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Review Question - QID 3646

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QID 3646 (Type "3646" in App Search)
A 10-year-old child falls from a standing height and sustains the injury shown in Figure A. Her medical history includes hearing defects and the facial appearance shown in Figure B. In addition to operative fixation of her fracture she is scheduled to receive cyclical intravenous pamidronate administration as a treatment after the fracture is healed. Which of the following is associated with this form of treatment?
  • A
  • B

No change in bone pain

3%

41/1266

No change in future fracture incidence

5%

68/1266

An increase in osteoblast density

11%

136/1266

An increased risk of secondary osteosarcoma

8%

106/1266

An increase in bone density

72%

906/1266

  • A
  • B

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The history and images are consistent with osteogenesis imperfecta (OI). Olecranon avulsion fractures are often seen in patients with OI and children presenting with these should be evaluated for OI. The clinical image of blue sclera can also be a characteristic finding in patients with OI. The Bisphosphonates have been shown to decrease fracture incidence and bone pain while improving bone density and overall function in OI patients.

Zeitlin et al performed a Level 5 review of OI. They state that Sillence Types I through IV are a mutation in the COL1A1 and COL1A2 genes that encode type I collagen. They report that cyclical intravenous pamidronate administration reduces bone pain and fracture incidence, and increases bone density and level of ambulation.

Burnei et al also performed a Level 5 review of OI. They report that the use of bone marrow transplantation to increase osteoblast density in OI patients is currently being researched as a potential treatment of OI.

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