Stickler Syndrome and Perthes disease 1.0

  • Research type

    Research Study

  • Full title

    What is the association between Stickler Syndrome and Legg-Calve-Perthes disease?

  • IRAS ID

    265012

  • Contact name

    Philip Alexander

  • Contact email

    philip.alexander@addenbrookes.nhs.uk

  • Sponsor organisation

    Cambridge University Hospitals NHS Foundation Trust

  • Duration of Study in the UK

    1 years, 0 months, 1 days

  • Research summary

    Stickler Syndrome is a disease affects eyes, joints, hearing and face. Perthes disease (Legg-Calvé-Perthes Disease) represents the unorganised cell death of most cells of the femoral head in children caused due to lack of blood supply, which itself is due to an unknown cause. In this study, we will report a novel association between Stickler Syndrome and Perthes disease. Patients with a history or clinical features of Perthes disease will be recruited to the study through the National Diagnostic Stickler Syndrome Service based at Addenbrookes Hospital, Cambridge. Additionally, the Stickler Syndrome Support Group intends to conduct a systematic nationwide survey to ascertain how many patients with Stickler Syndrome, within it’s members, have a concurrent diagnosis of Perthes disease. This survey will not be conducted by the research group but will be conducted by the Stickler Support Group (an independent support group), for their own records. If Stickler patients who have been given a past diagnosis of Perthes disease give their permission, then their information will be forwarded to the research team in Addenbrookes (Stickler Syndrome Support Service). These patients will also be recruited into the study.
    Selected patients will have their radiographs will be obtained from other hospitals if necessary. Radiographs will be reported by a radiologist specialising in skeletal dysplasia, to radiologically confirm the diagnosis of Perthes disease. Evidence from the radiographs of the affected patients will used to report the association. We hope to consolidate a series of case reports.

  • REC name

    London - West London & GTAC Research Ethics Committee

  • REC reference

    20/LO/0189

  • Date of REC Opinion

    7 May 2020

  • REC opinion

    Further Information Favourable Opinion