Clinical features and insulin regulation in infants with a syndrome of prolonged neonatal hyperinsulinism

Enzymic determination of d (−)-β-hydroxybutyric acid and acetoacetic acid in blood

Williamson, D. H., Mellanby, J., & Krebs, H. (1962) · Biochemical Journal · doi:10.1042/bj0820090 · Cited by 1,398

Births: final data for 2000.

Martin, J. A., Hamilton, B. E., Ventura, S. J., Menacker, F., & Park, M. M. (2002) · PubMed · Cited by 892

Glucose Catabolism in Cancer Cells

Mathupala, S. P., Rempel, A., & Pedersen, P. L. (2001) · Journal of Biological Chemistry · doi:10.1074/jbc.m108181200 · Cited by 386

Hyperinsulinism in short-chain L-3-hydroxyacyl-CoA dehydrogenase deficiency reveals the importance of β-oxidation in insulin secretion

Clayton, P. T., Eaton, S. D., Aynsley-Green, A., Edginton, M., Hussain, K., Krywawych, S., Datta, V., Malingré, H. E. M., Berger, R., & van den Berg, I. E. T. (2001) · Journal of Clinical Investigation · doi:10.1172/jci200111294 · Cited by 311

INCIDENCE OF HYPOGLYCEMIA IN NEWBORN INFANTS CLASSIFIED BY BIRTH WEIGHT AND GESTATIONAL AGE

Lubchenco, L. O., & Bard, H. (1971) · PEDIATRICS · doi:10.1542/peds.47.5.831 · Cited by 289

Hypoxia-Inducible Factor 1: Control of Oxygen Homeostasis in Health and Disease

Semenza, G. L. (2001) · Pediatric Research · doi:10.1203/00006450-200105000-00002 · Cited by 281

Glucose Catabolism in Cancer Cells.

Mathupala, S. P., Rempel, A., & Pedersen, P. L. (1995) · Journal of Biological Chemistry · doi:10.1074/jbc.270.28.16918 · Cited by 229

Genetics of neonatal hyperinsulinism

Gläser, B., THORNTON, P. S., Otonkoski, T., & Junien, C. (2000) · Archives of Disease in Childhood Fetal & Neonatal · doi:10.1136/fn.82.2.f79 · Cited by 220

HYPERINSULINISM IN INFANTS AND CHILDREN

Stanley, C. A. (1997) · Pediatric Clinics of North America · doi:10.1016/s0031-3955(05)70481-8 · Cited by 209

A point mutation inactivating the sulfonylurea receptor causes the severe form of persistent hyperinsulinemic hypoglycemia of infancy in Finland.

Otonkoski, T., Ämmälä, C., Huopio, H., Cote, G. J., Chapman, J., Cosgrove, K. E., Ashfield, R., Huang, E. S., Komulainen, J., Ashcroft, F. M., et al. (1999) · Diabetes · doi:10.2337/diabetes.48.2.408 · Cited by 155

Does the use of diazoxide for hyperinsulinaemic hypoglycaemia increase the risk of necrotising enterocolitis in neonates?

Solís‐García, G., Yeung, T., & Jasani, B. (2023) · Archives of Disease in Childhood · doi:10.1136/archdischild-2023-325726 · Cited by 3

A Brief Review of Hyperinsulinism in Small for Gestational Age Infants

Kumar, J. (2017) · Journal of Medical Science And clinical Research · doi:10.18535/jmscr/v5i1.29 · Cited by 3

Diazoxide toxicity with hypoglycemia in infants with trisomy 13

Nagaoka, M., Akaishi, M., Iida, K., & Yoshida, T. (2021) · Pediatrics & Neonatology · doi:10.1016/j.pedneo.2021.06.010 · Cited by 3

Experience of Octreotide Therapy for Hyperinsulinemic Hypoglycemia in Neonates Born Small for Gestational Age: A Case Series

Pan, S.-N., Zhang, M., & Li, Y. (2015) · Hormone Research in Paediatrics · doi:10.1159/000441108 · Cited by 3

What’s in a name? That which we call perinatal stress hyperinsulinism by any other name would smell so bitter

THORNTON, P. S., & León, D. D. D. (2021) · Journal of Perinatology · doi:10.1038/s41372-021-01056-z · Cited by 3

Watchful waiting versus pharmacological management of small-for-gestational-age infants with hyperinsulinemic hypoglycemia

Chandran, S., Jaya-Bodestyne, S. L., Rajadurai, V. S., Saffari, S. E., Chua, M. C., & Yap, F. K. P. (2023) · Frontiers in Endocrinology · doi:10.3389/fendo.2023.1163591 · Cited by 2

