Date of Graduation

8-14-2010

Degree Type

Capstone Project

Degree Name

Master of Science in Physician Assistant Studies

First Advisor

James Ferguson PA-C, MPH

Second Advisor

Annjanette Sommers MS, PAC

Third Advisor

Rob Rosenow PharmD, OD

Abstract

Background: Osteogenesis Imperfecta (OI) is a rare, hereditary disorder that involves improper collagen synthesis, specifically, that of type 1 collagen, leading to bone fragility. There are four types of classification of OI, each varying in severity from multiple fractures to perinatal death. Bisphosphonates has recently been recognized as a pharmacologic treatment that aids in bone strength. More randomized controlled trials have been conducted to examine the efficacy of bisphosphonates in increasing the bone mineral density and decreasing the incidence of fractures in children with OI.

Methods: A comprehensive search of the medical literature was conducted using various search modalities including MEDLINE, CINAHL, AND ISI World of Science and using the keywords “osteogenesis imperfecta”, “children” and “bisphosphonates”. Specific examples of bisphosphonates were also used as keywords in the search such as [“pamidronate”OR “alendronate” OR “olendronate” OR risedronate]. Trials were only included if published in English after 2003 and if the trial was randomized and controlled. Bibliographic reference of prior systematic review was also examined to identify applicable literature. Studies were rated using the Jadad criteria.

Results: Nine articles were found that met the selection criteria, all of which were randomized controlled studies. Overall, it was found that in five studies the incidence of fractures was decreased in children with OI and in seven studies bone mineral density increased in children with OI.

Conclusion: Bisphosphonate therapy appears to increase bone mineral density and decrease the incidence of fractures in children with OI. However, results would be strengthened by a larger, longer, randomized controlled study. Many questions about the use of bisphosphonates in growing children remain including the long-term effects of bisphosphonates, optimal dosing, and more importantly the safety of bisphosphonates in children.

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