{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,13]],"date-time":"2026-07-13T13:12:34Z","timestamp":1783948354436,"version":"3.55.0"},"reference-count":15,"publisher":"BMJ","issue":"2","license":[{"start":{"date-parts":[[2025,4,23]],"date-time":"2025-04-23T00:00:00Z","timestamp":1745366400000},"content-version":"unspecified","delay-in-days":22,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["BMJ Open Diab Res Care"],"accepted":{"date-parts":[[2025,4,7]]},"published-print":{"date-parts":[[2025,4]]},"abstract":"<jats:sec>\n                  <jats:title>Introduction<\/jats:title>\n                  <jats:p>Glucagon-like peptide-1 receptor agonists (GLP-1 RA) and dual glucose insulinotropic polypeptide (GIP)\/GLP-1 RA are widely prescribed, but their effectiveness in different subtypes of maturity-onset diabetes of the young (MODY) is unknown.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Research design and methods<\/jats:title>\n                  <jats:p>We present a descriptive case series of individuals from two MODY cohorts who used GLP-1 RA or dual GIP\/GLP-1 RA. Paired <jats:italic>t<\/jats:italic> tests were used to compare HbA1c, body mass index (BMI), and sulfonylurea (SU) dose before and after GLP-1 RA or dual GIP\/GLP-1 RA therapy.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Results<\/jats:title>\n                  <jats:p>10 individuals (4 hepatocyte nuclear factor-1\u03b1 (HNF1A)-MODY, 4 hepatocyte nuclear factor-4\u03b1 (HNF4A)-MODY, 1 ATP-binding cassette transporter subfamily C member 8 (ABCC8)-MODY, 1 hepatocyte nuclear factor-1\u03b2 (HNF1B)-MODY) were identified who used GLP-1 RA or dual GIP\/GLP-1 RA. In patients with HNF1A-MODY and HNF4A-MODY, GLP-1 RA reduced HbA1c by 1.3% (p=0.010), BMI by 2.90\u2009kg\/m<jats:sup>2<\/jats:sup> (p=0.008), and total daily dose of SU by 66.6% (p=0.005). Dual GIP\/GLP-1 RA treatment led to a non-statistically significant decrease in HbA1c of 1.8% (p=0.104), a statistically significant reduction in BMI of 8.73\u2009kg\/m<jats:sup>2<\/jats:sup> (p=0.010), and all patients discontinued SU (n=2) and one discontinued insulin. In patients with ABCC8-MODY and HNF1B-MODY, GLP-1 RA reduced HbA1c by 1.2% and 1.8%, BMI by 1.1 kg\/m<jats:sup>2<\/jats:sup> and 1.2\u2009kg\/m<jats:sup>2<\/jats:sup>, and the patients no longer required treatment with SU or insulin, respectively.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Conclusions<\/jats:title>\n                  <jats:p>GLP-1 RA and dual GIP\/GLP-1 RA lowered HbA1c, BMI, and SU dose in patients with MODY.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1136\/bmjdrc-2024-004885","type":"journal-article","created":{"date-parts":[[2025,4,23]],"date-time":"2025-04-23T23:15:15Z","timestamp":1745450115000},"page":"e004885","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":6,"title":["GLP-1 RA and dual GIP\/GLP-1 RA treatment in MODY: a descriptive case series"],"prefix":"10.1136","volume":"13","author":[{"given":"Alina Z","family":"Mehdi","sequence":"first","affiliation":[{"name":"Division of Metabolism, Endocrinology and Diabetes, Department of Internal Medicine, University of Michigan, Ann Arbor, Michigan, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lily","family":"Deng","sequence":"additional","affiliation":[{"name":"Division of Endocrinology, University of Cincinnati College of Medicine, Cincinnati, Ohio, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Colby L","family":"Chase","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, University of Michigan, Ann Arbor, Michigan, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria Cristina","family":"Foss-Freitas","sequence":"additional","affiliation":[{"name":"Division of Metabolism, Endocrinology and Diabetes, Department of Internal Medicine, University of Michigan, Ann Arbor, Michigan, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Brigid","family":"Gregg","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Division of Pediatric Endocrinology, University of Michigan Medical School, Ann Arbor, Michigan, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rochelle N","family":"Naylor","sequence":"additional","affiliation":[{"name":"Department of Pediatrics and Medicine, Section of Endocrinology, Diabetes and Metabolism, The University of Chicago, Chicago, Illinois, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9171-1144","authenticated-orcid":false,"given":"Elif A","family":"Oral","sequence":"additional","affiliation":[{"name":"Division of Metabolism, Endocrinology and Diabetes, Department of Internal