VZTRAJAJOČA HIPOGLIKEMIJA NOVOROJENČKA – PRIKAZ PRIMERA DEKLICE Z BECKWITH-WIEDEMANNOVIM SINDROMOM

Kavčič, M., Krivec, J. L., & Stefanija, M. A. (2022) · Slovenska pediatrija revija pediatrov Slovenije in specialistov šolske ter visokošolske medicine Slovenije · doi:10.38031/slovpediatr-2022-2-03 · Cited by 2

Fetal Erythroblastosis May Be an Indicator of Neonatal Transient Hyperinsulinism

Mizumoto, H., Iki, Y., Yamashita, S., Kawai, M., Katayama, T., & Hata, D. (2015) · Neonatology · doi:10.1159/000381701 · Cited by 2

Prolonged neonatal-onset hyperinsulinism in the absence of predisposing factors

Gisslen, T. A., Nathan, B. M., Thompson, T., & Rao, R. (2011) · Journal of Neonatal-Perinatal Medicine · doi:10.3233/npm-2011-2731 · Cited by 2

Prematurity and insulin sensitivity

Verónica Mericq, G. (2011) · Journal of Endocrinological Investigation · doi:10.1007/bf03347045 · Cited by 2

Treatment of Congenital Hyperinsulinism in Children: Reality and Prospects

Солнцева, А. В., & Волкова, Н. В. (2020) · Рецепт · doi:10.34883/pi.2020.2.2.046 · Cited by 2

The Neonate

Rozance, P. J., & Rosenberg, A. A. (2012) · Elsevier eBooks · doi:10.1016/b978-1-4377-1935-2.00021-1 · Cited by 2

Pathophysiology of Neonatal Hypoglycemia

Hawkes, C. P., & Stanley, C. A. (2016) · Elsevier eBooks · doi:10.1016/b978-0-323-35214-7.00153-0 · Cited by 2

Hypoglycemia

Lord, K., León, D. D. D., & Stanley, C. A. (2018) · Springer eBooks · doi:10.1007/978-3-319-73782-9_30 · Cited by 2

A homozygous exonic variant leading to exon skipping in ABCC8 as the cause of severe congenital hyperinsulinism

Diaz, J. V. R., Jin, Y., Garber, K. B., Cossen, K., Li, Y., Jin, P., Li, H., & Ham, J. N. (2022) · American Journal of Medical Genetics Part A · doi:10.1002/ajmg.a.62843 · Cited by 2

Defining genotype-phenotype correlations in children with congenital hyperinsulinism

Kapoor, R. R. (2010) · UCL Discovery (University College London) · Cited by 2

Transient Neonatal Hypocortisolism in Neonates with Hypoglycemia – Coexistence or Cause?

Mondal, S., Nagendra, L., Chowdhury, A. S., Palui, R., Biswas, S., Mukherjee, D., Khan, K., Sengupta, A., & Pandey, A. (2024) · Indian Journal of Endocrinology and Metabolism · doi:10.4103/ijem.ijem_158_23 · Cited by 1

Neonatal hypoglycemia: a review of the current diagnostic and management guidelines

Zervoglou, K., Harizopoulou, V. C., Bouroutzoglou, M., Kallia, T., & Antonakou, A. (2024) · Review of Clinical Pharmacology and Pharmacokinetics - International Edition · doi:10.61873/uchb6154 · Cited by 1

Case Report: The importance of genetic counseling for families with hyperinsulinism

Sanders, V. R., Lord, K., Sigal, W. M., McKnight, H., Adzick, N. S., States, L. J., Bhatti, T. R., & León, D. D. D. (2025) · Frontiers in Pediatrics · doi:10.3389/fped.2024.1520871 · Cited by 1

Prevalence and safety of diazoxide in the neonatal intensive care unit

Collins, L. C., Daniel, K. B., Tolia, V. N., Parikh, P., Gray, K. D., & Greenberg, R. G. (2026) · Journal of Perinatology · doi:10.1038/s41372-026-02568-2 · Cited by 1

Congenital Hyperinsulinism: Overview and Clinical Update

Marquard, J., Mayatepek, E., & Meißner, T. (2011) · DergiPark (Istanbul University) · doi:10.17334/jps.89868 · Cited by 1

Bile Acid Synthesis Defect and Hyperinsulinism

Rogers, M. E., Sylvester, F. A., & Lichtman, S. N. (2018) · ACG Case Reports Journal · doi:10.14309/crj.2018.89 · Cited by 1

