Clinical features and insulin regulation in infants with a syndrome of prolonged neonatal hyperinsulinism
Abstract
Objectives To characterize the clinical features and insulin regulation in infants with hypoglycemia due to prolonged neonatal hyperinsulinism.Study design Data were collected on 26 infants with hypoglycemia due to neonatal hyperinsulinism that later resolved. Acute insulin response (AIR) tests to calcium, leucine, glucose, and tolbutamide were performed in 11 neonates. Results were compared to children with genetic hyperinsulinism due to mutations of the adenosine triphosphate-dependent potassium (K(ATP)) channel and glutamate dehydrogenase (GDH).Results Among the 26 neonates, there were significantly more males, small-for-gestational-age infants, and cesarean deliveries. Only 5 of the 26 had no identifiable risk factor. Hyperinsulinism was diagnosed at a median age of 13 days (range, 2 to 180 days) and resolved by a median age of 181 days (range, 18 to 403 days). Diazoxide was effective in 19 of the 21 neonates treated. In the 11 neonates tested, the AIRs to calcium, leucine, glucose, and tolbutamide resembled those in normal controls and differed from genetic hyperinsulinism due to K(ATP) channel and GDH mutations.Conclusions We define a syndrome of prolonged neonatal hyperinsulinism that is responsive to diazoxide, persists for several months, and resolves spontaneously. AIR tests suggest that both the K(ATP) channel and GDH have normal function.
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There is no assurance that the plasma lactate level will be sufficiently elevated to compensate for low glucose in high-risk neonates.
“Of concern, as noted previously,3 plasma ketone levels are suppressed during hypoglycemia in high-risk neonates, making them particularly vulnerable to hypoglycemia-induced brain damage, and there is no assurance the plasma lactate level will be sufficiently elevated to compensate for low glucose.27,28,30,39”
From Recommendations from the Pediatric Endocrine Society for evaluation and management of persistent hypoglycemia in neonates, infants, and children (2015) · cites this paper as [30]
Ongoing hypoglycemia beyond the first 48 hours of life, and particularly beyond the first week, increases the concern for an underlying hypoglycemia disorder such as prolonged neonatal hyperinsulinism or a genetic disorder.
“The committee also focused on postnatal age, because ongoing hypoglycemia beyond the first 48 hours of life (and particularly beyond the first week) increases the concern for an underlying hypoglycemia disorder, such as prolonged neonatal hyperinsulinism,30 or a genetic disorder.”
From Recommendations from the Pediatric Endocrine Society for evaluation and management of persistent hypoglycemia in neonates, infants, and children (2015) · cites this paper as [30]
Cites
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 · see citing works
Martin, J. A., Hamilton, B. E., Ventura, S. J., Menacker, F., & Park, M. M. (2002) · PubMed · Cited by 889 · see citing works
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 374 · see citing works
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 · see citing works
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 · see citing works
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 279 · see citing works
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 · see citing works
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 · see citing works
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 · see citing works
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 · see citing works
Finegold, D. N., Stanley, C. A., & Baker, L. (1980) · The Journal of Pediatrics · doi:10.1016/s0022-3476(80)80817-1 · Cited by 150 · see citing works
Self-citationIncreased Gluconeogenic Substrates in the Small-for-Gestational-Age Infant
Haymond, M. W., Karl, I. E., & Pagliara, A. S. (1974) · New England Journal of Medicine · doi:10.1056/nejm197408152910702 · Cited by 148 · see citing works
HYPERINSULINISM IN ASPHYXIATED AND SMALL-FOR-DATES INFANTS WITH HYPOGLYCAEMIA
Collins, J. E., & Leonard, J. V. (1984) · The Lancet · doi:10.1016/s0140-6736(84)92685-0 · Cited by 141 · see citing works
Grimberg, A., Ferry, R. J., Kelly, A. T., Koo-McCoy, S., Polonsky, K. S., Gläser, B., Permutt, M. A., Aguilar‐Bryan, L., Stafford, D. E. J., THORNTON, P. S., et al. (2001) · Diabetes · doi:10.2337/diabetes.50.2.322 · Cited by 128 · see citing works
Hyperinsulinism in Infancy: Diagnosis by Demonstration of Abnormal Response to Fasting Hypoglycemia
Stanley, C. A., & Baker, L. (1976) · PEDIATRICS · doi:10.1542/peds.57.5.702 · Cited by 127 · see citing works
Semenza, G. L., Agani, F. H., Iyer, N. V., Jiang, B., Leung, S., Wiener, C., & Yu, A. Y. (1998) · CHEST Journal · doi:10.1378/chest.114.1_supplement.40s · Cited by 127 · see citing works
Letter: Allergy, immunodeficiency, and epilepsy.
