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The in cidence of CHI in the general population ranges from 1:30,000 to 1:50,000 live births. Timely diagnosis and treatment of CHI are critical, as persistent hypoglycemia can lead to irreversible damage to the central nervous system. This review summa rizes current understanding of the etiology and pathogenesis of CHI and presents an up-to-date classification of CHI based on duration, etiology, histological form, and response to diazoxide therapy. A detailed diagnostic algorithm is provided, including provocative tests, molecular genetic testing, and imaging studies. The stages of conservative treatment and indi cations for surgical treatment are discussed. The review is based on updated clinical guidelines and is intended for practicing physicians \u2014 pediatricians, endocrinologists, and neonatologists \u2014 who care for children with hypoglycemic conditions.<\/jats:p>","DOI":"10.14341\/probl13823","type":"journal-article","created":{"date-parts":[[2026,9,10]],"date-time":"2026-09-10T15:36:30Z","timestamp":1789054590000},"page":"80-97","update-policy":"https:\/\/doi.org\/10.14341\/crossmarkpolicy2018","source":"Crossref","is-referenced-by-count":0,"title":["Clinical practice guidelines \"Congenital hyperinsulinism\""],"prefix":"10.14341","volume":"72","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3127-5974","authenticated-orcid":true,"given":"A. V.","family":"Bolmasova","sequence":"first","affiliation":[{"name":"Endocrinology Research Centre"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8610-821X","authenticated-orcid":true,"given":"E. 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Arch Dis Child Fetal Neonatal Ed. 2000;82(2):F79-F86. doi: https:\/\/doi.org\/10.1136\/fn.82.2.f79","DOI":"10.1136\/fn.82.2.F79"},{"key":"ref2","doi-asserted-by":"crossref","unstructured":"\u0411\u043e\u043b\u043c\u0430\u0441\u043e\u0432\u0430 \u0410.\u0412., \u041c\u0435\u043b\u0438\u043a\u044f\u043d \u041c.\u0410., \u041a\u0440\u044b\u043b\u043e\u0432\u0430 \u041d.\u0410., \u0418\u043e\u043d\u043e\u0432 \u041e.\u0412., \u0420\u044e\u043c\u0438\u043d\u0430 \u0418.\u0418., \u0411\u043e\u043a\u0435\u0440\u0438\u044f \u0415.\u041b., \u041f\u0435\u043a\u0430\u0440\u0435\u0432\u0430 \u041d.\u0410., \u0414\u0435\u0433\u0442\u044f\u0440\u0435\u0432\u0430 \u0410.\u0412. \u0422\u0440\u0430\u043d\u0437\u0438\u0442\u043e\u0440\u043d\u044b\u0435 \u0432\u0430\u0440\u0438\u0430\u043d\u0442\u044b \u0432\u0440\u043e\u0436\u0434\u0435\u043d\u043d\u043e\u0433\u043e \u0433\u0438\u043f\u0435\u0440\u0438\u043d\u0441\u0443\u043b\u0438\u043d\u0438\u0437\u043c\u0430 \u0443 \u043d\u043e\u0432\u043e\u0440\u043e\u0436\u0434\u0435\u043d\u043d\u044b\u0445 \u0434\u0435\u0442\u0435\u0439. \u041f\u0440\u043e\u0431\u043b\u0435\u043c\u044b \u042d\u043d\u0434\u043e\u043a\u0440\u0438\u043d\u043e\u043b\u043e\u0433\u0438\u0438. 2020;66(4):61-67 doi: https:\/\/doi.org\/10.14341\/probl12572","DOI":"10.14341\/probl12572"},{"key":"ref3","doi-asserted-by":"crossref","unstructured":"Prentki M, Matschinsky FM, Madiraju SR. Metabolic signaling in fuel-induced insulin secretion. Cell Metab. 