Evidence mapPaperPMID 42324630Full record

ArticleDiabetic medicine : a journal of the British Diabetic Association2026

Wolfram syndrome and diabetes mellitus in Aotearoa, New Zealand: Phenotype and response to GLP-1 receptor agonist therapy.

Abigail L'Amie, Andrea L Vincent, Craig A Jefferies, Geoffrey Braatvedt, Esko Wiltshire, Fran Mouat, Benjamin B Albert, Rinki Murphy

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Article in Diabetic medicine : a journal of the British Diabetic Association, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 2 papers.

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0cells of the map it votes in
2citing papers in PubMed
field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

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Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

2 citing papers in PubMed.

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4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

8 authors.

Abigail L'AmieDepartment of Endocrinology, Greenlane Clinical Centre, Auckland Te Whatu Ora- Health, Auckland, New Zealand.ORCID https://orcid.org/0009-0005-6534-8918
Andrea L VincentFaculty of Medical and Health Sciences, University of Auckland, Auckland, New Zealand.
Craig A JefferiesFaculty of Medical and Health Sciences, University of Auckland, Auckland, New Zealand.ORCID https://orcid.org/0000-0002-0541-6094
Geoffrey BraatvedtMedical Specialists Group, Auckland, New Zealand.
Esko WiltshireChild Health Service, Te Whatu Ora/Health New Zealand Capital, Coast and Hutt Valley, Wellington, New Zealand.
Fran MouatStarship Children's Health, Te Whatu Ora, Health New Zealand, Auckland, New Zealand.
Benjamin B AlbertFaculty of Medical and Health Sciences, University of Auckland, Auckland, New Zealand.
Rinki MurphyDepartment of Endocrinology, Greenlane Clinical Centre, Auckland Te Whatu Ora- Health, Auckland, New Zealand.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

aimTo describe the clinical characteristics of patients with Wolfram syndrome (WFS) and diabetes mellitus (DM) in Aotearoa, New Zealand. Review of response to therapy in those treated with glucagon-like peptide-1 receptor agonists (GLP1RA).

methodsThis retrospective cohort study describes 7 patients with WFS1 genetic variants (1 patient with WFS-like syndrome), and DM from 5 New Zealand families (age range 5-33 years, 4 females, 3 males). All are receiving insulin. In 5 patients receiving GLP1RA therapy, we described their pre- and post-treatment biometric parameters, glycaemic control and visual acuity.

resultsGenetic testing identified compound heterozygous variants in the WFS1 gene (inherited from each parent) in five patients. Another two were heterozygous carriers of a single WFS1 missense variant, one of which was associated with uniparental disomy. Among patients receiving GLP1RA therapy, reductions were seen in HbA1c (mean 11.6 mmol/mol) and total daily insulin dose (mean 0.25 units/kg/day).

conclusionsWe 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. Longer-term follow-up is required to monitor for sustained benefit and for progression or improvement in other WFS clinical features.

Indexed as

Diabetes Mellitus, Type 2Glucagon-Like Peptide-1 Receptor AgonistsHypoglycemic AgentsMembrane ProteinsWolfram SyndromeAdolescentAdultBlood GlucoseChildChild, PreschoolFemaleGlucagon-Like Peptide-1 ReceptorGlycated HemoglobinHumansInsulinMaleBlood GlucoseGlucagon-Like Peptide-1 ReceptorGlucagon-Like Peptide-1 Receptor AgonistsGlycated HemoglobinHypoglycemic AgentsInsulinMembrane Proteinswolframin proteindiabetesGLP1 receptor agonistWFS1Wolfram

Identifiers

PMID42324630
PMCPMC13380341

What Socratic holds

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the Socratic graph.