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Management of Diabetes and Pancreatic Insufficiency After Pancreatectomy

  • Jefferson N. BrownellEmail author
  • Heather McKnight-Menci
  • Asim Maqbool
  • Paul S. Thornton
Chapter
Part of the Contemporary Endocrinology book series (COE)

Abstract

Following near-total pancreatectomy to treat diffuse hyperinsulinism, disruption of the endocrine and exocrine functions of the pancreas is likely to occur. Initially after surgery, patients may experience persistent hypoglycemia, persistent diabetes mellitus, or transient diabetes followed by hypoglycemia. Insulin is indicated in patients with hyperglycemia in the immediate post-op period to maintain normal glucose levels. For persistent diabetes after resection, dilute insulin is recommended to allow for more precise dosing and to accommodate infant feeding patterns; however, providers and caregivers must be attuned to risks associated with administering nonstandard insulin concentrations. Children who initially continued to have hypoglycemia postoperatively later develop diabetes and need to be screened regularly for development of elevated blood glucose and hemoglobin A1C. In the same manner, exocrine pancreatic insufficiency may have a delayed onset after a subtotal pancreatectomy, and symptoms often arise only after nutritional sequela have occurred. Thus, careful screening for exocrine insufficiency with stool studies and labs should occur at baseline and at regular intervals during growth and development, allowing for prompt treatment with pancreatic enzyme replacement therapy. With attention to both endocrine and exocrine deficits after pancreatectomy, these children can be expected to attain their growth potential and maximize their quality of life.

Keywords

Pancreas Diabetes Hyperglycemia Insulin Exocrine Insufficiency Enzymes 

References

  1. 1.
    Goossens A, Gepts W, Saudubray JM, Bonnefont JP, Nihoul-Fekete, Heitz PU, Klöppel G. Diffuse and focal nesidioblastosis. A clinicopathological study of 24 patients with persistent neonatal hyperinsulinemic hypoglycemia. Am J Surg Pathol. 1989;13(9):766–75.CrossRefGoogle Scholar
  2. 2.
    Ryan F, Devaney D, Joyce C, Nestorowicz A, Permutt MA, Glaser B, Barton DE, Thornton PS. Hyperinsulinism: the molecular aetiology of focal disease. Arch Dis Child. 1998;79(5):445–7.CrossRefGoogle Scholar
  3. 3.
    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(3):270–5.CrossRefGoogle Scholar
  4. 4.
    Snider KE, Becker S, Boyajian L, Shyng S-L, MacMullen C, Hughes N, Ganapathy K, Bhatti T, Stanley CA, Ganguly A. Genotype and phenotype correlations in 417 children with congenital Hyperinsulinism. J Clin Endocrinol Metab. 2013;98:E355–63.CrossRefGoogle Scholar
  5. 5.
    Blomberg BA, Moghbel MC, Saboury B, Stanley CA, Alavi A. The value of radiologic interventions and 18FDOPA PET in diagnosing and localizing focal congenital hyperinsulinism: systematic review and meta-analysis. Mol Imaging Biol. 2013;15:97Y105.Google Scholar
  6. 6.
    Arya VB, Senniappan S, Demirbilek H, Alam S, Flanagan SE, et al. Pancreatic endocrine and exocrine function in children following near-total pancreatectomy for diffuse congenital hyperinsulinism. PLoS One. 2014;9(5):e98054.  https://doi.org/10.1371/journal.pone.0098054.CrossRefPubMedPubMedCentralGoogle Scholar
  7. 7.
    Beltrand J, Caquard M, Arnoux J-B, Laborde K, Velho G, Verkarre G, et al. Glucose metabolism in 105 children and adolescents after pancreatectomy for congenital hyperinsulinism. Diabetes Care. 2012;35:198–203.CrossRefGoogle Scholar
  8. 8.
    Elleri D, Allen JM, Tauschmann M, El-Khairi R, Benitez-Aguirrre P, Acerini CL, Dunger DB, Hovorka R. Feasibility of overnight closed-loop therapy in young children with type 1 diabetes aged 3-6 years: comparison between diluted and standard insulin strength. BMJ Open Diabetes Res Care. 2014;2(1):e000040.  https://doi.org/10.1136/bmjdrc-2014-000040. eCollection 2014. PMID: 25512874 Free PMC Article.CrossRefPubMedPubMedCentralGoogle Scholar
  9. 9.
    Ruan Y, Elleri D, Allen JM, Tauschmann M, Wilinska ME, Dunger DB, Hovorka R. Pharmacokinetics of diluted (U20) insulin aspart compared with standard (U100) in children aged 3-6 years with type 1 diabetes during closed-loop insulin delivery: a randomised clinical trial. Diabetologia. 2015;58(4):687–90.  https://doi.org/10.1007/s00125-014-3483-6. Epub2014 Dec 24. PMID: 25537835 Free PMC Article.CrossRefPubMedGoogle Scholar
  10. 10.
    Mianowska B, Szadkowska A, Fendler W, Mlynarski W. Use of lispro insulin diluted with normal saline to 10 U/ml in an insulin pump: case report.Google Scholar
  11. 11.
    Olinder AL, Kernell A, Smide B. Treatment with CSII in two infants with neonatal diabetes mellitus. Pediatr Diabetes. 2006;7(5):284–8.CrossRefGoogle Scholar
  12. 12.
    Gueant JL, et al. Malabsorption of vitamin B12 in pancreatic insufficiency of the adult and of the child. Pancreas. 1990;5(5):559–67.CrossRefGoogle Scholar
  13. 13.
    Crosby J, et al. Gastrointestinal symptoms before and after total pancreatectomy with islet autotransplantation: the role of pancreatic enzyme dosing and adherence. Pancreas. 2015;44(3):453–8.PubMedPubMedCentralGoogle Scholar
  14. 14.
    Roulet M, Frascarolo P, Rappaz I, Pilet M. Essential fatty acid deficiency in well-nourished young cystic fibrosis patients. Eur J Pediatr. 1997;156(12):952–6.CrossRefGoogle Scholar
  15. 15.
    Lankisch PG. Exocrine pancreatic function tests. Gut. 1982;23:777–98. 2CrossRefGoogle Scholar
  16. 16.
    Niederau C, Grendell JH. Diagnosis of chronic pancreatitis. Gastroenterology. 1985;88:1973–95.CrossRefGoogle Scholar
  17. 17.
    Keller J, et al. Tests of pancreatic exocrine function – clinical significance in pancreatic and non-pancreatic disorders. Best Pract Res Clin Gastroenterol. 2009;23(3):425–39.CrossRefGoogle Scholar
  18. 18.
    Walkowiak J, Cichy WK, Herzig KH. Comparison of fecal elastase-1 determination with the secretin-cholecystokinin test in patients with cystic fibrosis. Scand J Gastroenterol. 1999;34(2):202–7.CrossRefGoogle Scholar
  19. 19.
    Maqbool A, Stallings VA. Optimizing nutritional status in CF. In: Mogayzel PJ, Boyle MP, Peeler DW, Ramsey M (Program Directors). eCystic fibrosis review. eLiterature reviews. Johns Hopkins Medicine. March 2009. www.hopkinscme.net/ofp/eCysticFibrosisReview.
  20. 20.
    Dougherty KA, Schall JI, Stallings VA. Suboptimal vitamin K status despite supplementation in children and young adults with cystic fibrosis. Am J Clin Nutr. 2010;92(3):660–7.CrossRefGoogle Scholar
  21. 21.
    Nakajima K, et al. Pancrelipase: an evidence-based review of its use for treating pancreatic exocrine insufficiency. Core Evid. 2012;7:77–91.CrossRefGoogle Scholar
  22. 22.
    Ng SM, Moore HS. Drug therapies for reducing gastric acidity in people with cystic fibrosis. Cochrane Database Syst Rev. 2016;22(8):CD003424.Google Scholar
  23. 23.
    Otten JL, Hellwig JP, Meyers LD, editors. Dietary reference intakes: the essential guide to nutrient requirements. Washington, DC: Institute of Medicine. National Academy Press; 2006.Google Scholar
  24. 24.
    Ross C, Caballero B, Cousins RJ, Tucker KL, Ziegler TR, editors. Modern nutrition in health and disease. 11th ed. Baltimore: Lippincott Williams & Wilkins; 2014.Google Scholar
  25. 25.
    Rifai N, Horvath AR, Wittwer WR, editors. Tietz textbook of clinical chemistry and molecular diagnostics. 6th ed. St Louis: Elsevier; 2018.Google Scholar
  26. 26.
    Huang SH, Schall JI, Zemel BS, Stallings VA. Vitamin E status in children with cystic fibrosis and pancreatic insufficiency. J Pediatr. 2006;148(4):556–9.CrossRefGoogle Scholar
  27. 27.
    Sinaasappel M, Stern M, Littlewood J, Wolfe S, Steinkamp G, Heijerman HG, et al. Nutrition in patients with cystic fibrosis: a European Consensus. J Cyst Fibros. 2002;1:51e75.CrossRefGoogle Scholar
  28. 28.
    Cystic Fibrosis Foundation, Borowitz D, Robinson KA, Rosenfeld M, Davis SD, Sabadosa KA, et al. Cystic fibrosis foundation evidence-based guidelines for management of infants with cystic fibrosis. J Pediatr. 2009;155:S73e93.Google Scholar
  29. 29.
    Sermet-Gaudelus I, Mayell SJ, Southern KW. European Cystic Fibrosis Society NSWG. Guidelines on the early management of infants diagnosed with cystic fibrosis following newborn screening. J Cyst Fibros. 2010;9:323e9.CrossRefGoogle Scholar

Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  • Jefferson N. Brownell
    • 1
    Email author
  • Heather McKnight-Menci
    • 2
  • Asim Maqbool
    • 1
  • Paul S. Thornton
    • 3
  1. 1.The Center for Pancreatic Disorders, Gastroenterology, Hepatology and NutritionThe Children’s Hospital of PhiladelphiaPhiladelphiaUSA
  2. 2.Division of Endocrinology and DiabetesThe Children’ Hospital of PhiladelphiaPhiladelphiaUSA
  3. 3.Endocrine and Diabetes Program, The Congenital Hyperinsulinism CenterCook Children’s Medical CenterFort WorthUSA

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