetics Unit, Shriners Hospital for Children and McGill University, Montreal, Quebec, Canada

Until recently, medical management of osteogenesis imperfecta, a genetic disorder of reduced bone mass and frequent fractures, was elusive, and treatment was focused on maximizing mobility and function. The introduction of bisphosphonates for the treatment of osteogenesis imperfecta 14 years ago changed this paradigm. Cyclic intravenous pamidronate therapy leads to an increase in bone density and a decrease in fracture rate in patients with osteogenesis imperfecta. Pamidronate therapy has a positive impact on functional parameters including improved energy, decreased bone pain, and increased ambulation. Histomorphometric studies have shown that the reduced osteoclast activity results in gains in cortical thickness and trabecular bone volume. Potential negative effects may include prolonged time to heal after osteotomies and a decrease in the rate of bone remodeling. Overall, it seems clear that the benefits of pamidronate therapy outweigh its potential risks in moderate-to-severe osteogenesis imperfecta, and pamidronate therapy has become the standard of care for patients with this condition. Questions remain regarding when treatment should be stopped and the need for pamidronate therapy in patients with mild osteogenesis imperfecta.

Key Words: osteogenesis imperfecta • pamidronate • bisphosphonate

Abbreviations: OI—osteogenesis imperfecta

Accepted Oct 5, 2006.


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