As, C. (1975) · PubMed · Cited by 124 · see citing works
Recent advances in hyperinsulinism and the pathogenesis of diabetes mellitus
Bruining, G. J. (1990) · Current Opinion in Pediatrics · doi:10.1097/00008480-199008000-00024 · Cited by 122 · see citing works
The Causes of Neonatal Hypoglycemia
Stanley, C. A., & Baker, L. (1999) · New England Journal of Medicine · doi:10.1056/nejm199904153401510 · Cited by 117 · see citing works
Hyperinsulinaemic hypoglycaemia in small for dates babies
Collins, J. E., Leonard, J. V., Teale, D., Marks, V, Williams, D. M., Kennedy, C. R., & Hall, M. A. (1990) · Archives of Disease in Childhood · doi:10.1136/adc.65.10.1118 · Cited by 110 · see citing works
Metabolic Fuel and Hormone Responses to Fasting in Newborn Infants
Stanley, C. A., Anday, E. K., Baker, L., & Delivoria-Papadopolous, M. (1979) · PEDIATRICS · doi:10.1542/peds.64.5.613 · Cited by 107 · see citing works
Stanley, C. A., THORNTON, P. S., Ganguly, A. A., MacMullen, C., UNDERWOOD, P. C., Bhatia, P., Steinkrauss, L. J., Wanner, L. A., Kaye, R. D., Ruchelli, E. D., et al. (2004) · The Journal of Clinical Endocrinology & Metabolism · doi:10.1210/jc.2003-030965 · Cited by 104 · see citing works
Acute Insulin Responses to Leucine in Children with the Hyperinsulinism/Hyperammonemia Syndrome
Kelly, A. T., Ng, D. D., Ferry, R. J., Grimberg, A., Koo-McCoy, S., THORNTON, P. S., & Stanley, C. A. (2001) · The Journal of Clinical Endocrinology & Metabolism · doi:10.1210/jcem.86.8.7755 · Cited by 98 · see citing works
Henwood, M. J., Kelly, A. T., MacMullen, C., Bhatia, P., Ganguly, A. A., THORNTON, P. S., & Stanley, C. A. (2005) · The Journal of Clinical Endocrinology & Metabolism · doi:10.1210/jc.2004-1604 · Cited by 68 · see citing works
Phillips, I. R. (1982) · The Lancet · doi:10.1016/s0140-6736(82)90281-1 · Cited by 66 · see citing works
Calcium-stimulated insulin secretion in diffuse and focal forms of congenital hyperinsulinism
Ferry, R. J., Kelly, A. T., Grimberg, A., Koo-McCoy, S., Shapiro, M. J., Fellows, K. E., Gläser, B., Aguilar‐Bryan, L., Stafford, D. E. J., & Stanley, C. A. (2000) · The Journal of Pediatrics · doi:10.1067/mpd.2000.107386 · Cited by 55 · see citing works
Melichar, V., Drahota, Z., & Hahn, P. (2009) · Biologia Neonatorum · doi:10.1159/000240051 · Cited by 31 · see citing works
Prolonged Hyperinsulinism and Hypoglycemia
Bhowmick, S. K., & Lewandowski, C. (1989) · Clinical Pediatrics · doi:10.1177/000992288902801205 · Cited by 27 · see citing works
Pégorier, J. P., Chatelain, F. C., Thumelin, S., & Girard, J. R. (1998) · Biochemical Society Transactions · doi:10.1042/bst0260113 · Cited by 23 · see citing works
HYPERINSULINISM IN ASPHYXIATED AND SMALL-FOR-DATES INFANTS WITH HYPOGLYCAEMIA
Klenka, H., & Seager, J. (1984) · The Lancet · doi:10.1016/s0140-6736(84)91182-6 · Cited by 16 · see citing works
Low, L. C. K., Yu, E. C. L., Chow, O. K. W., Yeung, C. Y., & YOUNG, R. T. T. (1989) · Journal of Paediatrics and Child Health · doi:10.1111/j.1440-1754.1989.tb01446.x · Cited by 15 · see citing works
Transient hyperinsulinism in asphyxiated newborn infants.
Schultz, K., & Soltész, G. (1991) · PubMed · Cited by 12 · see citing works
Hyperinsulinisme transitoire avec hypoglycémie par asphyxie néonatale
Giroux, J., Vernotte, E., Gagneur, A., Metz, C. N., Collet, M., & de Parscau, L. (1997) · Archives de Pédiatrie · doi:10.1016/s0929-693x(97)82612-7 · Cited by 6 · see citing works
Glucose metabolism in a term infant with transient hyperinsulinism and high carbohydrate intake
van Goudoever, J. B., Sulkers, E., Kalhan, S. C., & Sauer, P. J. J. (1993) · European Journal of Pediatrics · doi:10.1007/bf01956750 · Cited by 6 · see citing works
Prolonged hyperinsulinism and hypoglycemia in an asphyxiated, small for gestation infant
Bhowmick, S. K. (1989) · Medical Entomology and Zoology · Cited by 6 · see citing works
Hyperinsulinisme transitoire avec hypoglycémie par asphyxie néonatale
Sann, L. (1999) · Archives de Pédiatrie · doi:10.1016/s0929-693x(99)80214-0 · Cited by 3 · see citing works
Flagged first, then by citations
Cited by
Sorted by citations
Thornton, P. S., Stanley, C. A., De Leon, D. D., Harris, D., Haymond, M. W., Hussain, K., Levitsky, L. L., Murad, M. H., Rozance, P. J., Simmons, R. A., et al. (2015) · The Journal of Pediatrics · doi:10.1016/j.jpeds.2015.03.057 · Cited by 625 · see citing works
“Of concern, as noted previously,(3) plasma ketone levels are suppressed during hypoglycemia in high-risk neonates, making them particularly vulnerable to hypoglycemia-induced brain damage, and there is no assurance the plasma lactate level will be sufficiently elevated to compensate for low glucose.(27,28,30,39)”
Long-term metabolic risk among children born premature or small for gestational age.
