Skeletal phenotyping of patients with Osteogenesis Imperfecta

Completed

Conditions: Brittle Bone Disease
Osteogenesis Imperfecta
10028396

Registration Number: NL-OMON51155

Lead Sponsor: Isala Klinieken

Brief Summary: Not available

Detailed Description: Not available

Recruitment & Eligibility

Status: Completed

Sex: Not specified

Target Recruitment: 120

Inclusion Criteria

- Patients with Osteogenesis Imperfecta
- Adult (>18 years)
- Recent DEXA-scan ( < 3 years)

Exclusion Criteria

- Patients whose mobility does not allow proper and comfortable positioning for
scan acquisition due to extreme bowing of the extremities. Pre-screening on the
basis of already performed radiographs.
- Patients who have had a fracture at recent medical history (<2 years) at both
distal radii and tibiae.
- Patients who have had a malignancy at recent medical history (<2 years), who
have been treated with glucocorticoids less than 3 months ago, who have severe
kidney disease (eGFR <30 ml/min) or who suffer from other metabolic diseases
affecting bone.
- Female patients who are pregnant.

Study & Design

Study Type: Observational invasive

Study Design: Not specified

Primary Outcome Measures

Name	Time	Method
<p>The main outcome parameter is bone strength at the standard scan region,<br /><br>obtained with HR-pQCT-based micro-finite element (µFE-) analysis, in terms of<br /><br>bone stiffness and failure load. Additionally, volumetric bone and tissue<br /><br>mineral density and cortical and trabecular microarchitecture will be assessed<br /><br>with HR-pQCT. More specifically, density parameters include total, cortical,<br /><br>and trabecular volumetric bone and tissue mineral density. Microarchitecture<br /><br>parameters include trabecular bone volume fraction, number, thickness, and<br /><br>separation, and cortical thickness and porosity.</p><br>

Secondary Outcome Measures