title: Hyperinsulinemic Hypoglycemia.
abstract: In hyperinsulinemic hypoglycemia (HH) there is dysregulation of insulin secretion from pancreatic β-cells. Insulin secretion becomes inappropriate for the level of blood glucose leading to severe hypoglycemia. HH is associated with a high risk of brain injury because insulin inhibits lipolysis and ketogenesis thus preventing the generation of alternative brain substrates (such as ketone bodies). Hence HH must be diagnosed as soon as possible and the management instituted appropriately to prevent brain damage. This article reviews the mechanisms of glucose physiology in the newborn, the mechanisms of insulin secretion, the etiologic types of HH, and its management.
authors.affiliation: Developmental Endocrinology Research Group, Molecular Genetics Unit, Institute of Child Health, University College London, 30 Guilford Street, London WC1N 1EH, UK.
authors.forename: Maria
authors.initials: M
authors.lastname: Güemes
authors.affiliation: Developmental Endocrinology Research Group, Molecular Genetics Unit, Institute of Child Health, University College London, 30 Guilford Street, London WC1N 1EH, UK. Electronic address: Khalid.Hussain@ucl.ac.uk.
authors.forename: Khalid
authors.initials: K
authors.lastname: Hussain
mesh_terms: D001786:Blood Glucose / Q000032:analysis; D001927:Brain Diseases / Q000209:etiology / Q000517:prevention & control; D044903:Congenital Hyperinsulinism / Q000150:complications / Q000175:diagnosis* / Q000235:genetics / Q000628:therapy*; D003952:Diagnostic Imaging; D005820:Genetic Testing; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007231:Infant, Newborn; D007328:Insulin / Q000378:metabolism*; D000078790:Insulin Secretion; D050417:Insulin-Secreting Cells / Q000378:metabolism*; D010180:Pancreatectomy; D018027:Receptors, Glucagon / Q000037:antagonists & inhibitors; D020123:Sirolimus / Q000627:therapeutic use
chemical_list: D001786:Blood Glucose; D007166:Immunosuppressive Agents; D007328:Insulin; D018027:Receptors, Glucagon; D020123:Sirolimus
keywords: 18F-l-dihydroxyphenylalanine positron emission tomographic scan; Diazoxide; Hyperinsulinism; Hypoglycemia; Mammalian target of rapamycin (mTOR) inhibitor; Octreotide; Pancreatectomy