Osteogenesis Imperfecta Blood Pressure Study

Study Summary

Osteogenesis Imperfecta (OI) is a genetic disorder caused by mutations to the alpha-1 or alpha-2 chain of type I collagen. Clinically, the disorder is characterized by bones that fracture easily, often from little or no apparent trauma. There is no information about Some institutions perform blood pressure monitoring on these patients with a cuff, while other institutions avoid this method due to concern for fracture. Instead, they use alternative forms of monitoring such as an arterial line. These methods come with their own risks, including clotting, decreased perfusion, and pain. In addition, arterial lines require intensive (and more expensive) monitoring in a pediatric intensive care unit due to risk of dislodgement and rapid blood loss. These blood pressure monitoring recommendations for patients with OI do not appear to be based on strong evidence. Anecdotally, some patients and healthcare professionals report hearing about individuals with OI who have suffered fractures from blood pressure cuffs. However, this is not well documented in the medical literature. Regular and accurate blood pressure monitoring is particularly important in the postoperative period, due to blood loss and fluid shifts, as well as utilization of advanced pain management techniques that can potentially impact blood pressure.

Primary Outcome

Cuff blood pressure measurements obtained without injury to patient with Osteogenesis Imperfecta

Interventions

• PROCEDURE Manual cuff blood pressure