2013;18(2):162-185. doi: https:\/\/doi.org\/10.1016\/j.cmet.2013.05.018","DOI":"10.1016\/j.cmet.2013.05.018"},{"key":"ref4","doi-asserted-by":"crossref","unstructured":"Vajravelu ME, De Le\u00f3n DD. Genetic characteristics of patients with congenital hyperinsulinism. Curr Opin Pediatr. 2018;30(4):568-575. doi: https:\/\/doi.org\/10.1097\/MOP.0000000000000645","DOI":"10.1097\/MOP.0000000000000645"},{"key":"ref5","doi-asserted-by":"crossref","unstructured":"\u041c\u0435\u043b\u0438\u043a\u044f\u043d \u041c.\u0410., \u041a\u0430\u0440\u0435\u0432\u0430 \u041c.\u0410., \u041f\u0435\u0442\u0440\u044f\u0439\u043a\u0438\u043d\u0430 \u0415.\u0415., \u0412\u043e\u043b\u043a\u043e\u0432 \u0418.\u042d., \u0410\u0432\u0435\u0440\u044c\u044f\u043d\u043e\u0432\u0430 \u042e.\u0412., \u041a\u043e\u043b\u043e\u043c\u0438\u043d\u0430 \u0418.\u0413., \u0413\u0443\u0440\u0435\u0432\u0438\u0447 \u041b.\u0415., \u041f\u0435\u0442\u0435\u0440\u043a\u043e\u0432\u0430 \u0412.\u0410., Brusgaard K., Christesen H, \u0414\u0435\u0434\u043e\u0432 \u0418.\u0418. \u0412\u0440\u043e\u0436\u0434\u0435\u043d\u043d\u044b\u0439 \u0433\u0438\u043f\u0435\u0440\u0438\u043d\u0441\u0443\u043b\u0438\u043d\u0438\u0437\u043c. \u0420\u0435\u0437\u0443\u043b\u044c\u0442\u0430\u0442\u044b \u043c\u043e\u043b\u0435\u043a\u0443\u043b\u044f\u0440\u043d\u043e-\u0433\u0435\u043d\u0435\u0442\u0438\u0447\u0435\u0441\u043a\u0438\u0445 \u0438\u0441\u0441\u043b\u0435\u0434\u043e\u0432\u0430\u043d\u0438\u0439 \u0432 \u0440\u043e\u0441\u0441\u0438\u0439\u0441\u043a\u043e\u0439 \u043f\u043e\u043f\u0443\u043b\u044f\u0446\u0438\u0438. \u041f\u0440\u043e\u0431\u043b\u0435\u043c\u044b \u042d\u043d\u0434\u043e\u043a\u0440\u0438\u043d\u043e\u043b\u043e\u0433\u0438\u0438. 2012;58(2):3-9. doi: https:\/\/doi.org\/10.14341\/probl20125823-9","DOI":"10.14341\/probl20125823-9"},{"key":"ref6","doi-asserted-by":"crossref","unstructured":"\u041c\u0435\u043b\u0438\u043a\u044f\u043d \u041c.\u0410., \u0422\u044e\u043b\u044c\u043f\u0430\u043a\u043e\u0432 \u0410.\u041d., \u041a\u0430\u0440\u0435\u0432\u0430 \u041c.\u0410. \u0421\u0435\u043c\u0435\u0439\u043d\u044b\u0439 \u0441\u043b\u0443\u0447\u0430\u0439 \u0432\u0440\u043e\u0436\u0434\u0435\u043d\u043d\u043e\u0433\u043e \u0433\u0438\u043f\u0435\u0440\u0438\u043d\u0441\u0443\u043b\u0438\u043d\u0438\u0437\u043c\u0430, \u0430\u0441\u0441\u043e\u0446\u0438\u0438\u0440\u043e\u0432\u0430\u043d\u043d\u043e\u0433\u043e \u0441 \u043c\u0443\u0442\u0430\u0446\u0438\u0435\u0439 \u0432 \u0433\u0435\u043d\u0435 GLUD1. \u041f\u0440\u043e\u0431\u043b\u0435\u043c\u044b \u042d\u043d\u0434\u043e\u043a\u0440\u0438\u043d\u043e\u043b\u043e\u0433\u0438\u0438. 2017;63(3):195-200. https:\/\/doi.org\/10.14341\/probl2017633195-200","DOI":"10.14341\/probl2017633195-200"},{"key":"ref7","doi-asserted-by":"crossref","unstructured":"Thomas PM, Cote GJ, Wohllk N, et al. Mutations in the sulfonylurea receptor gene in familial persistent hyperinsulinemic hypoglycemia of infancy. Science. 