Abstract
The authors report the cases of two patients with neonatal onset of hypoketotic hypoglycemia with hyperinsulinism and a poor response to diazoxide. Pancreatic venous sampling showed a diffuse pancreatic hyperplasia in one patient and a focal lesion in the other. The second patient, diagnosed after a significant delay, suffered severe long-term neurological sequelae, despite having more limited hyperplasia; the brain MRI also showed severe pathological changes in cortex and white matter, predominantly in the parietooccipital region. Early and accurate diagnosis is critical in these patients in whom hypoglycemia is compounded by a lack of the ketone bodies which represent a vital alternative source of energy for the central nervous system.
Similar content being viewed by others
References
Brown RJK, Wallis PG (1963) Hypoglycaemia in the newborn infant. Lancet I: 1278–1282
Brunelle F, Negre V, Barth MO, Fekete CN, Czernichow P, Saudubray JM, Kuntz F, Tach T, Lallemand D (1989) Pancreatic venous sampling in infants and children with primary hyperinsulinism. Pediatr Radiol 19: 100–103
Chaussain JL, Georges P, Gendrel D, Donnadieu M, Job JC (1980) Serum branched chain amino acids in the diagnosis of hyperinsulinism in infancy. J Pediatr 97: 923–926
Cornblath M, Schwartz R (1991) Disorders of carbohydrate metabolism in infancy. Blackwell Boston
Finegold DN, Stanley CA, Baker L (1980) Glycemic response to glucagon during fasting hypoglycemia: an aid in the diagnosis of hyperinsulinism. J Pediatr 96: 257–259
Glaser B (1990) Long term treatment with somatostatin analogue SMS201-995: alternative to pancreatectomy in persistent hyperinsulinaemic hypoglycemia of infancy. Digestion 45: 27–35
Glaser B, Hirsch HJ, Landau H (1993) Persistent hyperinsulinemic hypoglycemia of infancy: long-term octreotide treatment without pancreatectomy. J Pediatr 123: 644–650
Grant DB, Dunger DB, Burns EC (1986) Long-term treatment with diazoxide in childhood hyperinsulinism. Acta Endocrinol 279 [Suppl]: 340–345
Horev Z, Ipp M, Levey P, Daneman D (1991) Familial hyperinsulinism: successful conservative management. J Pediatr 119: 715–720
Koh THHG, Aynsley-Green A, Tarbit M, Eyre JA (1988) Neural dysfunction during hypoglycemia. Arch Dis Child 63: 1353–1358
Labrune P, Bonnefont JP, Nihoule-Fékété C, Nezeloff C, Gepts W, Czernichow P, Rappaport R, Saudubray JM (1989) Evaluation of diagnostic and therapeutic methods in hyperinsulinism in newborn infants and infants. A propos of a retrospective study of 26 cases. Arch Franc Pediatr 46: 167–173
Lyonnet S, Bonnefont JP, Saudubray JM, Nihoule-Fékété C, Brunelle F (1989) Localization of focal lesion permitting partial pancreatectomy in infants (letter). Lancet II: 671
Parker J, Allen D (1991) Hypertrophic cardiomyopathy after prolonged diazoxide therapy for hyperinsulinemic hypoglycemia. J Pediatr 118: 906–909
Roche A, Raisonnier A, Gillon-Savouret MC (1982) Pancreatic venous sampling and arteriography in localizing insulinomas and gastrinomas: procedure and results in 59 cases. Radiology 145: 621–627
Spar JA, Lewine JD, Orrison WW (1994) Neonatal hypoglycemia: CT and MR findings. Am J Neuroradiol 15: 1477–1478
Susa JB, Cowett RM, Oh W, Schwartz R (1979) Suppression of gluconeogenesis and endogenous glucose production by exogenous insulin administration in the newborn lamb. Pediatr Res 13: 594–598
Thornton PS, Alter CA, Levitt Katz LE, Baker L, Stanley CA (1993) Short- and long-term use of octreotide in the treatment of congenital hyperinsulinism. J Pediatr 123: 637–643
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Zammarchi, E., Filippi, L., Fonda, C. et al. Different neurologic outcomes in two patients with neonatal hyperinsulinemic hypoglycemia. Child's Nerv Syst 12, 413–416 (1996). https://doi.org/10.1007/BF00395098
Received:
Issue Date:
DOI: https://doi.org/10.1007/BF00395098