{
  "abstract": "Wolfram syndrome (WS) is a rare neurodegenerative disorder characterized by insulin-dependent diabetes, diabetes insipidus, hearing loss, and optic atrophy, with no cure and a poor prognosis. Continuous glucose monitoring (CGM) and insulin pumps, developed for type 1 diabetes, are increasingly used in managing WS-related insulin-dependent diabetes (WSIDD), but their effectiveness for these patients remains underexplored.Aim Is there published evidence for the use of diabetes technology in patients with WSIDD?Methods A systematic scoping review was conducted in accordance with the PRISMA statement (PRISMA, 2020). A PICO framework was used to formulate the search criteria. A computerised literature search was conducted on Embase, Web of Science, PubMed, MEDLINE and FindIt@Bham from inception to May 2025. The search terms applied were: ‘Wolfram syndrome’, ‘insulin pump’ and ‘continuous glucose monitoring’, with the Boolean operator ‘AND’ applied to collate search terms. Two independent reviewers used defined inclusion and exclusion criteria to follow a process of screening titles and abstracts, full-texts, and then extracting relevant data in an excel spreadsheet.Results 33 papers had their titles and abstracts screened by title and abstract, of which 12 papers were selected for full text. Only seven papers were found to be relevant to the research question, depicting patients with WSIDD using CGMS and/or insulin pumps. Of the seven relevant studies, none collected qualitative data on WS patients using CGM, such as human factors and quality of life data, which would have been useful in understanding what challenges and benefits that WSIDD patients encounter when using CGMS.Conclusion There is very little published evidence for the use of insulin pumps and/or continuous glucose sensors for patients with WSIDD, despite awareness of these technologies being used widely in their care. The next steps from our scoping review will be to conduct qualitative data collection through an online survey and interviews with patients, co-designed with the patient organisation, Wolfram syndrome UK.",
  "authors": [
    {
      "affiliations": [
        "University of Birmingham Medical School"
      ],
      "name": "Josephine Elliott"
    },
    {
      "affiliations": [
        "University of Birmingham Medical School",
        "University of Warwick",
        "Institute of Cancer and Genomic Sciences, University of Birmingham",
        "Institute of Applied Health Sciences, University of Birmingham"
      ],
      "name": "Anna Heatley"
    },
    {
      "affiliations": [
        "University of Birmingham Medical School",
        "University of Warwick",
        "Institute of Cancer and Genomic Sciences, University of Birmingham",
        "Institute of Applied Health Sciences, University of Birmingham"
      ],
      "name": "Felicity Boardman"
    },
    {
      "affiliations": [
        "University of Birmingham Medical School",
        "University of Warwick",
        "Institute of Cancer and Genomic Sciences, University of Birmingham",
        "Institute of Applied Health Sciences, University of Birmingham"
      ],
      "name": "Timothy Barrett"
    },
    {
      "affiliations": [
        "University of Birmingham Medical School",
        "University of Warwick",
        "Institute of Cancer and Genomic Sciences, University of Birmingham",
        "Institute of Applied Health Sciences, University of Birmingham"
      ],
      "name": "Renuka Dias"
    }
  ],
  "title": "P22 A scoping review of diabetes technologies used in wolfram syndrome",
  "uid": "b039823e-e0e2-5943-9f24-dc8dac758485"
}