{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,20]],"date-time":"2026-08-20T12:58:08Z","timestamp":1787230688229,"version":"build-2736575974"},"reference-count":42,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2025,1,20]],"date-time":"2025-01-20T00:00:00Z","timestamp":1737331200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100002738","name":"Assistance Publique - H\u00f4pitaux de Paris","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100002738","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100019125","name":"Sorbonne Universit\u00e9","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100019125","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["dom-pubs.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Diabetes Obesity Metabolism"],"published-print":{"date-parts":[[2025,4]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Aim<\/jats:title>\n                    <jats:p>To describe the effects of Glucagon\u2010like peptide\u20101 receptor agonists (GLP\u20101RA) in patients with familial partial lipodystrophy (FPLD) assessed in a real\u2010life setting in a national reference network.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Patients and Methods<\/jats:title>\n                    <jats:p>We retrospectively collected clinical and metabolic parameters in patients with FPLD in the French lipodystrophy reference network, who initiated GLP\u20101RA. Data were recorded before, at one\u2010year (12\u2009\u00b1\u20096\u2009months) and at the latest follow\u2010up on GLP\u20101RA therapy (\u226518\u2009months).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Seventy\u2010six patients (89.4% of women), diagnosed with\n                      <jats:italic>LMNA<\/jats:italic>\n                      \u2010related FPLD2 (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u200957),\n                      <jats:italic>PPARG<\/jats:italic>\n                      \u2010related FPLD3 (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u20094),\n                      <jats:italic>PLIN1<\/jats:italic>\n                      \u2010related FPLD4 (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u20095) or FPLD1 (\n                      <jats:italic>n<\/jats:italic>\n                      \u2009=\u200910) initiated GLP\u20101RA therapy between 2008 and 2024. Patients were aged a median (IQR) 48\u2009years (34.5\u201357), body mass index (BMI) was 26.0\u2009kg\/m\n                      <jats:sup>2<\/jats:sup>\n                      (23.9\u201329.5), HbA1c 8.3% (7.5\u20139.3), triglycerides 2.31\u2009mmol\/L (1.62\u20133.88). GLP\u20101RA were used in addition to previously used antidiabetics, 50% of patients being insulin\u2010treated. After one year with GLP\u20101RA therapy, BMI, HbA1c and triglycerides significantly decreased to 25.6\u2009kg\/m\n                      <jats:sup>2<\/jats:sup>\n                      (22.7\u201329.1), 7.3% (6.6\u20138.3) and 1.97\u2009mmol\/L (1.5\u20133.2) respectively (\n                      <jats:italic>p<\/jats:italic>\n                      \u2009&lt;\u20090.001,\n                      <jats:italic>p<\/jats:italic>\n                      \u2009&lt;\u20090.001 and\n                      <jats:italic>p<\/jats:italic>\n                      \u2009&lt;\u20090.01, respectively), without significant changes in other antidiabetic and lipid\u2010lowering drugs. Gamma\u2010glutamyl\u2010transferase and alanine\u2010aminotransferase levels also significantly decreased. Effects on HbA1c, BMI and triglycerides persisted in the long term. One case of acute pancreatitis occurred during follow\u2010up, associated with severe hypertriglyceridemia in a non\u2010observant patient. Gastrointestinal symptoms affected 34% of patients, leading to GLP\u20101RA withdrawal in six patients.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>GLP\u20101RA significantly improved BMI, HbA1c and triglycerides in a large majority of patients with FPLD. Larger and prospective controlled studies are warranted for identification of predictive factors and safety.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1111\/dom.16175","type":"journal-article","created":{"date-parts":[[2025,1,20]],"date-time":"2025-01-20T04:15:42Z","timestamp":1737346542000},"page":"1815-1825","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":15,"title":["Safety and effectiveness in an uncontrolled setting of glucagon\u2010like\u2010peptide\u20101 receptor agonists in patients with familial partial lipodystrophy: Real\u2010life experience from a national reference network"],"prefix":"10.1111","volume":"27","author":[{"ORCID":"https:\/\/orcid.org\/0009-0000-6853-7742","authenticated-orcid":false,"given":"Sophie","family":"Lamothe","sequence":"first","affiliation":[{"name":"Endocrinology Department, Assistance Publique\u2013H\u00f4pitaux de Paris (AP\u2010HP) Saint\u2013Antoine University Hospital, National Reference Centre for Rare Diseases of Insulin Secretion and Insulin Sensitivity (PRISIS)  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ines","family":"Belalem","sequence":"additional","affiliation":[{"name":"Endocrinology Department, Assistance Publique\u2013H\u00f4pitaux de Paris (AP\u2010HP) Saint\u2013Antoine University Hospital, National Reference Centre for Rare Diseases of Insulin Secretion and Insulin Sensitivity (PRISIS)  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marie\u2010Christine","family":"Vantyghem","sequence":"additional","affiliation":[{"name":"Department of Endocrinology, Diabetology and Metabolism Lille University Hospital, University of Lille, INSERM U1190, European Genomic Institute for Diabetes  Lille France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Estelle","family":"Nobecourt","sequence":"additional","affiliation":[{"name":"Department of Endocrinology, Diabetology and Metabolism La R\u00e9union University Hospital  Saint Pierre de la R\u00e9union France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"H\u00e9l\u00e9na","family":"Mosbah","sequence":"additional","affiliation":[{"name":"Department of Endocrinology Diabetology and Nutrition CHU La Mil\u00e9trie  Poitiers France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sophie","family":"B\u00e9liard","sequence":"additional","affiliation":[{"name":"Department of Nutrition, Metabolic Diseases, Endocrinology Aix Marseille University, Inserm, INRA, C2VN, La Conception Hospital  Marseille France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Brigitte","family":"Delemer","sequence":"additional","affiliation":[{"name":"Department of Endocrinology, Diabetes and Nutrition Reims University Hospital, Hospital Robert\u2010Debr\u00e9  Reims 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