Dental Malocclusion and Craniofacial Development in OI

NCT02934451 | Active Not Recruiting DMC

• 1 other identifier: H38629
• Study Type: observational
• Target: 75
• Locations: 2 countries
• Sites: 3

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.

Conditions

Osteogenesis Imperfecta

Outcome Measures

Primary Outcomes (1)

• Teeth Misalignment

  Measure teeth misalignment in individuals with moderate to severe OI using scans of the teeth.

  5 years

Secondary Outcomes (1)

• Neck Defects

  5 years

Eligibility Criteria

• Age: 10 Years - 100 Years
• Sex: all
• Healthy Volunteers: No
• Age Groups: Child (0-17), Adult (18-64), Older Adult (65+)
• Sampling Method: Probability Sample

Study Population

Individuals with Severe OI

You may qualify if:

• 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 may not qualify 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

Sponsors & Collaborators

• Baylor College of Medicine: lead
• Hospital for Special Surgery, New York: collaborator
• University of Nebraska: collaborator
• Hugo W. Moser Research Institute at Kennedy Krieger, Inc.: collaborator
• Children's National Research Institute: collaborator
• University of California, Los Angeles: collaborator
• Oregon Health and Science University: collaborator
• Shriners Hospitals for Children: collaborator

Study Sites (3)

University of California Los Angeles

Los Angeles, California, 90095, United States

Location

Baylor College of Medicine

Houston, Texas, 77030, United States

Location

Shriners Hospital for Children

Montreal, Quebec, H3G 1A6, Canada

Location

MeSH Terms

Conditions

Osteogenesis Imperfecta

Condition Hierarchy (Ancestors)

Osteochondrodysplasias; Bone Diseases, Developmental; Bone Diseases; Musculoskeletal Diseases; Genetic Diseases, Inborn; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Collagen Diseases; Connective Tissue Diseases; Skin and Connective Tissue Diseases

Study Officials

• 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

Study Design

• Study Type: observational
• Observational Model: COHORT
• Time Perspective: PROSPECTIVE
• Sponsor Type: OTHER
• Responsible Party: PRINCIPAL INVESTIGATOR
• PI Title: Professor and Chairman

Study Record Dates

• First Submitted: October 12, 2016
• First Posted: October 17, 2016
• Study Start: August 1, 2016
• Primary Completion (Estimated): December 1, 2027
• Study Completion (Estimated): December 1, 2027
• Last Updated: January 27, 2026

Record last verified: 2025-01

Data Sharing

• IPD Sharing: Will not share

Locations

CA (1); US (2)