{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,12]],"date-time":"2026-09-12T04:11:14Z","timestamp":1789186274025,"version":"build-2803163510"},"reference-count":29,"publisher":"Wiley","issue":"8","license":[{"start":{"date-parts":[[2026,6,21]],"date-time":"2026-06-21T00:00:00Z","timestamp":1782000000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2026,6,21]],"date-time":"2026-06-21T00:00:00Z","timestamp":1782000000000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Diabet Med"],"published-print":{"date-parts":[[2026,8]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Aim<\/jats:title>\n                    <jats:p>To 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\u2010like peptide\u20101 receptor agonists (GLP1RA).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>This retrospective cohort study describes 7 patients with WFS1 genetic variants (1 patient with WFS\u2010like syndrome), and DM from 5 New Zealand families (age range 5\u201333\u2009years, 4 females, 3 males). All are receiving insulin. In 5 patients receiving GLP1RA therapy, we described their pre\u2010 and post\u2010treatment biometric parameters, glycaemic control and visual acuity.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Genetic testing identified compound heterozygous variants in the\n                      <jats:italic>WFS1<\/jats:italic>\n                      gene (inherited from each parent) in five patients. Another two were heterozygous carriers of a single\n                      <jats:italic>WFS1<\/jats:italic>\n                      missense variant, one of which was associated with uniparental disomy. Among patients receiving GLP1RA therapy, reductions were seen in HbA1c (mean 11.6\u2009mmol\/mol) and total daily insulin dose (mean 0.25\u2009units\/kg\/day).\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>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. Longer\u2010term follow\u2010up is required to monitor for sustained benefit and for progression or improvement in other WFS clinical features.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1111\/dme.70405","type":"journal-article","created":{"date-parts":[[2026,6,22]],"date-time":"2026-06-22T04:19:18Z","timestamp":1782101958000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Wolfram syndrome and diabetes mellitus in Aotearoa, New Zealand: Phenotype and response to\n                    <scp>GLP<\/scp>\n                    \u20101 receptor agonist therapy"],"prefix":"10.1111","volume":"43","author":[{"ORCID":"https:\/\/orcid.org\/0009-0005-6534-8918","authenticated-orcid":false,"given":"Abigail","family":"L'Amie","sequence":"first","affiliation":[{"name":"Department of Endocrinology, Greenlane Clinical Centre Auckland Te Whatu Ora\u2010 Health  Auckland New Zealand"},{"name":"Department of Endocrinology Te Whatu Ora Waitemat\u0101, Health New Zealand  Auckland New Zealand"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrea L.","family":"Vincent","sequence":"additional","affiliation":[{"name":"Faculty of Medical and Health Sciences University of Auckland  Auckland New Zealand"},{"name":"Ophthalmology, Greenlane Clinical Centre Auckland, Te Whatu Ora\u2010 Health New Zealand  Auckland New Zealand"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0541-6094","authenticated-orcid":false,"given":"Craig A.","family":"Jefferies","sequence":"additional","affiliation":[{"name":"Faculty of Medical and Health Sciences University of Auckland  Auckland New Zealand"},{"name":"Starship Children's Health Te Whatu Ora, Health New Zealand  Auckland New Zealand"},{"name":"Liggins Institute University of Auckland  Auckland New 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