Mericq, V., Martinez-Aguayo, A., Uauy, R., Iñiguez, G., Van der Steen, M., & Hokken-Koelega, A. (2017) · Nature Reviews Endocrinology · doi:10.1038/nrendo.2016.127 · Cited by 257 · see citing works
Re-evaluating “transitional neonatal hypoglycemia”: mechanism and implications for management
Stanley, C. A., Rozance, P. J., Thornton, P. S., De Leon, D. D., Harris, D., Haymond, M. W., Hussain, K., Levitsky, L. L., Murad, M. H., Simmons, R. A., et al. (2015) · The Journal of Pediatrics · doi:10.1016/j.jpeds.2015.02.045 · Cited by 245 · see citing works
Self-citationPerspective on the Genetics and Diagnosis of Congenital Hyperinsulinism Disorders
Stanley, C. A. (2016) · The Journal of Clinical Endocrinology & Metabolism · doi:10.1210/jc.2015-3651 · Cited by 239 · see citing works
Mechanisms of Disease: advances in diagnosis and treatment of hyperinsulinism in neonates
León, D. D. D., & Stanley, C. A. (2006) · Nature Clinical Practice Endocrinology & Metabolism · doi:10.1038/ncpendmet0368 · Cited by 198 · see citing works
Intrauterine Growth Restriction
Kesavan, K., & Devaskar, S. U. (2019) · Pediatric Clinics of North America · doi:10.1016/j.pcl.2018.12.009 · Cited by 189 · see citing works
Adamkin, D. H. (2016) · Seminars in Fetal and Neonatal Medicine · doi:10.1016/j.siny.2016.08.007 · Cited by 165 · see citing works
Hyperinsulinism in Infancy and Childhood: When an Insulin Level Is Not Always Enough
Palladino, A., Bennett, M. J., & Stanley, C. A. (2007) · Clinical Chemistry · doi:10.1373/clinchem.2007.098988 · Cited by 134 · see citing works
International Guidelines for the Diagnosis and Management of Hyperinsulinism
León, D. D. D., Arnoux, J. B., Banerjee, I., Bergadá, I., Bhatti, T. R., Conwell, L. S., Fu, J., Flanagan, S. E., Gillis, D. J., Meißner, T., et al. (2023) · Hormone Research in Paediatrics · doi:10.1159/000531766 · Cited by 125 · see citing works
Diagnosis and Management of Hyperinsulinaemic Hypoglycaemia of Infancy
Hussain, K. (2007) · Hormone Research in Paediatrics · doi:10.1159/000111789 · Cited by 112 · see citing works
Advances in the diagnosis and management of hyperinsulinemic hypoglycemia
Kapoor, R. R., James, C. T., & Hussain, K. (2009) · Nature Clinical Practice Endocrinology & Metabolism · doi:10.1038/ncpendmet1046 · Cited by 104 · see citing works
Consequences of a compromised intrauterine environment on islet function
Green, A. S., Rozance, P. J., & Limesand, S. W. (2010) · Journal of Endocrinology · doi:10.1677/joe-09-0399 · Cited by 99 · see citing works
Prevalence of Adverse Events in Children With Congenital Hyperinsulinism Treated With Diazoxide
Herrera, A. M., Vajravelu, M. E., Givler, S., Mitteer, L., Avitabile, C. M., Lord, K., & León, D. D. D. (2018) · The Journal of Clinical Endocrinology & Metabolism · doi:10.1210/jc.2018-01613 · Cited by 98 · see citing works
Congenital hyperinsulinism disorders: Genetic and clinical characteristics
Rosenfeld, E., Ganguly, A. A., & León, D. D. D. (2019) · American Journal of Medical Genetics Part C Seminars in Medical Genetics · doi:10.1002/ajmg.c.31737 · Cited by 95 · see citing works
Continuous glucose monitoring in neonates: a review
McKinlay, C. J. D., Chase, J. G., Dickson, J. L., Harris, D. L., Alsweiler, J. M., & Harding, J. E. (2017) · Maternal Health Neonatology and Perinatology · doi:10.1186/s40748-017-0055-z · Cited by 87 · see citing works
Hussain, K., Blankenstein, O., de Lonlay, P., & Christesen, H. T. (2007) · Archives of Disease in Childhood · doi:10.1136/adc.2006.115543 · Cited by 78 · see citing works
Update on neonatal hypoglycemia
Rozance, P. J. (2013) · Current Opinion in Endocrinology Diabetes and Obesity · doi:10.1097/med.0000000000000027 · Cited by 74 · see citing works
Limesand, S. W., & Rozance, P. J. (2017) · The Journal of Physiology · doi:10.1113/jp273324 · Cited by 74 · see citing works
