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Management of the Child with Persistent Hypoglycemia After Surgery

  • Amanda M. Ackermann
  • Diva D. De León-CrutchlowEmail author
Chapter
Part of the Contemporary Endocrinology book series (COE)

Abstract

Most children with focal hyperinsulinism are cured after surgical excision of the lesion; however, almost half of children with diffuse hyperinsulinism have persistent hypoglycemia after near-total pancreatectomy. A comprehensive assessment of the glycemic status should be undertaken once the patient is on a stable feeding regimen for several days. This assessment includes performing a Cure Fast to demonstrate the resolution of hyperinsulinism in children with focal hyperinsulinism, evaluation for the possibility of post-pancreatectomy diabetes after near-total pancreatectomy, and assessment for persistent hypoglycemia in children with diffuse hyperinsulinism. Treatment of persistent hyperinsulinism following pancreatectomy requires an individualized approach and often requires a combination of therapies to achieve a suitable regimen that can safely and reasonably be carried out at home.

Keywords

Pancreatectomy Hyperglycemia Hypoglycemia 

References

  1. 1.
    ADA. Diagnosis and classification of diabetes mellitus. Diabetes Care. 2014;37:S81.CrossRefGoogle Scholar
  2. 2.
    Jacobs DG, Haka-Ikse K, Wesson DE, Filler RM, Sherwood G. Growth and development in patients operated on for islet cell dysplasia. J Pediatr Surg. 1986;21:1184–9.  https://doi.org/10.1016/0022-3468(86)90035-7.CrossRefPubMedGoogle Scholar
  3. 3.
    Al-Rabeeah A, et al. Persistent hyperinsulinemic hypoglycemia of infancy: experience with 28 cases. J Pediatr Surg. 1995;30:1119–21.  https://doi.org/10.1016/0022-3468(95)90001-2.CrossRefPubMedGoogle Scholar
  4. 4.
    Leibowitz G, et al. Hyperinsulinemic hypoglycemia of infancy (nesidioblastosis) in clinical remission: high incidence of diabetes mellitus and persistent beta-cell dysfunction at long-term follow-up. J Clin Endocrinol Metab. 1995;80:386–92.  https://doi.org/10.1210/jcem.80.2.7852494.CrossRefPubMedGoogle Scholar
  5. 5.
    Soliman AT, Alsalmi I, Darwish A, Asfour MG. Growth and endocrine function after near total pancreatectomy for hyperinsulinaemic hypoglycaemia. Arch Dis Child. 1996;74:379–85.CrossRefGoogle Scholar
  6. 6.
    Cade A, Walters M, Puntis J, Arthur R, Stringer M. Pancreatic exocrine and endocrine function after pancreatectomy for persistent hyperinsulinaemic hypoglycaemia of infancy. Arch Dis Child. 1998;79:435–9.CrossRefGoogle Scholar
  7. 7.
    Rother KI, Matsumoto JM, Rasmussen NH, Schwenk WF. Subtotal pancreatectomy for hypoglycemia due to congenital hyperinsulinism: long-term follow-up of neurodevelopmental and pancreatic function. Pediatr Diabetes. 2001;2:115–22.  https://doi.org/10.1034/j.1399-5448.2001.002003115.x.CrossRefPubMedGoogle Scholar
  8. 8.
    Meissner T, Wendel U, Burgard P, Schaetzle S, Mayatepek E. Long-term follow-up of 114 patients with congenital hyperinsulinism. Eur J Endocrinol. 2003;149:43–51.  https://doi.org/10.1530/eje.0.1490043.CrossRefPubMedGoogle Scholar
  9. 9.
    Cherian MP, Abduljabbar MA. Persistent hyperinsulinemic hypoglycemia of infancy (PHHI): long-term outcome following 95% pancreatectomy. JPEM. 2005;18:1441–8.CrossRefGoogle Scholar
  10. 10.
    Beltrand J, et al. Glucose metabolism in 105 children and adolescents after pancreatectomy for congenital hyperinsulinism. Diabetes Care. 2012;35:198–203.  https://doi.org/10.2337/dc11-1296.CrossRefPubMedPubMedCentralGoogle Scholar
  11. 11.
    Lord K, Dzata E, Snider KE, Gallagher PR, De Leon DD. Clinical presentation and management of children with diffuse and focal hyperinsulinism: a review of 223 cases. J Clin Endocrinol Metab. 2013;98:E1786–9.  https://doi.org/10.1210/jc.2013-2094.CrossRefPubMedPubMedCentralGoogle Scholar
  12. 12.
    Lord K, et al. High risk of diabetes and neurobehavioral deficits in individuals with surgically treated hyperinsulinism. J Clin Endocrinol Metab. 2015;100:4133–9.  https://doi.org/10.1210/jc.2015-2539.CrossRefPubMedPubMedCentralGoogle Scholar
  13. 13.
    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;39:270–5.  https://doi.org/10.1016/j.jpedsurg.2003.11.019.CrossRefPubMedGoogle Scholar
