MPS II Siblings Study

  • Research type

    Research Study

  • Full title

    Retrospective chart review analysis of pairs of siblings with Mucopolysaccharidosis type II to evaluate the effectiveness of idursulfase started at 12 months of age and younger

  • IRAS ID

    308730

  • Contact name

    James Davison

  • Contact email

    James.davison@gosh.nhs.uk

  • Sponsor organisation

    Takeda Pharmaceutical Company Limited

  • Duration of Study in the UK

    0 years, 5 months, 20 days

  • Research summary

    Hunter syndrome is an inherited genetic disorder. Enzyme replacement therapy with IV idursulfase (Elaprase®) has shown clinical benefits in MPS II patients within both clinical trials and post marketing observational studies. Despite the clinical benefits of Elaprase in MPS II patients demonstrated by studies conducted to date, no evidence has so far been generated in very young patients who started ERT at an age of 12 months or less.
    Conducting a retrospective study, collecting data from patients who started ERT at an age of 12 months or less and describing their outcomes compared to their older siblings who started treatment at an age of 36 months or more, will help to address the current lack of scientific evidence on the effectiveness of Elaprase in this patient population and the impact of early initiation of ERT on clinical outcomes.

    Data will be collected retrospectively from patients’ medical charts from the date of diagnosis of MPS II to the last post Elaprase treatment initiation follow-up data available (i.e. minimum follow up time is 2 years).

  • REC name

    South West - Cornwall & Plymouth Research Ethics Committee

  • REC reference

    22/SW/0032

  • Date of REC Opinion

    10 Mar 2022

  • REC opinion

    Favourable Opinion