Adamkin, D. H. (2016) · Current Opinion in Pediatrics · doi:10.1097/mop.0000000000000319 · Cited by 68 · see citing works
Palladino, A., & Stanley, C. A. (2010) · Seminars in Pediatric Surgery · doi:10.1053/j.sempedsurg.2010.10.008 · Cited by 66 · see citing works
Yau, D., Laver, T. W., Dastamani, A., Senniappan, S., Houghton, J., Shaikh, M. G., Cheetham, T. D., Mushtaq, T., Kapoor, R. R., Randell, T. L., et al. (2020) · PLoS ONE · doi:10.1371/journal.pone.0228417 · Cited by 65 · see citing works
Management and Appropriate Use of Diazoxide in Infants and Children with Hyperinsulinism
Brar, P. C., Heksch, R. A., Cossen, K., León, D. D. D., Kamboj, M. K., Marks, S. D., Marshall, B. A., Miller, R., PAGE, L. C., Stanley, T. L., et al. (2020) · The Journal of Clinical Endocrinology & Metabolism · doi:10.1210/clinem/dgaa543 · Cited by 59 · see citing works
Clinical Aspects of Neonatal Hypoglycemia: A Mini Review
Edwards, T., & Harding, J. E. (2021) · Frontiers in Pediatrics · doi:10.3389/fped.2020.562251 · Cited by 58 · see citing works
Rate of Serious Adverse Events Associated with Diazoxide Treatment of Patients with Hyperinsulinism
THORNTON, P. S., Truong, L., Reynolds, C., Hamby, T., & Nedrelow, J. H. (2019) · Hormone Research in Paediatrics · doi:10.1159/000497458 · Cited by 57 · see citing works
Camacho, L. E., Chen, X., Hay, W. W., & Limesand, S. W. (2017) · American Journal of Physiology-Regulatory, Integrative and Comparative Physiology · doi:10.1152/ajpregu.00068.2017 · Cited by 51 · see citing works
Arya, V. B., Flanagan, S. E., Kumaran, A., Hamilton-Shield, J. P., Ellard, S., Hussain, K., & Kapoor, R. R. (2013) · Archives of Disease in Childhood Fetal & Neonatal · doi:10.1136/archdischild-2012-302880 · Cited by 50 · see citing works
Hyperinsulinism in the Neonate
Lord, K., & León, D. D. D. (2017) · Clinics in Perinatology · doi:10.1016/j.clp.2017.10.007 · Cited by 49 · see citing works
Development of Pulmonary Hypertension in an Infant Treated with Diazoxide
Nebesio, T. D., Hoover, W., Caldwell, R. L., Nitu, M. E., & Eugster, E. A. (2007) · Journal of Pediatric Endocrinology and Metabolism · doi:10.1515/jpem.2007.20.8.939 · Cited by 43 · see citing works
Prevalence and safety of diazoxide in the neonatal intensive care unit
Gray, K. D., Dudash, K., Escobar-Tomlienovich, C. F., Freel, C., Harrison, T., McMillan, C., Puia‐Dumitrescu, M., Cotten, C. M., Benjamin, R. W., Clark, R. H., et al. (2018) · Journal of Perinatology · doi:10.1038/s41372-018-0218-4 · Cited by 42 · see citing works
Pulmonary hypertension and reopening of the ductus arteriosus in an infant treated with diazoxide
Demirel, F., Ünal, S., Çetin, İ. İ., Esen, İ., & Yılmaz, A. A. (2011) · Journal of Pediatric Endocrinology and Metabolism · doi:10.1515/jpem.2011.238 · Cited by 40 · see citing works
Prematurity and Insulin Sensitivity
Verónica Mericq, G. (2006) · Hormone Research in Paediatrics · doi:10.1159/000091518 · Cited by 38 · see citing works
Activating AKT2 Mutation: Hypoinsulinemic Hypoketotic Hypoglycemia
Arya, V. B., Flanagan, S. E., Schober, E., Rami-Merhar, B. M., Ellard, S., & Hussain, K. (2013) · The Journal of Clinical Endocrinology & Metabolism · doi:10.1210/jc.2013-3228 · Cited by 37 · see citing works
Strategies to improve neurodevelopmental outcomes in babies at risk of neonatal hypoglycaemia
Alsweiler, J. M., Harris, D. L., Harding, J. E., & McKinlay, C. J. D. (2021) · The Lancet Child & Adolescent Health · doi:10.1016/s2352-4642(20)30387-4 · Cited by 36 · see citing works
High Prevalence of Severe Circulatory Complications with Diazoxide in Premature Infants
Yoshida, K., Kawai, M., Marumo, C., Kanazawa, H., Matsukura, T., Kusuda, S., Yorifuji, T., & Heike, T. (2014) · Neonatology · doi:10.1159/000356772 · Cited by 33 · see citing works