  14. 14.
    Suchi M, et al. Congenital hyperinsulinism: intraoperative biopsy interpretation can direct the extent of pancreatectomy. Am J Surg Pathol. 2004;28:1326–35.CrossRefGoogle Scholar
  15. 15.
    Laje P, et al. Pancreatic surgery in infants with Beckwith-Wiedemann syndrome and hyperinsulinism. J Pediatr Surg. 2013;48:2511–6.  https://doi.org/10.1016/j.jpedsurg.2013.05.016.CrossRefPubMedPubMedCentralGoogle Scholar
  16. 16.
    Hussain K, et al. The diagnosis of ectopic focal hyperinsulinism of infancy with [18F]-dopa positron emission tomography. J Clin Endocrinol Metab. 2006;91:2839–42.  https://doi.org/10.1210/jc.2006-0455.CrossRefPubMedGoogle Scholar
  17. 17.
    Reubi JC, Schonbrunn A. Illuminating somatostatin analog action at neuroendocrine tumor receptors. Trends Pharmacol Sci. 2013;34:676–88.  https://doi.org/10.1016/j.tips.2013.10.001.CrossRefPubMedGoogle Scholar
  18. 18.
    Harris AG. Somatostatin and somatostatin analogues: pharmacokinetics and pharmacodynamic effects. Gut. 1994;35:S1–4.  https://doi.org/10.1136/gut.35.3_Suppl.S1.CrossRefPubMedPubMedCentralGoogle Scholar
  19. 19.
    Thornton PS, Alter CA. Short- and long-term use of octreotide in the treatment of congenital hyperinsulinism. J Pediatr. 1993;123:637–43.  https://doi.org/10.1016/S0022-3476(05)80969-2.CrossRefPubMedGoogle Scholar
  20. 20.
    Le Quan Sang K-H, et al. Successful treatment of congenital hyperinsulinism with long-acting release octreotide. Eur J Endocrinol. 2012;166:333–9.  https://doi.org/10.1530/eje-11-0874.CrossRefPubMedGoogle Scholar
  21. 21.
    Modan-Moses D, Koren I, Mazor-Aronovitch K, Pinhas-Hamiel O, Landau H. Treatment of congenital hyperinsulinism with lanreotide acetate (somatuline autogel). J Clin Endocrinol Metab. 2011;96:2312–7.  https://doi.org/10.1210/jc.2011-0605.CrossRefPubMedGoogle Scholar
  22. 22.
    Laje P, Halaby L, Adzick NS, Stanley CA. Necrotizing enterocolitis in neonates receiving octreotide for the management of congenital hyperinsulinism. Pediatr Diabetes. 2010;11:142–7.  https://doi.org/10.1111/j.1399-5448.2009.00547.x.CrossRefPubMedGoogle Scholar
  23. 23.
    Huseyin D, et al. Long-term follow-up of children with congenital hyperinsulinism on octreotide therapy. J Clin Endocrinol Metab. 2014;99:3660–7.  https://doi.org/10.1210/jc.2014-1866.CrossRefGoogle Scholar
  24. 24.
    Yorifuji T, et al. Efficacy and safety of long-term, continuous subcutaneous octreotide infusion for patients with different subtypes of KATP-channel hyperinsulinism. Clin Endocrinol. 2013;78:891–7.  https://doi.org/10.1111/cen.12075.CrossRefGoogle Scholar
  25. 25.
    van der Steen I, et al. A multicenter experience with long-acting somatostatin analogues in patients with congenital hyperinsulinism. Horm Res Paediatr. 2018;89:82–9.CrossRefGoogle Scholar
  26. 26.
    Ackermann AM, Palladino AA. Managing congenital hyperinsulinism: improving outcomes with a multidisciplinary approach. Res Rep Endocr Disord. 2015;2015:103–17.  https://doi.org/10.2147/RRED.S56608.CrossRefGoogle Scholar
  27. 27.
    Vajravelu ME, Congdon M, Givler S, Shults J, De Leon DD. Continuous intragastric dextrose: a therapeutic option for refractory hypoglycemia in congenital hyperinsulinism. Horm Res Paediatr. 2018;7:1–7.  https://doi.org/10.1159/000491105.CrossRefGoogle Scholar
  28. 28.
    Herrera A, Vajravelu ME, Givler S, Mitteer L, Avitabile CM, Lord K, De Leon DD. Prevalence of adverse events in children with congenital hyperinsulinism treated with diazoxide. J Clin Endocrinol Metab. 2018;103(12):4365–72.CrossRefGoogle Scholar
  29. 29.
    Mohnike K, et al. Long-term non-surgical therapy of severe persistent congenital hyperinsulinism with glucagon. Horm Res. 2008;70:59–64.  https://doi.org/10.1159/000129680.CrossRefPubMedGoogle Scholar

Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  • Amanda M. Ackermann
    • 1
  • Diva D. De León-Crutchlow
    • 1
    Email author
  1. 1.Division of Endocrinology and Diabetes, Department of PediatricsPerelman School of Medicine at the University of Pennsylvania and The Children’s Hospital of PhiladelphiaPhiladelphiaUSA

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