Factors related to passing the safety fast test among neonates with hypoglycaemia in the neonatal intensive care unit

Olowoyeye, A., Eisenberg, R. E., Kim, M., & Havranek, T. (2020) · Journal of Paediatrics and Child Health · doi:10.1111/jpc.15247 · Cited by 1

Transient Metabolic Disturbances in the Newborn

Kubicka, Z. J., & Fiascone, J. M. (2016) · doi:10.9954/gb105 · Cited by 1

Hyperinsulinism

Clemente, E. G., Kanungo, S., Schmitt, C., & Maajali, D. (2022) · Endocrines · doi:10.3390/endocrines3010011 · Cited by 1

The Neonate

Rozance, P. J., & Rosenberg, A. A. (2017) · Elsevier eBooks · doi:10.1016/b978-0-323-32108-2.00022-6 · Cited by 1

Hyperinsulinism

Hussain, K., & de Lonlay, P. (2014) · Springer eBooks · doi:10.1007/978-3-642-40337-8_21 · Cited by 1

Bile Acid Synthesis Defect and Hyperinsulinism

Rogers, M., Sylvester, F. A., & Lichtman, S. N. (2018) · ACG Case Reports Journal · doi:10.14309/02075970-201805000-00089 · Cited by 1

Congenital hyperinsulinism in a Nigerian infant: A case report and review of literature

Yarhere, I. E., & Jaja, T. (2023) · American Journal of Pharmacotherapy and Pharmaceutical Sciences · doi:10.25259/ajpps_2023_002 · Cited by 1

Neonatal Hypoglycemia and Hyperglycemia

Werny, D. M., Huang, A., Tenney, J., & Pihoker, C. (2023) · Elsevier eBooks · doi:10.1016/b978-0-323-82823-9.00087-8 · Cited by 1

The Rising Incidence of Hyperinsulinemic Hypoglycemia: Connection With Maternal Health

Pillai, S. S., Fredette, M. E., Tanzer, J. R., Hoffman, L., & Topor, L. S. (2023) · Endocrine Practice · doi:10.1016/j.eprac.2023.09.001 · Cited by 1

Congenital hyperinsulinism

Lord, K., & León, D. D. D. (2023) · Elsevier eBooks · doi:10.1016/b978-0-323-91707-0.00016-4 · Cited by 1

Characterization of congenital hyperinsulinism in Argentina: Clinical features, genetic findings, and treatment outcomes

Pacheco, G., Bastida, M. G., Cáceres, J., Alonso, G., Aziz, M., Suárez, M. G., Flores, A., Femenia, V., Forclaz, M. V., Houghton, J., et al. (2025) · PLoS ONE · doi:10.1371/journal.pone.0321244 · Cited by 1

Hyperinsulinemic Hypoglycemia in Childhood

Özbek, M. N., & Orhan, Ö. (2023) · European Journal of Therapeutics · doi:10.58600/eurjther1758 · Cited by 1

Neonatal Hypoglycemia

Sigal, W. M., & León, D. D. D. (2024) · Elsevier eBooks · doi:10.1016/b978-0-323-69415-5.00023-0 · Cited by 1

Part Introduction

Hey, E. N. (2011) · Wiley eBooks · doi:10.1002/9781444329773.part2 · Cited by 1

Hiperinsulinismo congênito em crianças brasileiras: histopatologia, proliferação das células do pâncreas e genética dos canais K+ / ATP

Lovisolo, S. M. (2009) · doi:10.11606/t.5.2009.tde-03062009-095653

Hiperinsulinismo en lactantes y niños: cuando el nivel de insulina no siempre es suficiente

Palladino, A., Bennett, M. J., & Stanley, C. A. (2009) · Redalyc (Universidad Autónoma del Estado de México)

Caracterização molecular e funcional da enzina glutamato desidrogenase (GDH) em Ilhotas de ratos submetidos à restrição protéica e suplementados com leucina

da Silva, P. M. R. (2011) · doi:10.47749/t/unicamp.2011.798615

重症頭部外傷後に小児特発性高インスリン血症性低血糖症を来した1例(A case of persistent hyperinsulinemic hypoglycemia of infancy associated with severe brain injury)

和史, 野., 克俊, 丹., 修二, 上., 坂脇, 園., 喜屋武, 玲., 圭, 宮., & 英智, 成. (2015) · Nihon Kyukyu Igakukai Zasshi Journal of Japanese Association for Acute Medicine · doi:10.1002/jja2.12020