Männistö, J. M. E., Jääskeläinen, J., Otonkoski, T., & Huopio, H. (2021) · The Journal of Clinical Endocrinology & Metabolism · doi:10.1210/clinem/dgab024 · Cited by 32 · see citing works
Demirbilek, H., Rahman, S. A., Büyükyılmaz, G., & Hussain, K. (2017) · International Journal of Pediatric Endocrinology · doi:10.1186/s13633-017-0048-8 · Cited by 31 · see citing works
Metabolic Screening and Postnatal Glucose Homeostasis in the Newborn
Adamkin, D. H. (2015) · Pediatric Clinics of North America · doi:10.1016/j.pcl.2014.11.004 · Cited by 31 · see citing works
Stanley, C., Thornton, P., & León, D. D. (2023) · Frontiers in Pediatrics · doi:10.3389/fped.2023.1071206 · Cited by 27 · see citing works
Hypoglycemia in Infants and Children
Hoe, F. M. (2008) · Advances in Pediatrics · doi:10.1016/j.yapd.2008.07.008 · Cited by 29 · see citing works
Neonatal hypoglycemia: is 60 the new 40? The questions remain the same
Adamkin, D. H., & Polin, R. A. (2015) · Journal of Perinatology · doi:10.1038/jp.2015.125 · Cited by 28 · see citing works
Diazoxide for Neonatal Hyperinsulinemic Hypoglycemia and Pulmonary Hypertension
Newman-Lindsay, S., Lakshminrusimha, S., & Sankaran, D. (2022) · Children · doi:10.3390/children10010005 · Cited by 28 · see citing works
Yang, J., Hammoud, B., Li, C., Ridler, A., Yau, D., Kim, J., Won, K., Stanley, C. A., Hoshi, T., & Stanescu, D. E. (2021) · Endocrinology · doi:10.1210/endocr/bqab121 · Cited by 27 · see citing works
Natural history and neurodevelopmental outcomes in perinatal stress induced hyperinsulinism
Sigal, W. M., Alzahrani, O., Rios, G. M. G., Guzman, H., Radcliffe, J., Thomas, N. H., Jawad, A. F., & León, D. D. D. (2022) · Frontiers in Pediatrics · doi:10.3389/fped.2022.999274 · Cited by 24 · see citing works
Prolonged transitional neonatal hypoglycaemia: characterisation of a clinical syndrome
Bailey, M. J., Rout, A., Harding, J. E., Alsweiler, J. M., Cutfield, W. S., & McKinlay, C. J. D. (2020) · Journal of Perinatology · doi:10.1038/s41372-020-00891-w · Cited by 23 · see citing works
Kibbey, R. G., Choi, C. S., Lee, H., Cabrera, O., Pongratz, R. L., Zhao, X., Birkenfeld, A. L., Li, C., Berggren, P., Stanley, C. A., et al. (2014) · Diabetes · doi:10.2337/db14-0783 · Cited by 20 · see citing works
Balachandran, B., Mukhopadhyay, K., Sachdeva, N. K., Walia, R., & Attri, S. V. (2018) · Acta Paediatrica · doi:10.1111/apa.14252 · Cited by 20 · see citing works
Glucose Homeostasis in Newborns: An Endocrinology Perspective
Tas, E., GARIBALDI, L. R., & Muzumdar, R. H. (2020) · NeoReviews · doi:10.1542/neo.21-1-e14 · Cited by 21 · see citing works
Hubbard, E. M., & Hay, W. W. (2021) · Clinics in Perinatology · doi:10.1016/j.clp.2021.05.013 · Cited by 19 · see citing works
Aliefendioğlu, D., Çoban, A. Ç., Hatipoğlu, N., Ecevit, A., Arısoy, A. E., Mutlu, G. Y., Baş, F., Bıdecı, A., & Özek, E. (2019) · Türk Pediatri Arşivi · doi:10.5152/turkpediatriars.2018.01820 · Cited by 19 · see citing works
Stawarski, A., & Maleika, P. (2020) · Advances in Clinical and Experimental Medicine · doi:10.17219/acem/111806 · Cited by 19 · see citing works
Association of Fetal Catecholamines With Neonatal Hypoglycemia
Hoermann, H., van Faassen, M. H. J. R., Roeper, M., Hagenbeck, C., Herebian, D. A., Kobold, A. C. M., Dukart, J., Kema, I. P., Mayatepek, E., Meißner, T., et al. (2024) · Archives of Pediatrics and Adolescent Medicine · doi:10.1001/jamapediatrics.2024.0304 · Cited by 18 · see citing works
Stanescu, D. L., & Stanley, C. (2022) · Clinics of Perinatology · doi:10.1016/j.clp.2021.11.007 · Cited by 13 · see citing works
Managing congenital hyperinsulinism: improving outcomes with a multidisciplinary approach
Palladino, A., & Ackermann, A. M. (2015) · Research and Reports in Endocrine Disorders · doi:10.2147/rred.s56608 · Cited by 18 · see citing works
