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Congenital Hyperinsulinism

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Pediatric Surgery

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Abstract

Congenital hyperinsulinism (CHI) comprises a group of disorders characterized by excessive insulin secretion from pancreatic β-cells, resulting in severe hypoglycemia. In neonates and infants, CHI is considered the most frequent cause of persistent hypoglycemia. Prompt diagnosis and treatment are crucial as a delay may cause severe brain damage and long-term neurodevelopmental disability. Therapeutic strategies for CHI can be medical, surgical, or combined.

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References

  • Arnoux JB, Verkarre V, Saint-Martin C, et al. Congenital hyperinsulinismcurrent trends in diagnosis and therapy. Orphanet J Rare Dis. 2011;6:63.

    Article  PubMed  PubMed Central  Google Scholar 

  • Ferrara C, Patel P, Becker S, Stanley CA, Kelly A. Biomarkers of insulin for the diagnosis of hyperinsulinemic hypoglycemia in infants and children. J Pediatr. 2016;168:212–9.

    Article  CAS  PubMed  Google Scholar 

  • Kapoor RR, Locke J, Colclough K, et al. Persistent hyperinsulinemic hypoglycemia and maturity-onset diabetes of the young due to heterozygous HNF4A mutations. Diabetes. 2008;57:1659–63.

    Article  CAS  PubMed  Google Scholar 

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

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Nessa A, Rahman SA, Hussain K. Hyperinsulinemic hypoglycemia – the molecular mechanisms. Front Endocrinol (Lausanne). 2016;7:29.

    Google Scholar 

  • Pierro A, Nah SA. Surgical management of congenital hyperinsulinism of infancy. Semin Pediatr Surg. 2011;20(1):50–3.

    Article  PubMed  Google Scholar 

  • Roženková K, Güemes M, Shah P, Hussain K. The diagnosis and management of Hyperinsulinaemic hypoglycaemia. J Clin Res Pediatr Endocrinol. 2015;7(2):86–97.

    Article  PubMed  PubMed Central  Google Scholar 

  • Shah P, Demirbilek H, Hussain K. Persistent hyperinsulinaemic hypoglycaemia in infancy. Semin Pediatr Surg. 2014;23(2):76–82.

    Article  PubMed  Google Scholar 

  • Stanley CA. Perspective on the genetics and diagnosis of congenital hyperinsulinism disorders. J Clin Endocrinol Metab. 2016;101(3):815–26.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Yorifuji T. Congenital hyperinsulinism: current status and future perspectives. Ann Pediatr Endocrinol Metab. 2014;19(2):57–68.

    Article  PubMed  PubMed Central  Google Scholar 

  • Zani A, Nah SA, Ron O, et al. The predictive value of preoperative fluorine-18-l-3,4-dihydroxyphenylalanine positron emission tomography-computed tomography scans in children with congenital hyperinsulinism of infancy. J Pediatr Surg. 2011;46(1):204–8.

    Article  PubMed  Google Scholar 

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Authors and Affiliations

  1. Division of General and Thoracic Surgery, The Hospital for Sick Children, University of Toronto, 1524C-555 University Ave, M5G 1X8, Toronto, Ontario, Canada

    Augusto Zani & Agostino Pierro

Authors
  1. Augusto Zani
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  2. Agostino Pierro
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Corresponding author

Correspondence to Augusto Zani .

Editor information

Editors and Affiliations

  1. Our Lady’s Children's Hospital, National Children’s Research Centre, Dublin, Ireland

    Prem Puri

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© 2017 Springer-Verlag GmbH Germany

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Zani, A., Pierro, A. (2017). Congenital Hyperinsulinism. In: Puri, P. (eds) Pediatric Surgery. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-38482-0_65-1

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  • DOI: https://doi.org/10.1007/978-3-642-38482-0_65-1

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  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-642-38482-0

  • Online ISBN: 978-3-642-38482-0

  • eBook Packages: Springer Reference MedicineReference Module Medicine

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