{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,31]],"date-time":"2026-03-31T19:49:13Z","timestamp":1774986553090,"version":"3.50.1"},"reference-count":13,"publisher":"The Endocrine Society","issue":"11","license":[{"start":{"date-parts":[[2024,10,18]],"date-time":"2024-10-18T00:00:00Z","timestamp":1729209600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2024,10,18]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:p>Permanent neonatal diabetes mellitus (PNDM) is a genetic disorder, characterized by a decrease in endogenous insulin secretion. Therefore, exogenous insulin supplementation plays a central role in controlling glycemia. Although adding a sulfonylurea can help to discontinue insulin, discontinuation is sometimes difficult when the sulfonylurea is administered at older ages. A 24-year-old woman with longstanding PNDM who had poor glycemic control using insulin (47\u2005U\/day) and high-dose glibenclamide (0.6\u2005mg\/kg\/day), had successfully discontinued insulin after initiating the dipeptidyl peptidase-4 inhibitor sitagliptin (50\u2005mg\/day). Additionally, hemoglobin A1c levels decreased by 4.8%. Double dosing of sitagliptin and subsequent switching to the glucagon-like peptide-1 receptor agonist semaglutide (0.25\u2005mg\/week followed by 0.5\u2005mg\/week) further decreased hemoglobin A1c values, with graded improvements in endogenous insulin secretion. There were no episodes of hypoglycemia during which glibenclamide was titrated down from 0.6 to 0.4\u2005mg\/kg\/day. Intra- and inter-day glucose variability as assessed by continuous glucose monitoring was also improved. In patients with PNDM, administration and dose escalation of incretin-based drugs, in addition to a high-dose sulfonylurea, could be a useful treatment strategy. This strategy may be helpful for discontinuing insulin, downtitrating sulfonylureas, and subsequent achievement of better glycemic control regarding long-term stability and short-term variability.<\/jats:p>","DOI":"10.1210\/jcemcr\/luae188","type":"journal-article","created":{"date-parts":[[2024,10,18]],"date-time":"2024-10-18T09:30:41Z","timestamp":1729243841000},"source":"Crossref","is-referenced-by-count":1,"title":["Long-Term Effects of Incretin-Based Drugs on Glycemic Control in Permanent Neonatal Diabetes"],"prefix":"10.1210","volume":"2","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-9837-2755","authenticated-orcid":false,"given":"Ayaka","family":"Oshiro","sequence":"first","affiliation":[{"name":"Department of Medicine and Clinical Science, Graduate School of Medical Sciences, Kyushu University , Fukuoka 812-8582 ,","place":["Japan"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ryoichiro","family":"Aotani","sequence":"additional","affiliation":[{"name":"Department of Medicine and Clinical Science, Graduate School of Medical Sciences, Kyushu University , Fukuoka 812-8582 ,","place":["Japan"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Wakako","family":"Sakamoto","sequence":"additional","affiliation":[{"name":"Department of Medicine and Clinical Science, Graduate School of Medical Sciences, Kyushu University , Fukuoka 812-8582 ,","place":["Japan"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9006-5937","authenticated-orcid":false,"given":"Takanari","family":"Kitazono","sequence":"additional","affiliation":[{"name":"Department of Medicine and Clinical Science, Graduate School of Medical Sciences, Kyushu University , Fukuoka 812-8582 ,","place":["Japan"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8105-7102","authenticated-orcid":false,"given":"Toshiaki","family":"Ohkuma","sequence":"additional","affiliation":[{"name":"Department of Medicine and Clinical Science, Graduate School of Medical Sciences, Kyushu University , Fukuoka 812-8582 ,","place":["Japan"]}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"80","published-online":{"date-parts":[[2024,10,18]]},"reference":[{"issue":"8","key":"2024110705244188900_luae188-B1","doi-asserted-by":"crossref","first-page":"637","DOI":"10.1016\/S2213-8587(18)30106-2","article-title":"Effectiveness and safety of long-term treatment with sulfonylureas in patients with neonatal diabetes due to KCNJ11 mutations: an international cohort study","volume":"6","author":"Bowman","year":"2018","journal-title":"Lancet Diabetes Endocrinol"},{"issue":"7","key":"2024110705244188900_luae188-B2","doi-asserted-by":"crossref","first-page":"1430","DOI":"10.1007\/s00125-015-3593-9","article-title":"Age at the time of sulfonylurea initiation influences treatment outcomes in KCNJ11-related neonatal diabetes","volume":"58","author":"Thurber","year":"2015","journal-title":"Diabetologia"},{"issue":"5","key":"2024110705244188900_luae188-B3","doi-asserted-by":"crossref","first-page":"1213","DOI":"10.1111\/dom.14318","article-title":"An