CONN, J. J., Simm, P. J., Oats, J. J. N., Nankervis, A. J., Jacobs, S. E., Ellard, S., & Hattersley, A. T. (2009) · Australian and New Zealand Journal of Obstetrics and Gynaecology · doi:10.1111/j.1479-828x.2009.01009.x · Cited by 18 · see citing works
Kelly, A. C., Bidwell, C. A., Chen, X., Macko, A. R., Anderson, M. J., & Limesand, S. W. (2018) · Endocrinology · doi:10.1210/en.2018-00540 · Cited by 17 · see citing works
Chandran, S., Pravin, R. R., Chua, M. C., Saffari, S. E., Rajadurai, V. S., & Yap, F. K. P. (2021) · Archives of Disease in Childhood Fetal & Neonatal · doi:10.1136/archdischild-2021-322845 · Cited by 16 · see citing works
Biochemical studies in patients with hyperinsulinaemic hypoglycaemia
Alotaibi, H., Senniappan, S., Alam, S. T., & Hussain, K. (2013) · European Journal of Pediatrics · doi:10.1007/s00431-013-2053-0 · Cited by 16 · see citing works
Hypoglycemia in Newborns and Infants
Dekelbab, B. H., & Sperling, M. A. (2006) · Advances in Pediatrics · doi:10.1016/j.yapd.2006.04.004 · Cited by 15 · see citing works
Liver injury may increase the risk of diazoxide toxicity: a case report
Tas, E., Mahmood, B., GARIBALDI, L. R., & Sperling, M. A. (2015) · European Journal of Pediatrics · doi:10.1007/s00431-015-2488-6 · Cited by 14 · see citing works
Rozance, P. J., & Hay, W. W. (2016) · Molecular and Cellular Endocrinology · doi:10.1016/j.mce.2016.01.025 · Cited by 14 · see citing works
Transient neonatal hyperinsulinaemic hypoglycaemia: perinatal predictors of length and cost of stay
Kozen, K., Dassios, T. G., Kametas, N. A., Kapoor, R. R., & Greenough, A. (2018) · European Journal of Pediatrics · doi:10.1007/s00431-018-3242-7 · Cited by 14 · see citing works
Davidov, A. S., Elkon‐Tamir, E., Haham, A., Shefer, G., Weintrob, N., Oren, A., Lebenthal, Y., Mandel, D., & Eyal, O. (2019) · European Journal of Pediatrics · doi:10.1007/s00431-019-03544-z · Cited by 13 · see citing works
Hypoglycemia in Neonates and Infants
León, D. D. D., STANELY, C. A., & Sperling, M. A. (2008) · Elsevier eBooks · doi:10.1016/b978-141604090-3.50010-7 · Cited by 13 · see citing works
Clinical Predictors of Transient versus Persistent Neonatal Hyperinsulinism
Falzone, N., & Harrington, J. (2020) · Hormone Research in Paediatrics · doi:10.1159/000511139 · Cited by 13 · see citing works
Skovrlj, R., Marks, S. D., & Rodd, C. J. (2018) · Paediatrics & Child Health · doi:10.1093/pch/pxy166 · Cited by 12 · see citing works
Raisingani, M. G., & Brar, P. C. (2019) · Journal of Pediatric Endocrinology and Metabolism · doi:10.1515/jpem-2019-0066 · Cited by 12 · see citing works
Association Between Hypoglycemia and the Occurrence of Early Onset Sepsis in Premature Infants
Kumar, K. R., Shah, S., Fayyad, R. M., Turla, T. M., O’Sullivan, L., Wallace, B., Clark, R. H., Benjamin, D. K., Greenberg, R. G., & Hornik, C. P. V. (2023) · Journal of the Pediatric Infectious Diseases Society · doi:10.1093/jpids/piad067 · Cited by 11 · see citing works
Mosaic GLUD1 Mutations Associated with Hyperinsulinism Hyperammonemia Syndrome
Boodhansingh, K. E., Rosenfeld, E., Lord, K., Adzick, N. S., Bhatti, T. R., Ganguly, A. A., León, D. D. D., & Stanley, C. A. (2022) · Hormone Research in Paediatrics · doi:10.1159/000526203 · Cited by 11 · see citing works
Hyperinsulinemic Hypoglycemia: Experience in A Series of 17 Cases
Ağladıoğlu, S. Y., Şenay, S. E., Semra, Ç., Baş, V. N., Kendırcı, H. N. P., Önder, A., & Zehra, A. (2013) · Journal of Clinical Research in Pediatric Endocrinology · doi:10.4274/jcrpe.991 · Cited by 11 · see citing works
Hoermann, H., Roeper, M., Dafsari, R. S., Koestner, F., Reinauer, C., Mayatepek, E., Meißner, T., & Kummer, S. (2021) · Journal of Pediatric Endocrinology and Metabolism · doi:10.1515/jpem-2020-0639 · Cited by 10 · see citing works
Low‐dose diazoxide is safe and effective in infants with transient hyperinsulinism