1995;268(5209):426-429. doi: https:\/\/doi.org\/10.1126\/science.7716548","DOI":"10.1126\/science.7716548"},{"key":"ref8","doi-asserted-by":"crossref","unstructured":"Saint-Martin C, Arnoux JB, de Lonlay P, Bellann\u00e9 Chantelot C. KATP channel mutations in congenital hyperinsulinism. Semin Pediatr Surg. 2011;20(1):18-22. doi: https:\/\/doi.org\/10.1053\/j.sempedsurg.2010.10.012","DOI":"10.1053\/j.sempedsurg.2010.10.012"},{"key":"ref9","doi-asserted-by":"crossref","unstructured":"Pinney SE, MacMullen C, Becker S, et al. Clinical characteristics and biochemical mechanisms of congenital hyperinsulinism associated with dominant KATP channel mutations. J Clin Invest. 2008;118(8):2877-2886. doi: https:\/\/doi.org\/10.1172\/JCI35414","DOI":"10.1172\/JCI35414"},{"key":"ref10","doi-asserted-by":"crossref","unstructured":"de Lonlay P, Fournet JC, Rahier J, et al. Somatic deletion of the imprinted 11p15 region in sporadic persistent hyperinsulinemic hypoglycemia of infancy is specific of focal adenomatous hyperplasia and endorses partial pancreatectomy. J Clin Invest. 1997;100(4):802-807. doi: https:\/\/doi.org\/10.1172\/JCI119594","DOI":"10.1172\/JCI119594"},{"key":"ref11","doi-asserted-by":"crossref","unstructured":"Senniappan S, Shanti B, James C, Hussain K. Hyperinsulinaemic hypoglycaemia: genetic mechanisms, diagnosis and management. J Inherit Metab Dis. 2012;35(4):589-601. doi: https:\/\/doi.org\/10.1007\/s10545-011-9441-2","DOI":"10.1007\/s10545-011-9441-2"},{"key":"ref12","doi-asserted-by":"crossref","unstructured":"Tung JY, Boodhansingh K, Stanley CA, De Le\u00f3n DD. Clinical heterogeneity of hyperinsulinism due to HNF1A and HNF4A mutations. Pediatr Diabetes. 2018;19(5):910-916. doi: https:\/\/doi.org\/10.1111\/pedi.12655","DOI":"10.1111\/pedi.12655"},{"key":"ref13","doi-asserted-by":"crossref","unstructured":"Ferrara CT, Boodhansingh KE, Paradies E, et al. Novel Hypoglycemia Phenotype in Congenital Hyperinsulinism Due to Dominant Mutations of Uncoupling Protein 2. J Clin Endocrinol Metab. 2017;102(3):942-949. doi: https:\/\/doi.org\/10.1210\/jc.2016-3164","DOI":"10.1210\/jc.2016-3164"},{"key":"ref14","doi-asserted-by":"crossref","unstructured":"Pinney SE, Ganapathy K, Bradfield J, et al. Dominant form of congenital hyperinsulinism maps to HK1 region on 10q. Horm Res Paediatr. 2013;80(1):18-27. doi: https:\/\/doi.org\/10.1159\/000351943.","DOI":"10.1159\/000351943"},{"key":"ref15","doi-asserted-by":"crossref","unstructured":"De Leon DD, Stanley CA. Congenital Hypoglycemia Disorders: New Aspects of Etiology, Diagnosis, Treatment and Outcomes: Highlights of the Proceedings of the Congenital Hypoglycemia Disorders Symposium, Philadelphia April 2016. Pediatr Diabetes. 2017;18(1):3-9. doi: https:\/\/doi.org\/10.1111\/pedi.12453","DOI":"10.1111\/pedi.12453"},{"key":"ref16","doi-asserted-by":"crossref","unstructured":"Cabezas OR, Flanagan SE, Stanescu H, et al. Polycystic Kidney Disease with Hyperinsulinemic Hypoglycemia Caused by a Promoter Mutation in Phosphomannomutase 2. J Am Soc Nephrol. 2017;28(8):2529-2539. doi: https:\/\/doi.org\/10.1681\/ASN.2016121312","DOI":"10.1681\/ASN.2016121312"},{"key":"ref17","doi-asserted-by":"crossref","unstructured":"Flanagan SE, Vairo F, Johnson MB, et al. A CACNA1D mutation in a patient with persistent hyperinsulinaemic hypoglycaemia, heart defects, and severe hypotonia. Pediatr Diabetes. 