Hiperinsulinismo na infância: quando apenas uma dosagem de insulina não é suficiente

Palladino, A., Bennett, M. J., & Stanley, C. A. (2008) · Jornal Brasileiro de Patologia e Medicina Laboratorial · doi:10.1590/s1676-24442008000600004

Hyperinsulinismus bei Säuglingen und Kindern: wenn ein Insulinspiegel nicht immer ausreicht / Hyperinsulinism in infancy and childhood: when an insulin level is not always enough1)

Palladino, A., Bennett, M. J., & Stanley, C. A. (2009) · Journal of Laboratory Medicine · doi:10.1515/jlm.2009.020

Unique Presentation of an 8p Deletion in a Discordant Twin with Atrioventricular Canal Defect and Prolonged Hypoglycemia

Kumar, P., Elshershari, H., Parashette, K. R., Ize-Ludlow, D., & Harris, C. (2010) · Balkan Journal of Medical Genetics · doi:10.2478/v10034-010-0018-4

Langdurig hyperinsulinisme bij pasgeborenen

Berg, L., Trijbels-Smeulders, M. A. J. M., & Maingay-Visser, A. P. G. F. (2009) · Tijdschrift voor kindergeneeskunde · doi:10.1007/bf03086394

normoglycaemia during management basis and the importance of maintaining Hyperinsulinaemic hypoglycaemia: biochemical

Hussain, K., Blankenstein, O., de Lonlay, P., & Christesen, H. T. (2007)

Hypoglycemia in Infants and Children

(2020) · American Academy of PediatricsItasca, IL eBooks · doi:10.1542/9781610023610-31

DEBATING ISSUES AS TO THE PROGNOSIS AND DIAGNOSIS OF HYPOXIC-ISCHEMIC MYOCARDIAL INJURY BY ASPHYXIA IN NEWBORNS

Коробка, О. В., Bieloru, A. I., Hasiuk, N. І., Kovaliov, V. O., & Kovaliova, O. M. (2016) · Neonatology surgery and perinatal medicine · doi:10.24061/2413-4260.vi.1.19.2016.5

Glucose Derangements and Brain Function in Neonatal Encephalopathy

Pinchefsky, E. F. (2018) · TSpace (University of Toronto)

Neonatal formulary 7

Ainsworth, S. B. (2015) · John Wiley & Sons, Inc. eBooks

Hepatic Dysfunction and Hypoglycemia in a Newborn

Chugh, A. A., Younis, M., Shah, K. P., Hart, L., Hu, S., Hart, J. A. L., & Azzam, R. K. (2020) · Clinical Pediatrics · doi:10.1177/0009922820916896

Congenital hyperinsulinism: from molecular genetics to clinical practice

Solntsava, A. V., & Volkava, N. V. (2020) · Ukrainian Journal of Pediatric Endocrinology · doi:10.30978/ujpe2020-2-4

Persistent or Recurrent Hypoglycemia in Infants and Todlers

Lai, C. W., & León, D. D. D. (2020) · Springer eBooks · doi:10.1007/978-3-030-52215-5_51

Hyperinsulinemic Hypoglycemia in Neonates Due to Perinatal Stress: A Case Series

Prasad, A., Ankur, K., & Chetry, S. (2021) · Indian Pediatrics · doi:10.1007/s13312-021-2268-5

Diazoxide

Hey, E. N. (2007) · doi:10.1002/9780470750872.ch69

Drug monographs

Ainsworth, D. S. B. (2014) · Wiley eBooks · doi:10.1002/9781118819494.ch15

Bile Acid Synthesis Defect and Hyperinsulinism

Rogers, M., Sylvester, F. A., & Lichtman, S. N. (2018) · ACG Case Reports Journal · doi:10.14309/02075970-201805120-00005

Hypoglycemia

James, C. T., & Hussain, K. (2012) · Springer eBooks · doi:10.1007/978-3-642-02202-9_389

O

Ainsworth, S. B. (2020) · doi:10.1093/med/9780198840787.003.0027

Hypoglycemia

León, D. D. D., & Stanley, C. A. (2013) · Humana Press eBooks · doi:10.1007/978-1-60761-395-4_27

Transient Metabolic Disturbances in the Newborn

Kubicka, Z. J., & Fiascone, J. M. (2020) · American Academy of PediatricsItasca, IL eBooks · doi:10.1542/9781610022255-30

Pathophysiology of Hypoglycemia

Charles, Stanley, A., & Hardy, O. T. (2011) · Elsevier eBooks · doi:10.1016/b978-1-4160-3479-7.10053-9

