Calcified Tissue International

, Volume 84, Issue 3, pp 203–209

Intravenous Pamidronate in Osteogenesis Imperfecta Type VII

  • Moira S. Cheung
  • Francis H. Glorieux
  • Frank Rauch
Article

DOI: 10.1007/s00223-008-9211-9

Cite this article as:
Cheung, M.S., Glorieux, F.H. & Rauch, F. Calcif Tissue Int (2009) 84: 203. doi:10.1007/s00223-008-9211-9

Abstract

Cyclical intravenous treatment with pamidronate is widely used to treat osteogenesis imperfecta (OI) types I, III, and IV, which are due to dominant mutations affecting collagen type I alpha chains. There is no information about the effects of pamidronate in children with OI type VII, an autosomal-recessive form of OI caused by a mutation in the cartilage-associated protein gene. In this retrospective single-center study, we compared the effects of pamidronate in four girls with OI type VII (age range 3.9–12.7 years) to those in eight girls with OI types caused by collagen type I mutations who were matched for age and disease severity. During 3 years of pamidronate therapy, lumbar spine areal bone mineral density increased and lumbar vertebral bodies improved in shape in patients with OI type VII. Other outcomes such as fracture rates and mobility scores did not show statistically significant changes in this small study cohort. There were no significant side effects noted during the time of follow-up. Thus, intravenous treatment with pamidronate seems to be safe and of some benefit in patients with OI type VII.

Keywords

Cartilage associated protein Children Fractures Osteogenesis imperfecta Pamidronate 

Copyright information

© Springer Science+Business Media, LLC 2009

Authors and Affiliations

  • Moira S. Cheung
    • 1
  • Francis H. Glorieux
    • 1
  • Frank Rauch
    • 1
  1. 1.Genetics UnitShriners Hospital for Children and McGill UniversityMontrealCanada

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