Malhotra, N., Yau, D., Cunjamalay, A., Gunasekara, B., Athanasakopoulou, S., Gilbert, C., Morgan, K., Dattani, M., & Dastamani, A. (2023) · Clinical Endocrinology · doi:10.1111/cen.14987 · Cited by 9 · see citing works
Hewat, T. I., Laver, T. W., Houghton, J., Männistö, J. M. E., Alvi, S., Brearey, S. P., Cody, D., Dastamani, A., Torre, M. Á. D. L. S.-L., Murphy, N., et al. (2022) · Pediatric Diabetes · doi:10.1111/pedi.13333 · Cited by 9 · see citing works
Standards of admission capillary blood glucose levels in cesarean born neonates
Smolkin, T., Ulanovsky, I., Carasso, P., & Makhoul, I. R. (2017) · World Journal of Pediatrics · doi:10.1007/s12519-017-0016-7 · Cited by 9 · see citing works
Association of maternal nutrition with transient neonatal hyperinsulinism
Louvigné, M., Rouleau, S., Caldagues, E., Souto, I., Montcho, Y., Bouvagnet, A. M., Baud, O., Carel, J., Gascoin-Lachambre, G., & Coutant, R. (2018) · PLoS ONE · doi:10.1371/journal.pone.0195383 · Cited by 9 · see citing works
Diazoxide for the Treatment of Transitional Neonatal Hypoglycemia: A Systematic Review
Laing, D., Hanning, S. M., Harding, J. E., Mravicich, L. C., & McKinlay, C. J. D. (2021) · Journal of Neonatology · doi:10.1177/09732179211059607 · Cited by 9 · see citing works
Hypoglycemia in the newborn and infant
León, D. D. D., THORNTON, P. S., Stanley, C. A., & Sperling, M. A. (2014) · Elsevier eBooks · doi:10.1016/b978-1-4557-4858-7.00015-9 · Cited by 8 · see citing works
Hypoglycemia in the Newborn and Infant
León, D. D. D., THORNTON, P. S., Stanley, C. A., & Sperling, M. A. (2021) · Elsevier eBooks · doi:10.1016/b978-0-323-62520-3.00007-5 · Cited by 8 · see citing works
Wang, Y., Liu, H., Zhang, L., Wang, X., Wang, M., Chen, Z., & Zhang, F. (2023) · BMC Endocrine Disorders · doi:10.1186/s12902-023-01532-x · Cited by 8 · see citing works
Chong, J. H., CHANDRAN, S. R., Agarwal, P., & Rajadurai, V. S. (2013) · BMJ Case Reports · doi:10.1136/bcr-2013-200920 · Cited by 8 · see citing works
Yang, T. B., McMahon, P., León, D. D. D., & Treat, J. R. (2016) · Pediatric Dermatology · doi:10.1111/pde.12975 · Cited by 8 · see citing works
Neonatal hyperinsulinemic hypoglycemia in a patient with 9p deletion syndrome
Bayat, A., Kirchhoff, M., Madsen, C. G., & Kreiborg, S. (2018) · European Journal of Medical Genetics · doi:10.1016/j.ejmg.2018.03.009 · Cited by 7 · see citing works
Hipoglucemia neonatal: revisión de las prácticas habituales
(2019) · Archivos Argentinos de Pediatria · doi:10.5546/aap.2019.s195 · Cited by 7 · see citing works
Approach to the Neonate With Hypoglycemia
Lord, K., & León, D. D. D. (2024) · The Journal of Clinical Endocrinology & Metabolism · doi:10.1210/clinem/dgae267 · Cited by 6 · see citing works
Transient hyperinsulinism in neonates
Викторовна, Б. А., Melikyan, M. A., Анатольевна, К. Н., Ionov, О. V., Ryumina, I. I., Bokerija, E. L., Pekareva, N. A., & Degtyareva, А. V. (2020) · Problems of Endocrinology · doi:10.14341/probl12572 · Cited by 6 · see citing works
A better taxonomy for neonatal hypoglycemia is needed
McKinlay, C. J. D., Alsweiler, J. M., Bailey, M. J., Cutfield, W. S., Rout, A., & Harding, J. E. (2021) · Journal of Perinatology · doi:10.1038/s41372-021-01058-x · Cited by 6 · see citing works
Laing, D., Walsh, E. P. G., Alsweiler, J. M., Hanning, S. M., Meyer, M. P., Ardern, J., Cutfield, W. S., Rogers, J., Gamble, G. D., Chase, J. G., et al. (2022) · BMJ Open · doi:10.1136/bmjopen-2021-059452 · Cited by 5 · see citing works
Diazoxide-Responsive Forms of Congenital Hyperinsulinism
Yau, D., & Stanley, C. A. (2019) · Contemporary Endocrinology · doi:10.1007/978-3-030-02961-6_2 · Cited by 5 · see citing works
Immediate Metabolic Consequences of Intrauterine Growth Restriction and Low Birthweight
Bhatia, J. J. S., & Gates, A. (2013) · Nestlé Nutrition Institute Workshop series · doi:10.1159/000348474 · Cited by 5 · see citing works
Hypoglycemia: Symptom or Diagnosis?