Medicine, University of Michigan, Ann Arbor, Michigan, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0502-674X","authenticated-orcid":false,"given":"William H","family":"Herman","sequence":"additional","affiliation":[{"name":"Division of Metabolism, Endocrinology and Diabetes, Department of Internal Medicine, University of Michigan, Ann Arbor, Michigan, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8178-0886","authenticated-orcid":false,"given":"Mansa","family":"Krishnamurthy","sequence":"additional","affiliation":[{"name":"Endocrinology, Cincinnati Children\u2019s Hospital Medical Center, Cincinnati, Ohio, USA"},{"name":"Cincinnati Children\u2019s Hospital Medical Center"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4964-7716","authenticated-orcid":false,"given":"David T","family":"Broome","sequence":"additional","affiliation":[{"name":"Department of Metabolism, Endocrinology and Diabetes (MEND), University of Michigan Michigan Medicine, Ann Arbor, Michigan, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"239","published-online":{"date-parts":[[2025,4,23]]},"reference":[{"key":"2025073112575667000_13.2.e004885.1","first-page":"234","article-title":"Maturity onset diabetes of the young: clinical characteristics, diagnosis and management","volume":"10","author":"Kavvoura","year":"2012","journal-title":"Pediatr Endocrinol Rev"},{"key":"2025073112575667000_13.2.e004885.2","unstructured":"Hoffman LS , Jialal I . Diabetes, Maturity Onset in the Young (Mody). StatPearls:StatPearls Publishing, 2019. Available: https:\/\/www.ncbi.nlm.nih.gov\/books\/NBK532900\/1"},{"key":"2025073112575667000_13.2.e004885.3","doi-asserted-by":"crossref","first-page":"S101","DOI":"10.2337\/diabetes.50.2007.S101","article-title":"beta-cell genes and diabetes: quantitative and qualitative differences in the pathophysiology of hepatic nuclear factor-1alpha and glucokinase mutations","volume":"50 Suppl 1","author":"Pearson","year":"2001","journal-title":"Diabetes"},{"key":"2025073112575667000_13.2.e004885.4","doi-asserted-by":"publisher","DOI":"10.1093\/emboj\/17.22.6701"},{"key":"2025073112575667000_13.2.e004885.5","doi-asserted-by":"publisher","DOI":"10.1007\/s001250050044"},{"key":"2025073112575667000_13.2.e004885.6","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(03)14571-0"},{"key":"2025073112575667000_13.2.e004885.7","doi-asserted-by":"publisher","DOI":"10.2337\/diab.46.4.726"},{"key":"2025073112575667000_13.2.e004885.8","doi-asserted-by":"publisher","DOI":"10.2337\/diacare.16.9.1254"},{"key":"2025073112575667000_13.2.e004885.9","doi-asserted-by":"publisher","DOI":"10.2337\/dc11-0035"},{"key":"2025073112575667000_13.2.e004885.10","doi-asserted-by":"publisher","DOI":"10.4158\/EP13254.OR"},{"key":"2025073112575667000_13.2.e004885.11","doi-asserted-by":"publisher","DOI":"10.2337\/dc13-3007"},{"key":"2025073112575667000_13.2.e004885.12","doi-asserted-by":"crossref","unstructured":"Tyler Broome D . SAT085 Oral Glucagon-like Peptide-1 Receptor Agonist As Adjuvant Treatment In HNF1A-MODY. Journal of the Endocrine Society 2023;7. doi:10.1210\/jendso\/bvad114.951","DOI":"10.1210\/jendso\/bvad114.951"},{"key":"2025073112575667000_13.2.e004885.13","doi-asserted-by":"crossref","DOI":"10.2337\/dc20-0012","article-title":"Novel Use of GLP-1 Receptor Agonist Therapy in HNF4A-MODY","volume":"43","author":"Broome","year":"2020","journal-title":"Diabetes Care"},{"key":"2025073112575667000_13.2.e004885.14","doi-asserted-by":"crossref","first-page":"e65","DOI":"10.2337\/dc24-0730","article-title":"Use of a Dual GIP\/GLP-1 Receptor Agonist in HNF1A-MODY and HNF4A-MODY","volume":"47","author":"Broome","year":"2024","journal-title":"Diabetes Care"},{"key":"2025073112575667000_13.2.e004885.15","doi-asserted-by":"publisher","DOI":"10.2337\/db20-1989-P"}],"container-title":["BMJ Open Diabetes Research &amp; Care"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1136\/bmjdrc-2024-004885","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,7,31]],"date-time":"2025-07-31T19:58:02Z","timestamp":1753991882000},"score":1,"resource":{"primary":{"URL":"https:\/\/drc.bmj.com\/lookup\/doi\/10.1136\/bmjdrc-2024-004885"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,4]]},"references-count":15,"journal-issue":{"issue":"2","published-online":{"date-parts":[[2025,4,23]]},"published-print":{"date-parts":[[2025,4]]}},"alternative-id":["10.1136\/bmjdrc-2024-004885"],"URL":"https:\/\/doi.org\/10.1136\/bmjdrc-2024-004885","relation":{},"ISSN":["2052-4897"],"issn-type":[{"value":"2052-4897","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,4]]}}}