J Korean Soc Endocrinol.  2004 Oct;19(5):485-491.

Effects of Pamidronate Treatment on Osteogenesis Imperfecta

• Lee SW
• Kim HJ
• Cho JH
• Lee HS
• Jung YM
• Kim DJ
• Lee KW
• Chung YS

Abstract

BACKGROUND: Osteogenesis imperfecta (OI) is a congenital disorder of type I collagen, with variable phenotypes, due to increased bone fragility and low bone mass. Previous pharmacological treatments for OI have been attempted with calcitonin and growth hormone but with little beneficial effects. Recently, Glorieux reported the beneficial effects of bisphosphonates in OI.
METHODS
In this study, the effects of pamidronate treatment were evaluated in 9 patients with OI. All patients received intravenous pamidronate infusions, which was dose adjusted according to the patients' age. The outcome measures included the biochemical bone markers; serum alkaline phosphatase, urine deoxy-pyridinoline, urine Ca/Cr ratio, and bone mineral density (BMD).
RESULTS
Serum alkaline phosphatase, urine deoxypyridinoline, and urine Ca/Cr ratio were slightly decreased after 1 year of therapy, although these changes were not statistically significant. The BMDs of the lumbar spine and proximal femur were significantly increased after 1-year of pamidronate treatment. No fractures were reported during the 1 year treatment periods.
CONCLUSION
Pamidronate treatment had an effect on the BMD in osteogenesis imperfecta, probably due to decreasing bone resorption

MeSH Terms

Alkaline Phosphatase
Bone Density
Bone Resorption
Calcitonin
Collagen Type I
Congenital, Hereditary, and Neonatal Diseases and Abnormalities
Diphosphonates
Femur
Growth Hormone
Humans
Osteogenesis Imperfecta*
Osteogenesis*
Outcome Assessment (Health Care)
Phenotype
Spine
Alkaline Phosphatase
Calcitonin
Collagen Type I
Diphosphonates
Growth Hormone