eMedicine Specialties > Pediatrics: General Medicine > Endocrinology

Hyperinsulinemia: Multimedia

Author: Robert J Ferry Jr, MD, Chief, Division of Pediatric Endocrinology and Diabetes, Le Bonheur Children's Medical Center, University of Tennessee Health Science Center at Memphis and St Jude Children's Research Hospital; Lieutenant Colonel (Medical Corps), 162nd Area Support Medical Company, Army National Guard
Coauthor(s): Kenneth Kwok-Chun Chan, MD, Consulting Staff, Department of Pediatrics, Andover Pediatrics; Ab Sadeghi-Nejad, MD, Chief, Division of Pediatric Endocrinology and Metabolism, New England Medical Center; Professor, Department of Pediatrics, Tufts University School of Medicine
Contributor Information and Disclosures

Updated: Jul 9, 2008

Multimedia

Mechanisms of insulin secretion.Media file 1: Mechanisms of insulin secretion.
Mechanisms of insulin secretion.

Mechanisms of insulin secretion.

More on Hyperinsulinemia

Overview: Hyperinsulinemia
Differential Diagnoses & Workup: Hyperinsulinemia
Treatment & Medication: Hyperinsulinemia
Follow-up: Hyperinsulinemia
Multimedia: Hyperinsulinemia
References

References

  1. Abdulhadi-Atwan M, Bushmann J, et al. Novel de novo mutation in sulfonylurea receptor 1 presenting as hyperinsulinism in infancy followed by overt diabetes in early adolescence. Diabetes. Jul 2008;57(7):1935-40. [Medline].

  2. Arbizu Lostao J, Fernandez-Marmiesse A, Garrastachu Zumarran P, et al. [18F-fluoro-L-DOPA PET-CT imaging combined with genetic analysis for optimal classification and treatment in a child with severe congenital hyperinsulinism.]. An Pediatr (Barc). May 2008;68(5):481-5. [Medline].

  3. Glaser B, Kesavan P, Heyman M, et al. Familial hyperinsulinism caused by an activating glucokinase mutation. N Engl J Med. 1998;338:226-30. [Medline].

  4. Grimberg A, Ferry RJ Jr, Kelly A, et al. Dysregulation of insulin secretion in children with congenital hyperinsulinism due to sulfonylurea receptor mutations. Diabetes. 2001;50:322-8. [Medline].

  5. Shah JH, Maguire DJ, Munce TB, Cotterill A. Alanine in HI: a silent mutation cries out!. Adv Exp Med Biol. 2008;614:145-50. [Medline].

  6. Stanley CA, Baker L. The causes of neonatal hypoglycemia. N Engl J Med. Apr 15 1999;340(15):1200-1. [Medline].

  7. Stanley CA, Lieu YK, Hsu BY, et al. Hyperinsulinism and hyperammonemia in infants with regulatory mutations of the glutamate dehydrogenase gene. N Engl J Med. 1998;338:1352-7. [Medline].

  8. Suchi M, MacMullen CM, Thornton PS. Molecular and immunohistochemical analyses of the focal form of congenital hyperinsulinism. Mod Pathol. 2006;19:122-9. [Medline].

  9. Thomas PM, Cote GJ, Wohllk N, et al. Mutations in the sulfonylurea receptor gene in familial persistent hyperinsulinemic hypoglycemia of infancy. Science. 1995;268:426-9. [Medline].

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  11. Craver RD, Hill CB. Cure of hypoglycemic hyperinsulinism by enucleation of a focal islet cell adenomatous hyperplasia. J Pediatr Surg. 1997;32:1526-7. [Medline].

  12. Cucchiaro G, Markowitz SD, Kaye R, et al. Blood glucose control during selective arterial stimulation and venous sampling for localization of focal hyperinsulinism lesions in anesthetized children. Anesth Analg. 2004;99:1044-8, table of contents. [Medline].

  13. De Leon DD, Stanley CA. Mechanisms of Disease: advances in diagnosis and treatment of hyperinsulinism in neonates. Nat Clin Pract Endocrinol Metab. 2007;3:57-68. [Medline].

  14. de Lonlay-Debeney P, Poggi-Travert F, Fournet JC. Clinical features of 52 neonates with hyperinsulinism. N Engl J Med. 1999;340:1169-75. [Medline].

  15. Ferry RJ Jr, Franklin SL, Geffner ME. Hypoglycemia. In: Kappy MS, Allen DB, Geffner ME, eds. Principles and Practice of Pediatric Endocrinology. Springfield, Ill: Charles C Thomas Publisher, Ltd; 2005:607-34.

  16. Ferry RJ Jr, Kelly A, Grimberg A, et al. Calcium-stimulated insulin secretion in diffuse and focal forms of congenital hyperinsulinism. J Pediatr. 2000;137:239-46. [Medline].

  17. Hoe FM, Thornton PS, Wanner LA. Clinical features and insulin regulation in infants with a syndrome of prolonged neonatal hyperinsulinism. J Pediatr. Feb 2006;148(2):207-12. [Medline].

  18. Hussain K, Aynsley-Green A, Stanley CA. Medications used in the treatment of hypoglycemia due to congenital hyperinsulinism of infancy (HI). Pediatr Endocrinol Rev. Nov 2004;2 Suppl 1:163-7. [Medline].

  19. Kane C, Shepherd RM, Squires PE, et al. Loss of functional KATP channels in pancreatic beta-cells causes persistent hyperinsulinemic hypoglycemia of infancy. Nat Med. 1996;2:1344-7. [Medline].

