Clinical trial NCT02934451: Dental Malocclusion and Craniofacial Development in OI
Official title: Dental Malocclusion and Craniofacial Development in OI
Study type: OBSERVATIONAL
Overall status: ACTIVE_NOT_RECRUITING
Conditions: Osteogenesis Imperfecta
Lead sponsor: Baylor College of Medicine
Sponsor class: OTHER
Collaborators: name: Shriners Hospitals for Children
name: Hospital for Special Surgery, New York
name: University of Nebraska
name: Hugo W. Moser Research Institute at Kennedy Krieger, Inc.
name: Children's National Research Institute
name: University of California, Los Angeles
name: Oregon Health and Science University
First posted: 2016-10-17
Enrollment: 75
Sex: ALL
Minimum age (years): 10
Maximum age (years): 100
Standard ages: CHILD
ADULT
OLDER_ADULT
Accepts healthy volunteers: no
Observational model: COHORT
Time perspective: PROSPECTIVE
Sampling method: PROBABILITY_SAMPLE
Study population: Individuals with Severe OI

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.

Eligibility criteria: 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

Primary outcomes:
measure: Teeth Misalignment
timeFrame: 5 years
Secondary outcomes:
measure: Neck Defects
timeFrame: 5 years
Other outcomes:
Locations:
city: Los Angeles
country: United States
facility: University of California Los Angeles
state: California
city: Houston
country: United States
facility: Baylor College of Medicine
state: Texas
city: Montreal
country: Canada
facility: Shriners Hospital for Children
state: Quebec
Study officials:
affiliation: McGill University
name: Jean-Marc Retrouvey, D.M.D.
role: STUDY_CHAIR
affiliation: Baylor College of Medicine
name: Reid Sutton, M.D.
role: STUDY_CHAIR
affiliation: Shriners Hospital for Children
name: Frank Rauch, M.D.
role: STUDY_CHAIR
References: