Congenital Hyperinsulinism Center

Resources for Professionals

Diagnosis and treatment of hyperinsulinism algorithm

Source: Lord K, De León DD. Monogenic hyperinsulinemic hypoglycemia: current insights into the pathogenesis and management. Int J Pediatr Endocrinol. 2013 Feb 6;2013(1):3.

Table 1 - Criteria for diagnosing hyperinsulinism
Figure 2 - Algorithm for the treatment of hyperinsulinism

Please call the CHOP HI Center with any questions or when you are ready to refer patients: 215-590-7682.

Informational brochures and resources from CHOP

Networking

Hyperinsulinism Discussion Group (English language)

Websites

Congenital Hyperinsulinism

Diabetes

Research articles

Hardy OT, Hernandez-Pampaloni M, Saffer JR, Suchi M, Ruchelli E, Zhuang H, et al. Diagnosis and localization of focal congenital hyperinsulinism by 18F-fluorodopa PET scan. J Pediatr. 2007 Feb;150(2):140-5.

Hardy OT, Hernandez-Pampaloni M, Saffer JR, Scheuermann JS, Ernst LM, Freifelder R, et al. Accuracy of [18F]fluorodopa positron emission tomography for diagnosing and localizing focal congenital hyperinsulinism. J Clin Endocrinol Metab. 2007 Dec;92(12):4706-11.

Peranteau WH, Bathaii SM, Pawel B, Hardy O, Alavi A, Stanley CA, Adzick NS. Multiple ectopic lesions of focal islet adenomatosis identified by positron emission tomography scan in an infant with congenital hyperinsulinism. J Pediatr Surg. 2007 Jan;42(1):188-92.

Adzick NS, Thornton PS, Stanley CA, Kaye RD, Ruchelli E. A multidisciplinary approach to the focal form of congenital hyperinsulinism leads to successful treatment by partial pancreatectomy. J Pediatr Surg. 2004 Mar;39(3):270-5.

Dunne MJ, Shepherd RM, Cosgrove KE, Macfarlane WM, Lindley KJ, James RF, et al. Persistent hyperinsulinaemic hyperglycaemia of infancy-derived cells; implications for beta-cells that replicate in vitro. J Mol Endocrinol. 2000 Jun;24(3):313-20.

Glaser B, Kesavan P, Heyman M, Davis E, Cuesta A, Buchs A, et al. Familial hyperinsulinism caused by an activating glucokinase mutation. New Engl J Med. 1998 Jan 22;338(4)226-30.

Glaser B, Thornton P, Otonkoski T, Junien C. Genetics of neonatal hyperinsulinism. Arch Dis Child Fetal Neonatal Ed. 2000 Mar;82(2):79-86.

Nestorowicz A, Glaser B, Wilson BA, Shyng SL, Nichols CG, Stanley CA, et al. Genetic heterogeneity in familial hyperinsulinism [published erratum appears in Hum Mol Genet 1998 Sep;7(9):1527]. Hum Mol Genet. 1998 Jul;7(7):1119-28.

Ryan F, Devaney D, Joyce C, Nestorowicz A, Permutt MA, Glaser B, et al. Hyperinsulinism: molecular aetiology of focal disease. Arch Dis Child. 1998 Nov;79(5):445-7.

Stanley CA, Lieu YK, Hsu BY, Burlina AB, Greenberg CR, Hopwood NJ, et al. Hyperinsulinism and hyperammonemia in infants with regulatory mutations of the glutamate dehydrogenase gene. N Engl J Med. 1998 May 7;338(19):1352-7.

Stanley CA, Fang J, Kutyna K, Hsu BY, Ming JE, Glaser B, Poncz M. Molecular basis and characterization of the hyperinsulinism/hyperammonemia syndrome: predominance of mutations in exons 11 and 12 of the glutamate dehydrogenase gene. HI/HA Contributing Investigators. Diabetes. 2000 Apr;49(4):667-73.

Thomas PM, Cote GJ, Wohllk N, Haddad B, Mathew PM, Rabl W, et al. Mutations in the sulfonylurea receptor gene in familial persistent hyperinsulinemic hypoglycemia of infancy. [see comments]. Science. 1995 Apr 21;268(5209):426-9.

Thornton PS, Satin-Smith MS, Herold K, Glaser B, Chiu KC, Nestorowicz A, et al. Familial hyperinsulinism with apparent autosomal dominant inheritance: clinical and genetic differences from the autosomal recessive variant. [see comments]. J Pediatr. 1998 Jan;132(1):9-14.

Weinzimer SA, Stanley CA, Berry GT, Yudkoff M, Tuchman M, Thornton PS. A syndrome of congenital hyperinsulinism and hyperammonemia. [see comments]. J Pediatr. 1997 Apr;130(4):661-4.

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