Musculoskeletal health in adults with osteogenesis imperfecta

  • Research type

    Research Study

  • Full title

    Musculoskeletal health in adults with osteogenesis imperfecta

  • IRAS ID

    232383

  • Contact name

    Alex Ireland

  • Contact email

    a.ireland@mmu.ac.uk

  • Sponsor organisation

    Manchester Metropolitan University

  • Duration of Study in the UK

    0 years, 11 months, 28 days

  • Research summary

    Research Summary

    Osteogenesis imperfecta (OI, commonly known as brittle bone disease) is a group of genetic disorders which result in brittle, fracture-prone bones due to lower quality and/or quantity of type I collagen. However, soft tissues (muscle/tendons/ligaments) also contain a high proportion of type-I collagen, which could mean that their health and function is also affected. However, this has not been explored.

    We will use highly repeatable non-invasive clinical imaging and functional tests which are well-established in our laboratory to measure muscle, tendon and bone in young adults (age 18-35y) with type I OI and age-matched controls. We are examining individuals with type I OI (the most common form making up 50% of diagnoses), as absence of substantial growth deficits and limb deformity allow easier comparison with unaffected adults. This study will provide the first description of muscle and tendon structure/function and physical function in adults with OI, and how it relates to degree of bone strength deficit in these individuals. This information is essential to understanding the basis of several non-bony problems affecting adults with OI.

    Summary of Results

    Whilst 20 controls (target of 20) were recruited, only six individuals with osteogenesis imperfecta were recruited against a target of 20, despite approaching a large number of prospective participants. This included increasing our recruitment sites. In addition, the COVID-19 pandemic made recruitment and testing impossible in the second half of the project, and then university building work meant that the imaging and functional assessment equipment on which the study was based was decommissioned so further recruitment and testing was not possible. Therefore from this small dataset we are unable to make meaningful inferences.

  • REC name

    North West - Preston Research Ethics Committee

  • REC reference

    18/NW/0337

  • Date of REC Opinion

    9 Jun 2018

  • REC opinion

    Further Information Favourable Opinion