Wolfram syndrome and diabetes mellitus in Aotearoa, New Zealand: Phenotype and response to GLP ‐1 receptor agonist therapy
Authors' conclusion
We report genotypic and phenotypic variability in association with clinical features, including age of onset and severity. GLP1RA therapy was associated with improvements in diabetic control.
Study context
- Population
- Patients with WFS1 genetic variants (1 with Wolfram syndrome (WFS)-like syndrome) and diabetes mellitus (DM) from 5 New Zealand families, age 5–33 years, 4 females, 3 males; all receiving insulin5 on GLP-1 RA
- Participants
- 7 patients
- Exposure
- Pre- vs post-GLP-1 RA therapy
- Outcome
- Genotype
Main finding
As reported in the abstract
Compound heterozygous WFS1 variants (inherited from each parent)
5 patients
Heterozygous carriers of a single WFS1 missense variant, one associated with uniparental disomy
2 patients
Observational data: associations, not effects.
Further findings
Response to GLP-1 RA therapy
- Reduction in HbA1c (mean)
- 11.6 mmol/mol
- Reduction in total daily insulin dose (mean)
- 0.25 units/kg/day
Original publication
Wolfram syndrome and diabetes mellitus in Aotearoa, New Zealand: Phenotype and response to GLP ‐1 receptor agonist therapy. Diabet Med. Published 21 June 2026. doi:10.
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