Cited by 174Pancreatic function and diabetesDiabetes and associated disordersMetabolism and Genetic Disorders

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

Hoe, Francis M., Thornton, Paul S., Wanner, Laura A., Steinkrauss, Linda, Simmons, Rebecca A., Stanley, Charles A.·2006·The Journal of Pediatrics

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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Crossref · 10.1016/j.jpeds.2005.10.002

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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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