Osteogenesis Imperfecta (OI) Medication

Updated: Mar 03, 2022
  • Author: Manoj Ramachandran, MBBS, MRCS, FRCS; Chief Editor: Harris Gellman, MD  more...
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Medication Summary

The goals of pharmacotherapy are to reduce morbidity and prevent complications. [31]



Class Summary

Bisphosphonates are drugs that have been used off label for the treatment of osteogenesis imperfecta (OI). Drugs in this class may slow the loss of existing bones and may reduce long bone fractures and vertebral compressions. The most commonly used drug in this class is pamidronate. [34, 36, 32]

Bisphosphonates are analogues of inorganic pyrophosphate and act by binding to hydroxyapatite in bone matrix, thereby inhibiting the dissolution of crystals. They prevent osteoclast attachment to the bone matrix and osteoclast recruitment and viability.

For maximum gut absorption, all oral bisphosphonates should be taken at least 2 hours before or after meals. The newer bisphosphonates are not completely free of the risk of causing a mineralization defect, but their safe therapeutic window is much wider. They clearly are more potent than etidronate in reducing disease activity and normalizing alkaline phosphatase levels

Pamidronate (Aredia, APD)

Pamidronate is a potent second-generation bisphosphonate that acts principally by inhibiting osteoclastic bone resorption. Cyclic intravenous (IV) pamidronate is given in a dose of 7.5 mg/kg/y at 4- to 6-month intervals.

Alendronate (Fosamax)

Alendronate is a potent third-generation bisphosphonate that principally acts by inhibiting osteoclastic bone resorption.

Risedronate (Actonel, Atelvia)

Risedronate is a potent aminobisphosphonate that principally acts by inhibiting osteoclastic bone resorption.

Tiludronate (Skelid)

Tiludronate is a sulfur-containing bisphosphonate of intermediate potency between etidronate and newer nitrogen-containing bisphosphonates. No food, indomethacin, or calcium should be ingested within 2 hours before and 2 hours after administration. A 3-month posttreatment evaluation follows.

Zoledronate (Reclast, Zometa)

Zoledronate inhibits bone resorption. It inhibits osteoclastic activity and induces osteoclastic apoptosis.