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European Journal of Pediatrics

, Volume 163, Issue 1, pp 38–41 | Cite as

Severe transient hyperinsulinaemic hypoglycaemia: two neonates without predisposing factors and a review of the literature

  • Fabian YapEmail author
  • Wolfgang Högler
  • Amish Vora
  • Robert Halliday
  • Geoffrey Ambler
Original Paper

Abstract

We report on transient hyperinsulinism (HI), presenting as severe congenital HI, in two neonates born without intrauterine growth restriction, maternal diabetes, perinatal asphyxia or Rhesus/platelet isoimmunisation. The neonates developed early (<6 h of life), symptomatic, non-ketotic hypoglycaemia (0–0.66 mmol/l), associated with elevated insulin levels (40–200 mU/l), and required high glucose infusion rates (22–24 mg/kg per min) to maintain normoglycaemia. However, both babies were diazoxide-sensitive and did not require glucose infusions beyond 2 weeks of life. Neither neonate had elevated serum ammonia levels or evidence of a metabolic disorder. Conclusion:transient hyperinsulinism can occur in newborns delivered uneventfully without significant perinatal complications. The unusual sensitivity to medical treatment in these cases of neonatal-onset hyperinsulinaemic hypoglycaemia underscores the importance of careful medical management of severe congenital hyperinsulinism. Careful consideration of the indication and if necessary, timing and extent of pancreatectomy is required, while maintaining euglycaemia to protect the developing brain.

Keywords

Congenital hyperinsulinism Diazoxide Hypoglycaemia Neonates Transient hyperinsulinism 