adult patient with permanent neonatal diabetes successfully discontinued insulin therapy after initiating sitagliptin added to sulphonylurea","volume":"23","author":"Oshiro","year":"2021","journal-title":"Diabetes Obes Metab"},{"issue":"5","key":"2024110705244188900_luae188-B4","doi-asserted-by":"crossref","first-page":"744","DOI":"10.3390\/ijms17050744","article-title":"Postprandial C-peptide to glucose ratio as a marker of \u03b2 cell function: implication for the management of type 2 diabetes","volume":"17","author":"Saisho","year":"2016","journal-title":"Int J Mol Sci"},{"issue":"1","key":"2024110705244188900_luae188-B5","doi-asserted-by":"crossref","first-page":"171","DOI":"10.1111\/j.1742-1241.2006.01246.x","article-title":"Efficacy and tolerability of the dipeptidyl peptidase-4 inhibitor sitagliptin as monotherapy over 12 weeks in patients with type 2 diabetes","volume":"61","author":"Scott","year":"2007","journal-title":"Int J Clin Pract"},{"issue":"5","key":"2024110705244188900_luae188-B6","doi-asserted-by":"crossref","first-page":"383","DOI":"10.1507\/endocrj.K09E-272","article-title":"Dose-ranging efficacy of sitagliptin, a dipeptidyl peptidase-4 inhibitor, in Japanese patients with type 2 diabetes mellitus","volume":"57","author":"Iwamoto","year":"2010","journal-title":"Endocr J"},{"issue":"6","key":"2024110705244188900_luae188-B7","doi-asserted-by":"crossref","first-page":"1695","DOI":"10.2337\/db05-1602","article-title":"Chronic inhibition of dipeptidyl peptidase-4 with a sitagliptin analog preserves pancreatic beta-cell mass and function in a rodent model of type 2 diabetes","volume":"55","author":"Mu","year":"2006","journal-title":"Diabetes"},{"issue":"3","key":"2024110705244188900_luae188-B8","first-page":"002352","article-title":"Successful use of a GLP-1 receptor agonist as add-on therapy to sulfonylurea in the treatment of KCNJ11 neonatal diabetes","volume":"8","author":"Hinds\u00f8","year":"2021","journal-title":"Eur J Case Rep Intern Med"},{"key":"2024110705244188900_luae188-B9","doi-asserted-by":"publisher","DOI":"10.1210\/clinem\/dgae213","article-title":"Improvement of \u03b2-cell function after switching from DPP-4 inhibitors to oral semaglutide: sWITCH-SEMA2 post hoc analysis","author":"Nomoto","year":"2024","journal-title":"J Clin Endocrinol Metab"},{"issue":"1","key":"2024110705244188900_luae188-B10","doi-asserted-by":"crossref","first-page":"e002573","DOI":"10.1136\/bmjdrc-2021-002573","article-title":"Time in range, as measured by continuous glucose monitor, as a predictor of microvascular complications in type 2 diabetes: a systematic review","volume":"10","author":"Raj","year":"2022","journal-title":"BMJ Open Diabetes Res Care"},{"issue":"3","key":"2024110705244188900_luae188-B11","doi-asserted-by":"crossref","first-page":"393","DOI":"10.1111\/pedi.12599","article-title":"Hypoglycemia in sulfonylurea-treated KCNJ11-neonatal diabetes: mild-moderate symptomatic episodes occur infrequently but none involving unconsciousness or seizures","volume":"19","author":"Lanning","year":"2018","journal-title":"Pediatr Diabetes"},{"issue":"15","key":"2024110705244188900_luae188-B12","doi-asserted-by":"crossref","first-page":"1410","DOI":"10.1056\/NEJMoa1003795","article-title":"Severe hypoglycemia and risks of vascular events and death","volume":"363","author":"Zoungas","year":"2010","journal-title":"N Engl J Med"},{"issue":"2","key":"2024110705244188900_luae188-B13","doi-asserted-by":"crossref","first-page":"135","DOI":"10.1111\/dom.12587","article-title":"Bi-directional interaction between hypoglycaemia and cognitive impairment in elderly patients treated with glucose-lowering agents: a systematic review and meta-analysis","volume":"18","author":"Mattishent","year":"2016","journal-title":"Diabetes Obes Metab"}],"container-title":["JCEM Case Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/academic.oup.com\/jcemcr\/article-pdf\/2\/11\/luae188\/60396635\/luae188.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/jcemcr\/article-pdf\/2\/11\/luae188\/60396635\/luae188.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,11,7]],"date-time":"2024-11-07T05:24:56Z","timestamp":1730957096000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/jcemcr\/article\/doi\/10.1210\/jcemcr\/luae188\/7826029"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,10,18]]},"references-count":13,"journal-issue":{"issue":"11","published-print":{"date-parts":[[2024,10,18]]}},"URL":"https:\/\/doi.org\/10.1210\/jcemcr\/luae188","relation":{},"ISSN":["2755-1520"],"issn-type":[{"value":"2755-1520","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[2024,11]]},"published":{"date-parts":[[2024,10,18]]},"article-number":"luae188"}}