2017;18(4):320-323. doi: https:\/\/doi.org\/10.1111\/pedi.12512","DOI":"10.1111\/pedi.12512"},{"key":"ref18","doi-asserted-by":"crossref","unstructured":"Giri D, Vignola ML, Gualtieri A, et al. Novel FOXA2 mutation causes Hyperinsulinism, Hypopituitarism with Craniofacial and Endoderm derived organ abnormalities. Hum Mol Genet. 2017;26(22):4315-4326. doi: https:\/\/doi.org\/10.1093\/hmg\/ddx318","DOI":"10.1093\/hmg\/ddx318"},{"key":"ref19","unstructured":"\u041c\u0435\u043b\u0438\u043a\u044f\u043d \u041c.\u0410. \u0412\u0440\u043e\u0436\u0434\u0435\u043d\u043d\u044b\u0439 \u0433\u0438\u043f\u0435\u0440\u0438\u043d\u0441\u0443\u043b\u0438\u043d\u0438\u0437\u043c: \u043c\u043e\u043b\u0435\u043a\u0443\u043b\u044f\u0440\u043d\u0430\u044f \u043e\u0441\u043d\u043e\u0432\u0430, \u043a\u043b\u0438\u043d\u0438\u0447\u0435\u0441\u043a\u0438\u0435 \u043e\u0441\u043e\u0431\u0435\u043d\u043d\u043e\u0441\u0442\u0438 \u0438 \u043f\u0435\u0440\u0441\u043e\u043d\u0430\u043b\u0438\u0437\u0438\u0440\u043e\u0432\u0430\u043d\u043d\u043e\u0435 \u043b\u0435\u0447\u0435\u043d\u0438\u0435. \u0414\u0438\u0441\u0441\u0435\u0440\u0442\u0430\u0446\u0438\u044f \u0434\u043e\u043a\u0442\u043e\u0440\u0430 \u043c\u0435\u0434\u0438\u0446\u0438\u043d\u0441\u043a\u0438\u0445 \u043d\u0430\u0443\u043a. 2019 \u0433\u043e\u0434."},{"key":"ref20","doi-asserted-by":"crossref","unstructured":"Rahier J, Guiot Y, Sempoux C. Persistent Hypoglycaemia in a Turner Syndrome with Ring (X). Case Rep Pediatr. 2015; hyperinsulinaemic hypoglycaemia of infancy: a heterogeneous syndrome unrelated to nesidioblastosis. Arch Dis Child Fetal Neonatal Ed. 2000;82(2):F108-F112. doi: https:\/\/doi.org\/10.1136\/fn.82.2.f108","DOI":"10.1136\/fn.82.2.F108"},{"key":"ref21","doi-asserted-by":"crossref","unstructured":"Arnoux JB, Verkarre V, Saint-Martin C, et al. Congenital hyperinsulinism: current trends in diagnosis and therapy. Orphanet J Rare Dis. 2011;6:63. Published 2011 Oct 3. doi: https:\/\/doi.org\/10.1186\/1750-1172-6-63","DOI":"10.1186\/1750-1172-6-63"},{"key":"ref22","doi-asserted-by":"crossref","unstructured":"Palladino AA, Bennett MJ, Stanley CA. Hyperinsulinism in infancy and childhood: when an insulin level is not always enough. Clin Chem. 2008;54(2):256-263. doi: https:\/\/doi.org\/10.1373\/clinchem.2007.098988","DOI":"10.1373\/clinchem.2007.098988"},{"key":"ref23","doi-asserted-by":"crossref","unstructured":"Choufani S, Shuman C, Weksberg R. Beckwith-Wiedemann syndrome. Am J Med Genet C Semin Med Genet. 2010;154C(3):343-354. doi: https:\/\/doi.org\/10.1002\/ajmg.c.30267","DOI":"10.1002\/ajmg.c.30267"},{"key":"ref24","doi-asserted-by":"crossref","unstructured":"Subbarayan A, Hussain K. Hypoglycemia in Kabuki syndrome. Am J Med Genet A. 2014;164A(2):467-471. doi: https:\/\/doi.org\/10.1002\/ajmg.a.36256","DOI":"10.1002\/ajmg.a.36256"},{"key":"ref25","doi-asserted-by":"crossref","unstructured":"\u0411\u0435\u043d\u0438\u043d\u0430 \u0410.\u0420., \u041c\u0435\u043b\u0438\u043a\u044f\u043d \u041c.