Abbreviations

AGA

appropriate for gestational age

BGL

blood glucose level

BWS

Beckwith-Wiedemann syndrome

HI

hyperinsulinism

HI/HA

hyperinsulinism/hyperammonaemia

IUGR

intrauterine growth restriction

PHHI

persistent hyperinsulinaemic hypoglycaemia of infancy

SGA

small for gestational age

SUR

sulphonylurea receptor

TNHI

transient neonatal hyperinsulinism

UVC

umbilical venous catheter

References

  1. 1.
    Adams JT, Donn SM (1994) Association of isoimmune thrombocytopenia and neonatal hyperinsulinism/hypoglycemia. Am J Perinatol 11: 374–376PubMedGoogle Scholar
  2. 2.
    Aynsley-Green A, Hussain K, Hall J, Saudubray JM, Nihoul-Fekete C, Lonlay-Debeney P, Brunelle F, Otonkoski T, Thornton P, Lindley KJ (2000) Practical management of hyperinsulinism in infancy. Arch Dis Child Fetal Neonatal Ed 82: F98–F107PubMedGoogle Scholar
  3. 3.
    Bhowmick SK, Lewandowski C (1989) Prolonged hyperinsulinism and hypoglycemia in an asphyxiated, small for gestation infant. Case management and literature review. Clin Pediatr (Phila) 28: 575–578Google Scholar
  4. 4.
    Brown G, Brown R, Hey E (1978) Fetal hyperinsulinism in rhesus isoimmunization. Am J Obstet Gynecol 131: 682–686PubMedGoogle Scholar
  5. 5.
    Clark W, O’Donovan D (2001) Transient hyperinsulinism in an asphyxiated newborn infant with hypoglycemia. Am J Perinatol 18: 175–178PubMedGoogle Scholar
  6. 6.
    Collins JE, Leonard JV (1984) Hyperinsulinism in asphyxiated and small-for-dates infants with hypoglycaemia. Lancet 2: 311–313PubMedGoogle Scholar
  7. 7.
    Collins JE, Leonard JV, Teale D, Marks V, Williams DM, Kennedy CR, Hall MA (1990) Hyperinsulinaemic hypoglycaemia in small for dates babies. Arch Dis Child 65: 1118–1120PubMedGoogle Scholar
  8. 8.
    De Lonlay P, Fournet JC, Touati G, Groos MS, Martin D, Sevin C, Delagne V, Mayaud C, Chigot V, Sempoux C, Brusset MC, Laborde K, Bellane-Chantelot C, Vassault A, Rahier J, Junien C, Brunelle F, Nihoul-Fekete C, Saudubray JM, Robert JJ (2002) Heterogeneity of persistent hyperinsulinaemic hypoglycaemia. A series of 175 cases. Eur J Pediatr 161: 37–48PubMedGoogle Scholar
  9. 9.
    Doyle ME, Egan JM (2003) Pharmacological agents that directly modulate insulin secretion. Pharmacol Rev 55: 105–131PubMedGoogle Scholar
  10. 10.
    Giroux JD, Vernotte E, Gagneur A, Metz C, Collet M, de Parscau L (1997) Transitory hyperinsulinism with hypoglycemia in asphyxia neonatorum. Arch Pediatr 4: 1213–1216PubMedGoogle Scholar
  11. 11.
    Klenka HM, Seager J (1984) Hyperinsulinism in asphyxiated and small-for-dates infants with hypoglycaemia. Lancet 2: 975Google Scholar
  12. 12.
    Lonlay-Debeney P, Poggi-Travert F, Fournet JC, Sempoux C, Vici CD, Brunelle F, Touati G, Rahier J, Junien C, Nihoul-Fekete C, Robert JJ, Saudubray JM (1999) Clinical features of 52 neonates with hyperinsulinism. N Engl J Med 340: 1169–1175PubMedGoogle Scholar
  13. 13.
    Martin FI, Dahlenburg GW, Russell J, Jeffery P (1975) Neontal hypoglycaemia in infants of insulin-dependent diabetic mothers. Arch Dis Child 50: 472–476PubMedGoogle Scholar
  14. 14.
    Meissner T, Mayatepek E (2002) Clinical and genetic heterogeneity in congenital hyperinsulinism. Eur J Pediatr 161: 6-20PubMedGoogle Scholar
  15. 15.
    Meissner T, Wendel U, Burgard P, Schaetzle S, Mayatepek E (2003) Long-term follow-up of 114 patients with congenital hyperinsulinism. Eur J Endocrinol 149: 43–51PubMedGoogle Scholar
  16. 16.
    Munns CF, Batch JA (2001) Hyperinsulinism and Beckwith-Wiedemann syndrome. Arch Dis Child Fetal Neonatal Ed 84: F67–F69PubMedGoogle Scholar
  17. 17.
    Obenshain SS, Adam PA, King KC, Teramo K, Raivio KO, Raiha N, Schwartz R (1970) Human fetal insulin response to sustained maternal hyperglycemia. N Engl J Med 283: 566–570PubMedGoogle Scholar
  18. 18.
    Rahier J, Guiot Y, Sempoux C (2000) Persistent hyperinsulinaemic hypoglycaemia of infancy: a heterogenous syndrome unrelated to nesidioblastosis. Arch Dis Child Fetal Neonatal Ed 82: F108–F112PubMedGoogle Scholar
  19. 19.
    Sann L (1999) Transitory hyperinsulinism with hypoglycemia in asphyxia neonatorum. Arch Pediatr 6: 228PubMedGoogle Scholar
  20. 20.
    Schwitzgebel VM, Gitelman SE (1998) Neonatal hyperinsulinism. Clin Perinatol 25: 1015–1038PubMedGoogle Scholar
  21. 21.
    Stanley CA (2002) Advances in diagnosis and treatment of hyperinsulinism in infants and children. J Clin Endocrinol Metab 87: 4857–4859PubMedGoogle Scholar
  22. 22.
    Stanley CA, Baker L (1999) The causes of neonatal hypoglycemia. N Engl J Med 340: 1200–1201PubMedGoogle Scholar
  23. 23.
    Stanley CA, Lieu YK, Hsu BY, Burlina AB, Greenberg CR, Hopwood NJ, Perlman K, Rich BH, Zammarchi E, Poncz M (1998) Hyperinsulinism and hyperammonemia in infants with regulatory mutations of the glutamate dehydrogenase gene. N Engl J Med 338: 1352–1357Google Scholar
  24. 24.
    Touati G, Poggi-Travert F, Ogier DB, Rahier J, Brunelle F, Nihoul-Fekete C, Czernichow P, Saudubray JM (1998) Long-term treatment of persistent hyperinsulinaemic hypoglycaemia of infancy with diazoxide: a retrospective review of 77 cases and analysis of efficacy-predicting criteria. Eur J Pediatr 157: 628–633PubMedGoogle Scholar
  25. 25.
    Tyrrell VJ, Ambler GR, Yeow WH, Cowell CT, Silink M (2001) Ten years’ experience of persistent hyperinsulinaemic hypoglycaemia of infancy. J Paediatr Child Health 37: 483–488PubMedGoogle Scholar
  26. 26.
    Zaffanello M, Zamboni G, Maffeis C, Antoniazzi F, Tato L (2002) Neonatal hyperinsulinemic hypoglycemia. Two case reports. Minerva Pediatr 54: 325–333PubMedGoogle Scholar

Copyright information

© Springer-Verlag 2003

Authors and Affiliations

  • Fabian Yap
    • 1
    • 3
    Email author
  • Wolfgang Högler
    • 1
    • 4
  • Amish Vora
    • 2
  • Robert Halliday
    • 2
  • Geoffrey Ambler
    • 1
  1. 1.Institute of Endocrinology and DiabetesThe Children’s Hospital at WestmeadWestmead , Sydney Australia
  2. 2.Department of NeonatologyThe Children’s Hospital at WestmeadSydney Australia
  3. 3.Department of PaediatricsKK Women’s and Children’s Hospital Singapore
  4. 4.Department of PaediatricsUniversity of InnsbruckInnsbruck Austria

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