{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,23]],"date-time":"2026-03-23T06:02:13Z","timestamp":1774245733880,"version":"3.50.1"},"reference-count":51,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2026,3,23]],"date-time":"2026-03-23T00:00:00Z","timestamp":1774224000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100004826","name":"Natural Science Foundation of Beijing Municipality","doi-asserted-by":"publisher","award":["\u4e0d\u30027212034"],"award-info":[{"award-number":["\u4e0d\u30027212034"]}],"id":[{"id":"10.13039\/501100004826","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100011494","name":"Baoding City Science and Technology and Intellectual Property Bureau","doi-asserted-by":"publisher","award":["\u7f16\u53f71951ZF076"],"award-info":[{"award-number":["\u7f16\u53f71951ZF076"]}],"id":[{"id":"10.13039\/501100011494","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Endocrinol."],"abstract":"\n Introduction<\/jats:title>\n \n In this study, we describe surgical indications, procedure selection, and postoperative outcomes in Chinese children with diffuse congenital hyperinsulinism (DCHI). Pancreatic involvement was assessed using\n 18<\/jats:sup>\n F-L-Fluoro-3,4-dihydroxyphenylalanine positron emission tomography\/computed tomography (\n 18<\/jats:sup>\n F\u2013L\u2013DOPA PET\/CT) to support clinical decision-making.\n <\/jats:p>\n <\/jats:sec>\n \n Methods<\/jats:title>\n \n Clinical and genetic characteristics were summarized for 27 children with DCHI, stratified into a surgical group (\n n<\/jats:italic>\n \u00a0=\u00a012) and a non-surgical group (\n n<\/jats:italic>\n \u00a0=\u00a015). Clinical characteristics were summarized descriptively. An exploratory Firth penalized logistic regression was applied to illustrate descriptive patterns in clinical features considered during surgical intervention selection at this center, without implying causal relationships or predictive effects. Metabolic outcomes after subtotal pancreatectomy (STP) and near-total pancreatectomy (NTP) were compared descriptively. Median postoperative follow-up was 2.9 years (range 2.0\u20138.8 years). Neurodevelopmental outcomes were assessed using standardized tools at variable ages. Kaplan\u2013Meier analysis was applied descriptively to illustrate long-term neurological trends.\n <\/jats:p>\n <\/jats:sec>\n \n Results<\/jats:title>\n \n Of the 27 patients with medically refractory diffuse congenital hyperinsulinism, 12 (44.4%) required surgical intervention, including subtotal pancreatectomy (STP, n = 6) and near-total pancreatectomy (NTP,\n n<\/jats:italic>\n \u00a0=\u00a06). The median age at diagnosis was 3 days (IQR 2\u20137), with a male-to-female ratio of 1.25:1. Among the 12 surgical patients, 8 underwent genetic testing, and all (8\/8, 100%) were found to harbor ABCC8\/KCNJ11 mutations. Overall hypoglycemia control patterns were comparable between the NTP and STP groups, although distinct long-term metabolic trade-offs were observed. At the final follow-up, postoperative diabetes (PD) was observed in 16.7% of NTP patients, while long-term neurodevelopmental outcomes were comparable between the surgical and non-surgical groups.\n <\/jats:p>\n <\/jats:sec>\n \n Conclusions<\/jats:title>\n Subtotal and near-total pancreatectomy were not observed to result in major perioperative complications in this cohort of Chinese pediatric patients with medically refractory DCHI. Clinicians may need to carefully balance hypoglycemia control against the frequency of postoperative diabetes when selecting the extent of resection. Early diagnosis, timely hypoglycemia management, and structured long-term follow-up are essential to optimize long-term outcomes.<\/jats:p>\n <\/jats:sec>","DOI":"10.3389\/fendo.2026.1760749","type":"journal-article","created":{"date-parts":[[2026,3,23]],"date-time":"2026-03-23T05:20:45Z","timestamp":1774243245000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":0,"title":["Single-center experience description of surgical management of diffuse congenital hyperinsulinism in a pediatric non-current cohort"],"prefix":"10.3389","volume":"17","author":[{"given":"Xiaoxiang","family":"Li","sequence":"first","affiliation":[{"name":"Department of Endocrinology, Genetics and Metabolism, Beijing Children\u2019s Hospital, Capital Medical University, National Center for Children\u2019s Health","place":["Beijing, China"]},{"name":"Department of Pediatrics, Beijing Luhe Hospital, Capital Medical University","place":["Beijing, China"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Congli","family":"Chen","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, West China Second University Hospital, Sichuan University","place":["Chengdu, China"]},{"name":"Key Laboratory of Birth Defects and Related Diseases of Women and Children, Ministry of Education, West China Second University Hospital, Sichuan University","place":["Chengdu, China"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yangmingyue","family":"Ji","sequence":"additional","affiliation":[{"name":"Department of Endocrinology, Genetics and Metabolism, Beijing Children\u2019s Hospital, Capital Medical University, National Center for Children\u2019s Health","place":["Beijing, China"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yuxi","family":"Wang","sequence":"additional","affiliation":[{"name":"Department of Endocrinology, Genetics and Metabolism, Beijing Children\u2019s Hospital, Capital Medical University, National Center for Children\u2019s Health","place":["Beijing, China"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yanmei","family":"Sang","sequence":"additional","affiliation":[{"name":"Department of Endocrinology, Genetics and Metabolism, Beijing Children\u2019s Hospital, Capital Medical University, National Center for Children\u2019s Health","place":["Beijing, China"]}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1965","published-online":{"date-parts":[[2026,3,23]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","DOI":"10.1097\/MOP.0000000000001243","article-title":"Bridging the gaps: recent advances in diagnosis, care, and outcomes in congenital hyperinsulinism","volume":"35","author":"Rosenfeld","year":"2023","journal-title":"Curr Opin Pediatr"},{"key":"B2","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0228417","article-title":"Using referral rates for genetic testing to determine the incidence 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