\u0410. \u0412\u0440\u043e\u0436\u0434\u0435\u043d\u043d\u044b\u0439 \u0433\u0438\u043f\u0435\u0440\u0438\u043d\u0441\u0443\u043b\u0438\u043d\u0438\u0437\u043c \u0432 \u0441\u043e\u0441\u0442\u0430\u0432\u0435 \u0441\u0438\u043d\u0434\u0440\u043e\u043c\u0430 \u041a\u0430\u0431\u0443\u043a\u0438. \u041f\u0440\u043e\u0431\u043b\u0435\u043c\u044b \u042d\u043d\u0434\u043e\u043a\u0440\u0438\u043d\u043e\u043b\u043e\u0433\u0438\u0438. 2022;68(5):91-96. doi: https:\/\/doi.org\/10.14341\/probl13145","DOI":"10.14341\/probl13145"},{"key":"ref26","doi-asserted-by":"crossref","unstructured":"Nakamura Y, Takagi M, Yoshihashi H, et al. A case with neonatal hyperinsulinemic hypoglycemia: It is a characteristic complication of Sotos syndrome. Am J Med Genet A. 2015;167A(5):1171-1174. doi: https:\/\/doi.org\/10.1002\/ajmg.a.36996","DOI":"10.1002\/ajmg.a.36996"},{"key":"ref27","doi-asserted-by":"crossref","unstructured":"Cappella M, Graziani V, Pragliola A, et al. Hyperinsulinemic 2015:561974. doi: https:\/\/doi.org\/10.1155\/2015\/561974","DOI":"10.1155\/2015\/561974"},{"key":"ref28","doi-asserted-by":"crossref","unstructured":"Kapoor RR, James C, Hussain K. Hyperinsulinism in developmental syndromes. Endocr Dev. 2009;14:95-113. doi: https:\/\/doi.org\/10.1159\/000207480","DOI":"10.1159\/000207480"},{"key":"ref29","doi-asserted-by":"crossref","unstructured":"Ludwig A, Ziegenhorn K, Empting S, et al. Glucose metabolism and neurological outcome in congenital hyperinsulinism. Semin Pediatr Surg. 2011;20(1):45-49. doi: https:\/\/doi.org\/10.1053\/j.sempedsurg.2010.10.005","DOI":"10.1053\/j.sempedsurg.2010.10.005"},{"key":"ref30","doi-asserted-by":"crossref","unstructured":"Thornton PS, Stanley CA, De Leon DD, et al. Recommendations from the Pediatric Endocrine Society for Evaluation and Management of Persistent Hypoglycemia in Neonates, Infants, and Children. J Pediatr. 2015;167(2):238-245. doi: https:\/\/doi.org\/10.1016\/j.jpeds.2015.03.057.","DOI":"10.1016\/j.jpeds.2015.03.057"},{"key":"ref31","doi-asserted-by":"crossref","unstructured":"Demirbilek H, Hussain K. Congenital Hyperinsulinism: Diagnosis and Treatment Update. J Clin Res Pediatr Endocrinol. 2017;9(Suppl 2):69-87. doi: https:\/\/doi.org\/10.4274\/jcrpe.2017.S007","DOI":"10.4274\/jcrpe.2017.S007"},{"key":"ref32","doi-asserted-by":"crossref","unstructured":"De Leon DD, Arnoux JB, Banerjee I, et al. International Guidelines for the Diagnosis and Management of Hyperinsulinism. Horm Res Paediatr. 2024;97(3):279-298. doi: https:\/\/doi.org\/10.1159\/000531766","DOI":"10.1159\/000531766"},{"key":"ref33","doi-asserted-by":"crossref","unstructured":"Banerjee I, Salomon-Estebanez M, Shah P, Nicholson J, Cosgrove KE, Dunne MJ. Therapies and outcomes of congenital hyperinsulinism induced hypoglycaemia. Diabet Med. 2019;36(1):9-21. doi: https:\/\/doi.org\/10.1111\/dme.13823.","DOI":"10.1111\/dme.13823"},{"key":"ref34","doi-asserted-by":"crossref","unstructured":"Kapoor RR, Flanagan SE, James C, Shield J, Ellard S, Hussain K. Hyperinsulinaemic hypoglycaemia. Arch Dis Child. 2009;94(6):450-457. doi: https:\/\/doi.org\/10.1136\/adc.2008.148171","DOI":"10.1136\/adc.2008.148171"},{"key":"ref35","doi-asserted-by":"crossref","unstructured":"Kapoor RR, James C, Hussain K. Advances in the diagnosis and management of hyperinsulinemic hypoglycemia. Nat Clin Pract Endocrinol Metab. 