Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
| Name | Class |
|---|---|
| Shriners Hospitals for Children | OTHER |
| Hospital for Special Surgery, New York | OTHER |
| University of Nebraska | OTHER |
| Hugo W. Moser Research Institute at Kennedy Krieger, Inc. |
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
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.
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.
Not provided
Not provided
Not provided
Not provided
Not provided
Not provided
| Measure | Description | Time Frame |
|---|---|---|
| Teeth Misalignment | Measure teeth misalignment in individuals with moderate to severe OI using scans of the teeth. | 5 years |
| Measure | Description | Time Frame |
|---|---|---|
| Neck Defects | Determine neck defects in individuals with moderate to severe OI using scans of the neck and jaw. | 5 years |
Not provided
Inclusion Criteria:
Exclusion Criteria:
Not provided
Not provided
Individuals with Severe OI
Not provided
| Name | Affiliation | Role |
|---|---|---|
| Jean-Marc Retrouvey, D.M.D. | McGill University | Study Chair |
| Reid Sutton, M.D. | Baylor College of Medicine | Study Chair |
| Frank Rauch, M.D. | Shriners Hospital for Children | Study Chair |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| University of California Los Angeles | Los Angeles | California | 90095 | United States | ||
| Baylor College of Medicine |
The clinical information collected for this study will be stored in a computer database at the Data Management and Coordinating Center at the University of South Florida in Tampa, FL and also sent to a Federal data repository. A data repository provides a way for researchers to store the information collected during the research study for future research studies. The data management center uses several layers of protection for the clinical data stored in its computer database. It meets all of the local and federal security requirements for research datacenters. Information is stored only using a study identification number. Only the investigators and the study staff who collect and enter study data will have access to the key which links the identification number to the participant.
Not provided
Not provided
Not provided
Not provided
Not provided
| ID | Term |
|---|---|
| D010013 | Osteogenesis Imperfecta |
| ID | Term |
|---|---|
| D010009 | Osteochondrodysplasias |
| D001848 | Bone Diseases, Developmental |
| D001847 | Bone Diseases |
| D009140 | Musculoskeletal Diseases |
Not provided
Not provided
| OTHER |
| Children's National Research Institute | OTHER |
| University of California, Los Angeles | OTHER |
| Oregon Health and Science University | OTHER |
Not provided
Not provided
Not provided
| Houston |
| Texas |
| 77030 |
| United States |
| Shriners Hospital for Children | Montreal | Quebec | H3G 1A6 | Canada |
| D030342 | Genetic Diseases, Inborn |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |
| D003095 | Collagen Diseases |
| D003240 | Connective Tissue Diseases |
| D017437 | Skin and Connective Tissue Diseases |