Halaby, L. P., & Steinkrauss, L. J. (2012) · Journal of Pediatric Nursing · doi:10.1016/j.pedn.2011.10.005 · Cited by 5 · see citing works
Recent updates in the management of infants and children with hyperinsulinism
THORNTON, P. S. (2021) · Current Opinion in Pediatrics · doi:10.1097/mop.0000000000001022 · Cited by 5 · see citing works
The IUGR infant: A case study and associated problems with IUGR infants
Smith, J. M., Murphy, M., & Kandasamy, Y. (2013) · Journal of Neonatal Nursing · doi:10.1016/j.jnn.2012.12.005 · Cited by 5 · see citing works
Hoermann, H., Roeper, M., Welters, A., Baertling, F., Mayatepek, E., Meißner, T., & Kummer, S. (2023) · The Journal of Pediatrics · doi:10.1016/j.jpeds.2023.113399 · Cited by 4 · see citing works
Disorders of Carbohydrate Metabolism
Werny, D. M., Taplin, C. E., Bennett, J. T., & Pihoker, C. (2018) · Elsevier eBooks · doi:10.1016/b978-0-323-40139-5.00099-1 · Cited by 4 · see citing works
Early postnatal activation of the hypoxia pathway disrupts β-cell functionPreprint
Yang, J., Hammoud, B., Ridler, A., Won, K., Hoshi, T., Stanley, C. A., Stanescu, D. E., & Ackermann, A. M. (2021) · bioRxiv (Cold Spring Harbor Laboratory) · doi:10.1101/2021.06.09.447705 · Cited by 4 · see citing works
Congenital Hyperinsulinism of a Large Italian Cohort: A Retrospective Study
Tagliaferri, F., Iannuzzi, R., Canciani, G., Bernabei, S. M., Campana, C., Caviglia, S., Greco, B., Lepri, F. R., Novelli, A., Pizzoferro, M., et al. (2024) · Hormone Research in Paediatrics · doi:10.1159/000538943 · Cited by 3 · see citing works
Hiperinsulinismo congénito del recién nacido: A propósito de un caso clínico
Josefina Sáez, B., Pattillo, J. C., Orellana, P., & Godoy, C. (2017) · Revista chilena de pediatría · doi:10.4067/s0370-41062017000300010 · Cited by 3 · see citing works
Blanco, M., Khan, O., Stanley, K., Hageman, J. R., & Greeley, S. A. W. (2014) · Pediatric Annals · doi:10.3928/00904481-20140221-08 · Cited by 3 · see citing works
Rodrigues, M., Rana, P., Lee, G. T., Mahajan, C. N., Nyp, M. F., & Pandey, V. (2021) · Journal of Pediatric Endocrinology and Metabolism · doi:10.1515/jpem-2021-0515 · Cited by 3 · see citing works
Etiology of the Neonatal Hypoglycemias.
Stanley, C. A., & León, D. D. (2024) · Advances in Pediatrics · doi:10.1016/j.yapd.2024.03.001 · Cited by 3 · see citing works
Barbosa, A. D. M., Figueiredo, I., & de Lima, G. M. (2018) · InTech eBooks · doi:10.5772/intechopen.69676 · Cited by 3 · see citing works
- Authors
- Hoe, F. M., Thornton, P. S., Wanner, L. A., Steinkrauss, L., Simmons, R. A., & Stanley, C. A.
- Affiliations
- Children's Hospital of Philadelphia (US)
- Corresponding author
- Stanley, Charles A.
- Venue
- The Journal of Pediatrics
- Publisher
- Elsevier BV
- Published
- 2006-02
- Volume
- 148
- Issue
- 2
- Pages
- 207-212
- ISSN
- 0022-3476 (ISSN-L)
- OpenAlex
- W2050708063
- Cited by
- 174
- References
- 36
- Citations by year
- 2026: 6 · 2025: 3 · 2024: 9 · 2023: 14 · 2022: 11 · 2021: 16 · 2020: 17 · 2019: 8 · 2018: 15 · 2017: 9 · 2016: 9 · 2015: 10 · 2014: 6 · 2013: 9 · 2012: 3
- FWCI
- 6.10
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- The Journal of Pediatrics (publishedVersion)
- PubMed (publishedVersion)
- Funders
- National Institute of Diabetes and Digestive and Kidney Diseases (R01-DK 56268, R01DK056268)
- National Center for Research Resources (M01-RR-00240, M01RR000240)
- Topics
- Health Sciences › Medicine › Surgery › Pancreatic function and diabetes
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Diabetes and associated disorders
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Clinical Biochemistry › Metabolism and Genetic Disorders
- Keywords
- neonatal hyperinsulinism, neonatal hypoglycemia, insulin regulation, acute insulin response, diazoxide, ATP-sensitive potassium channels, glutamate dehydrogenase, spontaneous resolution, small for gestational age
- MeSH terms
- Insulin Secretion, Antihypertensive Agents, Calcium, Diazoxide, Enteral Nutrition, Fasting, Female, Glucose, Humans, Hyperinsulinism, Hypoglycemia, Hypoglycemic Agents, Infant, Infant, Newborn, Infusions, Intravenous, Insulin, Leucine, Male, Prospective Studies, Risk Factors, Sweetening Agents, Tolbutamide, Case-Control Studies
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