  20. Levitt Katz LE, Satin-Smith MS, Collett-Solberg P, et al. Insulin-like growth factor binding protein-1 levels in the diagnosis of hypoglycemia caused by hyperinsulinism. J Pediatr. Aug 1997;131(2):193-9. [Medline].

  21. Lovvorn HN III, Nance ML, Ferry RJ Jr. Congenital hyperinsulinism and the surgeon: lessons learned over 35 years. J Pediatr Surg. 1999;34:786-92; discussion 792-3. [Medline].

  22. Palladino AA, Bennett MJ, Stanley CA. Hyperinsulinism in infancy and childhood: when an insulin level is not always enough. Clin Chem. 2008;54:256-63. [Medline].

  23. Stanley CA. Hyperinsulinism/hyperammonemia syndrome: insights into the regulatory role of glutamate dehydrogenase in ammonia metabolism. Mol Genet Metab. Apr 2004;81 Suppl 1:S45-51. [Medline].

  24. Steinkrauss L, Lipman TH, Hendell CD. Effects of hypoglycemia on developmental outcome in children with congenital hyperinsulinism. J Pediatr Nurs. Apr 2005;20(2):109-18. [Medline].

  25. Suchi M, Thornton PS, Adzick NS, et al. Congenital hyperinsulinism: intraoperative biopsy interpretation can direct the extent of pancreatectomy. Am J Surg Pathol. Oct 2004;28(10):1326-35. [Medline].

Further Reading

Keywords

hyperinsulinemia, beta-cell hyperplasia, hyperinsulinemic hypoglycemia, nesidioblastosis, neonatal hyperglycemia, primary hyperinsulinemia, hypoglycemia in children, hypoglycemia in infants, hyperinsulinism, growth hormone deficiency, hypocortisolemia, maternal diabetes, birth asphyxia, macrosomia, Beckwith-Wiedemann syndrome, omphalocele, macroglossia, visceromegaly, drug-induced hyperinsulinism, maternal toxemia, erythroblastosis fetalis, Munchausen syndrome by proxy, nesidioblastosis, islet adenomatosis, beta-cell adenoma, persistent hyperinsulinemic hypoglycemia of infancy, PHHI, leucine-sensitive hypoglycemia, islet cell dysmaturation syndrome

Contributor Information and Disclosures

Author

Robert J Ferry Jr, MD, Chief, Division of Pediatric Endocrinology and Diabetes, Le Bonheur Children's Medical Center, University of Tennessee Health Science Center at Memphis and St Jude Children's Research Hospital; Lieutenant Colonel (Medical Corps), 162nd Area Support Medical Company, Army National Guard
Robert J Ferry Jr, MD is a member of the following medical societies: American Academy of Pediatrics, American Diabetes Association, American Medical Association, Endocrine Society, Lawson-Wilkins Pediatric Endocrine Society, Society for Pediatric Research, and Texas Pediatric Society
Disclosure: Nutropin Speakers Bureau Honoraria Speaking and teaching

Coauthor(s)

Kenneth Kwok-Chun Chan, MD, Consulting Staff, Department of Pediatrics, Andover Pediatrics
Kenneth Kwok-Chun Chan, MD is a member of the following medical societies: American Academy of Pediatrics
Disclosure: Nothing to disclose.

Ab Sadeghi-Nejad, MD, Chief, Division of Pediatric Endocrinology and Metabolism, New England Medical Center; Professor, Department of Pediatrics, Tufts University School of Medicine
Ab Sadeghi-Nejad, MD is a member of the following medical societies: American Academy of Pediatrics, American Association for the Advancement of Science, American Pediatric Society, Endocrine Society, Lawson-Wilkins Pediatric Endocrine Society, Massachusetts Medical Society, and Society for Pediatric Research
Disclosure: Nothing to disclose.

Medical Editor

Thomas A Wilson, MD, Professor of Clinical Pediatrics, Department of Pediatrics; Director of Pediatric Endocrinology, Division of Pediatric Endocrinology, Department of Pediatrics, State University of New York at Stony Brook
Thomas A Wilson, MD is a member of the following medical societies: Endocrine Society, Lawson-Wilkins Pediatric Endocrine Society, and Phi Beta Kappa
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine.com, Inc
Disclosure: Pfizer Inc Stock Investment from broker recommendation; Avanir Pharma Stock Investment from broker recommendation

Managing Editor

George P Chrousos, MD, FAAP, MACP, MACE, Professor and Chair, Department of Pediatrics, Athens University Medical School
George P Chrousos, MD, FAAP, MACP, MACE is a member of the following medical societies: American Academy of Pediatrics, American College of Endocrinology, American College of Physicians, American Pediatric Society, American Society for Clinical Investigation, Association of American Physicians, Endocrine Society, Lawson-Wilkins Pediatric Endocrine Society, and Society for Pediatric Research
Disclosure: Nothing to disclose.

CME Editor

Merrily P M Poth, MD, Professor, Department of Pediatrics and Neuroscience, Uniformed Services University of the Health Sciences
Merrily P M Poth, MD is a member of the following medical societies: American Academy of Pediatrics, Endocrine Society, and Lawson-Wilkins Pediatric Endocrine Society
Disclosure: Nothing to disclose.

Chief Editor

Stephen Kemp, MD, PhD, Professor, Department of Pediatrics, Section of Pediatric Endocrinology, University of Arkansas and Arkansas Children's Hospital
Stephen Kemp, MD, PhD is a member of the following medical societies: American Academy of Pediatrics, American Association of Clinical Endocrinologists, American Pediatric Society, Endocrine Society, Phi Beta Kappa, Southern Medical Association, and Southern Society for Pediatric Research
Disclosure: Genentech, Inc. Honoraria Speaking and teaching; Pfiser, Inc. Honoraria Consulting

 
 
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