Summary

Eligibility
for people ages 10-100 (full criteria)
Location
at Los Angeles, California and other locations
Dates
study started
completion around

Description

Summary

Osteogenesis imperfecta (OI) is a rare inherited disorder that causes bones to break easily. Individuals with osteogenesis imperfecta break bones often and may have other problems, including hearing loss and pain and difficulty getting around. People with moderate to severe OI may also be diagnosed with dentinogenesis imperfecta (DI). DI is characterized by grey or brown teeth that may chip and wear down and break easily. People with DI may also have skull and neck defects. These patients may have severe teeth misalignment resulting in clinically significant chewing problems. Teeth misalignment in OI is very hard to treat because of the quality and quantity of bone. The overall goal of this study is to improve dental health to improve the quality of life of people with OI.

Details

Investigators will look at dental health in people with OI and will describe teeth misalignment and head and neck defects in individuals with moderate to severe Osteogenesis Imperfecta (OI).

Investigators will look at results from the Longitudinal study of OI to complete the study evaluations. Several x-rays will be performed for this study. Participants will have a 3D scan of the mouth and a Cone Beam CT scan of the jaw at a baseline visit and at 3 years after the baseline visit. These study visits can be at the same time as the Longitudinal Study of OI study visits.

Keywords

Osteogenesis Imperfecta, Malocclusion

Eligibility

You can join if…

Open to people ages 10-100

  • Males and females with Clinical diagnosis of OI other than OI type I
  • Individuals 10 years or older
  • Participant of the Brittle Bone Disease (BBD) Longitudinal Study (7701)

You CAN'T join if...

  • Individuals who cannot be correctly positioned for valid radiographic analysis (e.g., due to severe scoliosis or short neck secondary to basilar invagination)
  • Women who are pregnant

Locations

  • University of California Los Angeles
    Los Angeles California 90095 United States
  • Baylor College of Medicine
    Houston Texas 77030 United States
  • Shriners Hospital for Children
    Montreal Quebec H3G 1A6 Canada

Details

Status
in progress, not accepting new patients
Start Date
Completion Date
(estimated)
Sponsor
Baylor College of Medicine
ID
NCT02934451
Study Type
Observational
Participants
About 75 people participating
Last Updated