Osteogenesis Imperfecta
Conditions
Brief 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.
Detailed description
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.
Interventions
None listed
Sponsors
Baylor College of Medicine
Shriners Hospitals for Children
Hospital for Special Surgery, New York
University of Nebraska
Hugo W. Moser Research Institute at Kennedy Krieger, Inc.
Children's National Research Institute
University of California, Los Angeles
Oregon Health and Science University
Study design
Observational model
COHORT
Time perspective
PROSPECTIVE
Eligibility
Sex/Gender
ALL
Age
10 Years to 100 Years
Healthy volunteers
No
Inclusion criteria
* 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)
Exclusion criteria
* 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
Design outcomes
Primary
| Measure | Time frame | Description |
|---|---|---|
| Teeth Misalignment | 5 years | Measure teeth misalignment in individuals with moderate to severe OI using scans of the teeth. |
Secondary
| Measure | Time frame | Description |
|---|---|---|
| Neck Defects | 5 years | Determine neck defects in individuals with moderate to severe OI using scans of the neck and jaw. |
Countries
Canada, United States
Contacts
STUDY_CHAIRJean-Marc Retrouvey, D.M.D.
McGill University
STUDY_CHAIRReid Sutton, M.D.
Baylor College of Medicine
STUDY_CHAIRFrank Rauch, M.D.
Shriners Hospital for Children
Outcome results
None listed