Congenital Hyperinsulinism Surgery Research

Surgeons at Children’s Hospital of Philadelphia (CHOP) perform more surgeries to treat congenital hyperinsulinism than any other center. Learn more about our research efforts to improve outcomes for children having surgery to treat HI.


Surgical treatment of congenital hyperinsulinism. Adzick NS. Semin Pediatr Surgery. 2020;29(3):150924. 


Surgical treatment of congenital hyperinsulinism: Results from 500 pancreatectomies in neonates and children. Adzick NS, De Leon D, States L, Lord K. Bhatti T, Becker S, Stanley C. JPediatric Surg. 2019;54(1), 27-32. 


High risks of diabetes and neurobehavioral deficits in individuals with surgically treated hyperinsulinism. Lord K, Radcliffe J, Gallagher PR, Adzick NS, Stanley CA, De León DD. J Clin Endocrinol Metab. 2015;100(11):4133-9. 


Laje P, Palladino AA, Bhatti TR, States LJ, Stanley CA, Adzick NS. Pancreatic surgery in infants with Beckwith-Wiedemann Syndrome and Hyperinsulinism. J Pediatr Surg. 2013 Dec;48(12):2511-6.


Laje P, Stanley CA, Palladino AA, Becker SA, Adzick NS. Pancreatic head resection and Roux-en-Y pancreaticojejunostomy for the treatment of the focal form of congenital hyperinsulinism. J Pediatr Surg. 2012 Jan;47(1):130-5.


Macmullen CM, Zhou Q, Snider KE, Tewson PH, Becker SA, Aziz AR, Ganguly A, Shyng SL, Stanley CA. Diazoxide-unresponsive congenital hyperinsulinism in children with dominant mutations of the -cell sulfonylurea receptor SUR1. [Erratum appears in Diabetes. 2011 Nov;60(11):3097] Diabetes. 60(6):1797-804, 2011 Jun.


Laje P, Stanley CA, Adzick NS. Intussusception after pancreatic surgery in children: a case series. Journal of Pediatric Surgery. 45(7):1496-9, 2010 Jul.

Necrotizing enterocolitis in neonates receiving octreotide for the management of congenital hyperinsulinism. [Review] [32 refs] Laje P. Halaby L. Adzick NS. Stanley CA. Pediatric Diabetes. 11(2):142-7, 2010 Mar.

Palladino AA, Stanley CA. GDH-HI (Hyperinsulinism-Hyperammonemia) The hyperinsulinism/hyperammonemia syndrome. [Review] Reviews in Endocrine & Metabolic Disorders. 11(3):171-8, 2010 Sep.


Neurological aspects of hyperinsulinism-hyperammonaemia syndrome. [Erratum appears in Dev Med Child Neurol. 2009 Jan;51(1):77. Note: Chadefaux-Vekemans, Bernadette [added]; Dan, Bernard [added]; de Lonlay, Pascale [added]]

Sayed S, Langdon DR, Odili S, Chen P, Buettger C, Schiffman AB, Suchi M, Taub R, Grimsby J, Matschinsky FM, Stanley CA. Extremes of clinical and enzymatic phenotypes in children with hyperinsulinism caused by glucokinase activating mutations. Diabetes. 58(6):1419-27, 2009 Jun. [Case Reports. Journal Article. Research Support, N.I.H., Extramural]

Meissner T, Marquard J, Cobo-Vuilleumier N, Maringa M, Rodriguez-Bada P, Garcia-Gimeno MA, Baixeras E, Weber J, Olek K, Sanz P, Mayatepek E, Cuesta-Munoz AL. Diagnostic difficulties in glucokinase hyperinsulinism. Hormone & Metabolic Research. 41(4):320-6, 2009 Apr. [Case Reports. Journal Article. Research Support, Non-U.S. Gov't]


Bahi-Buisson N. Roze E. Dionisi C. Escande F. Valayannopoulos V. Feillet F. Heinrichs C. Chadefaux-Vekemans B. Dan B. de Lonlay P. Developmental Medicine & Child Neurology. 50(12):945-9, 2008 Dec.

Pinney SE, MacMullen C, Becker S, Lin YW, Hanna C, Thornton P, Ganguly A, Shyng SL, Stanley CA. Clinical characteristics and biochemical mechanisms of congenital hyperinsulinism associated with dominant KATP channel mutations. Journal of Clinical Investigation. 118(8):2877-86, 2008 Aug. [Journal Article]


Peranteau WH, Ganguly A, Steinmiller L, Thornton P, Johnson MP, Howell LJ, Adzick NS, Stanley CA: Prenatal diagnosis and postnatal management of diffuse congenital hyperinsulinism: A case report. Fetal Diagnosis and Therapy 21:515-518, 2006.


Magge SN, Shyng SL, MacMullen C, Steinkrauss L, Ganguly A, Katz LE. Stanley CA. Familial leucine-sensitive hypoglycemia of infancy due to a dominant mutation of the beta-cell sulfonylurea receptor. Journal of Clinical Endocrinology & Metabolism. 89(9):4450-6, 2004 Sep.

Stanley CA, Thornton PS, Ganguly A, MacMillen C, Underwood P, Bhatia P, Steinkrauss L, Kaye R, Ruchelli E, Suchi M, Adzick NS: Preoperative evaluation of infants with focal or diffuse congenital hyperinsulinism by intravenous acute insulin response tests and selective pancreatic arterial calcium stimulation. Journal of Clinical Endocrinology and Metabolism 89:288-296, 2004.

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 39:270-275, 2004.


Hsu BY, Kelly A, Thornton PS, Greenberg CR, Dilling LA, Stanley CA. Protein-sensitive and fasting hypoglycemia in children with the hyperinsulinism/hyperammonemia syndrome. Journal of Pediatrics. 138(3):383-9, 2001 Mar.


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.


Lovvorn HN, Nance ML, Ferry RJ, Stolte L, Baker L, O’Neill JA, Schnaufer L, Stanley CA, Adzick NS: Congenital hyperinsulinism and the surgeon: Lessons learned over 35 years. J Pediatr Surg 34:786-793, 1999.


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.

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.


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