Muscle connective tissue in limb development and disease

  • Research type

    Research Study

  • Full title

    The role of muscle connective tissue in normal and abnormal upper limb development, and in the normal response to disease.

  • IRAS ID

    148404

  • Contact name

    Malcolm Logan

  • Contact email

    malcolm.logan@kcl.ac.uk

  • Clinicaltrials.gov Identifier

    NCT02611089

  • Duration of Study in the UK

    2 years, 7 months, 1 days

  • Research summary

    The objective of this work is to understand the mechanisms controlling normal limb development and how disruption of these events results in limb defects at birth. We aim to use this knowledge to improve the diagnosis and surgical treatment of a disabling, disfiguring limb anomaly called radial dysplasia. We also aim to harness the knowledge of how the limb normally develops to 'engineer' tissues to treat patients, after inherited defects, cancer or trauma.

    In individuals with radial dysplasia the thumb and radius (one of two forearm bones) are typically missing. Additionally, the forearm muscles, tendons and the surrounding fascia are abnormal, bending the wrist into a ‘U’ shape. Despite sophisticated surgical treatment, the forearm bowing typically recurs as the child grows, and the affected forearm is very short.

    Our laboratory has shown, in an experimental mouse model, the underlying problem is a change in the ‘muscle connective tissue’ layer. We wish to explore this further by comparing samples of the derivatives of muscle connective tissue from radial dysplasia patients with control samples.

    We will ask patients undergoing corrective hand surgery to let us take small tissue samples during some of their operations. We will also ask patients having other forms of hand surgery, such as surgery for hand injuries, to let us take similar samples for comparison. In either case, their planned operations always require an incision on the hand or forearm, and taking the tissue samples will not change their outcomes, the final size of the scar, or the way their scar develops. They will not have to attend any extra clinic sessions, only their routine surgical follow-up. We will examine the tissue samples in a laboratory to look for changes in tissue architecture and development, cell signalling, and how different cell populations behave when grown in culture.

  • REC name

    London - Hampstead Research Ethics Committee

  • REC reference

    15/LO/2085

  • Date of REC Opinion

    23 Dec 2015

  • REC opinion

    Favourable Opinion