2009;5(2):101-112. doi: https:\/\/doi.org\/10.1038\/ncpendmet1046","DOI":"10.1038\/ncpendmet1046"},{"key":"ref36","doi-asserted-by":"crossref","unstructured":"Mohnike K, Blankenstein O, Christesen HT, et al. Proposal for a standardized protocol for 18F-DOPA-PET (PET\/CT) in congenital hyperinsulinism. Horm Res. 2006;66(1):40-42. doi: https:\/\/doi.org\/10.1159\/000093471","DOI":"10.1159\/000093471"},{"key":"ref37","doi-asserted-by":"crossref","unstructured":"Hardy OT, Hernandez-Pampaloni M, Saffer JR, et al. Accuracy of [18F] fluorodopa positron emission tomography for diagnosing and localizing focal congenital hyperinsulinism. J Clin Endocrinol Metab. 2007;92(12):4706-4711. doi: https:\/\/doi.org\/10.1210\/jc.2007-1637","DOI":"10.1210\/jc.2007-1637"},{"key":"ref38","doi-asserted-by":"crossref","unstructured":"Meintjes M, Endozo R, Dickson J, et al. 18F-DOPA PET and enhanced CT imaging for congenital hyperinsulinism: initial UK experience from a technologist's perspective. 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Long-term medical treatment in congenital hyperinsulinism: a descriptive analysis in a large cohort of patients from different clinical centers. Orphanet J Rare Dis. 2015;10:150. Published 2015 Nov 25. doi: https:\/\/doi.org\/10.1186\/s13023-015-0367-x","DOI":"10.1186\/s13023-015-0367-x"},{"key":"ref55","doi-asserted-by":"crossref","unstructured":"Laje P, Halaby L, Adzick NS, Stanley CA. Necrotizing enterocolitis in neonates receiving octreotide for the management of congenital hyperinsulinism. 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Both Low Blood Glucose and Insufficient Treatment Confer Risk of Neurodevelopmental Impairment in Congenital Hyperinsulinism: A Multinational Cohort Study. Front Endocrinol (Lausanne). 2017;8:156. Published 2017 Jul 10. doi: https:\/\/doi.org\/10.3389\/fendo.2017.00156","DOI":"10.3389\/fendo.2017.00156"},{"key":"ref63","doi-asserted-by":"crossref","unstructured":"Mazor-Aronovitch K, Gillis D, Lobel D, et al. Long-term neurodevelopmental outcome in conservatively treated congenital hyperinsulinism. Eur J Endocrinol. 2007;157(4):491-497. doi: https:\/\/doi.org\/10.1530\/EJE-07-0445","DOI":"10.1530\/EJE-07-0445"},{"key":"ref64","doi-asserted-by":"crossref","unstructured":"Arya VB, Flanagan SE, Kumaran A, et al. Clinical and molecular characterisation of hyperinsulinaemic hypoglycaemia in infants born small-for-gestational age. Arch Dis Child Fetal Neonatal Ed. 2013;98(4):F356-F358. doi: https:\/\/doi.org\/10.1136\/archdischild-2012-302880","DOI":"10.1136\/archdischild-2012-302880"},{"key":"ref65","doi-asserted-by":"crossref","unstructured":"Giouleka S, Gkiouleka M, Tsakiridis I, et al. Diagnosis and Management of Neonatal Hypoglycemia: A Comprehensive Review of Guidelines. Children (Basel). 2023;10(7):1220. Published 2023 Jul 14. doi: https:\/\/doi.org\/10.3390\/children10071220","DOI":"10.3390\/children10071220"},{"key":"ref66","unstructured":"Abramowski A, Ward R, Hamdan AH. Neonatal Hypoglycemia. In: StatPearls. 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