D

Ainsworth, S. B. (2020) · doi:10.1093/med/9780198840787.003.0017

PERSISTENT HYPOGLYCAEMIA IN A NEWBORN – CASE REPORT OF A GIRL WITH BECKWITH-WIEDEMANN SYNDROME

Kavčič, M., Krivec, J. L., & Stefanija, M. A. (2022) · Slovenska pediatrija revija pediatrov Slovenije in specialistov šolske ter visokošolske medicine Slovenije · doi:10.38031/slovpediatr-2022-2-03en

Umbilical Artery Cord Blood Glucose Predicted neonatal hypoglycemia in Gestational Diabetes Mellitus and Other at-risk Newborns after Vaginal DeliveryPreprint

Wang, Y., Liu, H., Zhang, L., Wang, X., Wang, M., Chen, Z., & Zhang, F. (2022) · Research Square · doi:10.21203/rs.3.rs-1958011/v1

Hypoglycemia in Infants and Children

(2020) · American Academy of PediatricsItasca, IL eBooks · doi:10.1542/9781610024181-31

Hypoglycemia in Children Referred to a Tertiary Care Pediatric Endocrine Clinic: Age-Dependent Etiological Variations

Margolis, M. G., Lilos, P., Phillip, M., & de Vries, L. (2023) · Hormone Research in Paediatrics · doi:10.1159/000535779

Kongenitaler Hyperinsulinismus

Empting, S., & Mohnike, K. L. (2024) · Kinder- und Jugendmedizin · doi:10.1055/a-2418-3900

Hypoglycemia

Lord, K., León, D. D. D., & Stanley, C. A. (2024) · Springer eBooks · doi:10.1007/978-3-031-66296-6_29

Unsolved dental problems in children with congenital hyperinsulin

Пестрякова, И. Ю., Викторовна, Б. А., Георгиевна, А. О., & Минвалиевна, Х. А. (2025) · Stomatology · doi:10.17116/stomat202510406167

Neonatal sepsis and its associated factors among asphyxiated newborns admitted in West Oromia Tertiary Hospitals, Ethiopia

Daka, D. T., Terefe, B., Gedefaw, G. D., Abate, A. T., & Legesse, B. T. (2026) · Scientific Reports · doi:10.1038/s41598-026-35866-y

Anesthesia considerations in pediatric pancreatectomy for congenital hyperinsulinemic hypoglycemia: A retrospective case series

Arafah, O., Nassef, M., Akhtar, A. B., Al-Shammari, H., & Norris, A. (2026) · Saudi Journal of Anaesthesia · doi:10.4103/sja.sja_869_25

Evidence for and Definitions of Normal and Abnormal Low Glucose Concentrations

Adamkin, D. H. (2026) · Springer eBooks · doi:10.1007/978-3-032-29094-6_5

Neurodevelopmental Consequences of Neonatal Hypoglycemia

Rosenfeld, E., & León, D. D. D. (2026) · Cambridge University Press eBooks · doi:10.1017/9781009418034.028

Early Short-course Diazoxide in Selected Neonates with Hyperinsulinemic Hypoglycemia: A Retrospective Observational Study

Srinath, M., Chandran, R., Balakrishnan, U., & Amboiram, P. (2026) · Journal of Neonatology · doi:10.1177/09732179261471219

Hyperinsulinisme transitoire avec hypoglycémie par asphyxie néonatale

Sann, L. (1999) · Archives de Pédiatrie · doi:10.1016/s0929-693x(99)80214-0 · Cited by 3

Prolonged neonatal-onset hyperinsulinism in the absence of predisposing factors

Gisslen, T. A., Nathan, B. M., Thompson, T., & Rao, R. (2011) · Journal of Neonatal-Perinatal Medicine · doi:10.3233/npm-2011-2731 · Cited by 2

Heterogeneity of persistent hyperinsulinaemic hypoglycaemia. A series of 175 cases

de Lonlay, P., Fournet, J., Touati, G., Groos, M.-S., Martin, D., Sevin, C., Delagne, V., Mayaud, C., Chigot, V., Sempoux, C. L., et al. (2001) · European Journal of Pediatrics · doi:10.1007/s004310100847 · Cited by 151

Hypoglycemia in the neonate.

Stanley, C. A. (2006) · PubMed · Cited by 17

A Case of Hyperinsulinemic Hypoglycemia in Premature Infant Treated with Oral Diazoxide

Pyeon, J. E., Jeong, H. R., & Koh, J. W. (2022) · Soonchunhyang Medical Science · doi:10.15746/sms.22.028