{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,13]],"date-time":"2026-08-13T20:37:09Z","timestamp":1786653429061,"version":"build-2736575974"},"reference-count":88,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"1","content-domain":{"domain":["lww.com","ovid.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2013,1]]},"abstract":"<jats:p>\n                    Congenital hyperinsulinism (CHI) is the result of unregulated insulin secretion from the pancreatic \u03b2-cells leading to severe hypoglycaemia. In these patients it is important to make an accurate diagnosis and initiate the appropriate management so as to avoid hypoglycemic episodes and prevent the potentially associated complications like epilepsy, neurological impairment and cerebral palsy. At a genetic level abnormalities in eight different genes (\n                    <jats:italic toggle=\"yes\">ABCC8, KCNJ11, GLUD1, GCK, HADH, SLC16A1, HNF4A<\/jats:italic>\n                    and\n                    <jats:italic toggle=\"yes\">UCP2<\/jats:italic>\n                    ) have been reported with CHI. Loss of function mutations in\n                    <jats:italic toggle=\"yes\">ABCC8\/KCNJ11<\/jats:italic>\n                    lead to the most severe forms of CHI which are usually medically unresponsive. At a histological level there are two major subgroups, diffuse and focal, each with a different genetic etiology. The focal form is sporadic in inheritance and is localized to a small region of the pancreas whereas the diffuse form is inherited in an autosomal recessive (or dominant) manner. Imaging using a specialized positron emission tomography scan with the isotope fluroine-18 L-3, 4-dihydroxyphenyalanine (18F-DOPA-PET-CT) is used to accurately locate the focal lesion pre-operatively and if removed can cure the patient from hypoglycemia. Understanding the molecular mechanisms, the histological basis, improvements in imaging modalities and surgical techniques have all improved the management of patients with CHI.\n                  <\/jats:p>","DOI":"10.4103\/2230-8210.107822","type":"journal-article","created":{"date-parts":[[2013,2,27]],"date-time":"2013-02-27T00:53:50Z","timestamp":1361926430000},"page":"19-30","update-policy":"https:\/\/doi.org\/10.1097\/lww.0000000000001000","source":"Crossref","is-referenced-by-count":46,"title":["The molecular mechanisms, diagnosis and management of congenital hyperinsulinism"],"prefix":"10.4103","volume":"17","author":[{"given":"Senthil","family":"Senniappan","sequence":"first","affiliation":[{"name":"Department of Pediatric Endocrinology, Great Ormond Street Hospital for Children NHS Trust WC1N 3JH and Institute of Child Health, University College London, WC1N 1EH, United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ved Bhushan","family":"Arya","sequence":"additional","affiliation":[{"name":"Department of Pediatric Endocrinology, Great Ormond Street Hospital for Children NHS Trust WC1N 3JH and Institute of Child Health, University College London, WC1N 1EH, United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Khalid","family":"Hussain","sequence":"additional","affiliation":[{"name":"Department of Pediatric Endocrinology, Great Ormond Street Hospital for Children NHS Trust WC1N 3JH and Institute of Child Health, University College London, WC1N 1EH, United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","reference":[{"key":"R1-20260327","doi-asserted-by":"crossref","first-page":"F98","DOI":"10.1136\/fn.82.2.F98","article-title":"Practical management of hyperinsulinism in infancy","volume":"82","author":"Aynsley-Green","year":"2000","journal-title":"Arch Dis Child Fetal Neonatal 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