The DOI matches its Crossref record; 36 of the 36 references it cites were found in a registry or an index.
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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“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]
In the citing paper
“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]
Citations
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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
“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
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
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
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
Metadata
- 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 › Endocrinology, Diabetes and Metabolism › 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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