Skip to main content
PMC home page
Archives of Disease in Childhood logoLink to Archives of Disease in Childhood
. 1998 Nov;79(5):440–444. doi: 10.1136/adc.79.5.440

Long term follow up of persistent hyperinsulinaemic hypoglycaemia of infancy

J Cresto, J Abdenur, I Bergada, R Martino
PMCID: PMC1717747  PMID: 10193260

Abstract

Twenty six children with hypoglycaemia were diagnosed and followed between 1975 and 1995. Diagnosis was confirmed by a high insulin:glucose ratio, and low free fatty acid and 3-hydroxybutyrate on fasting. All patients were treated with diazoxide at a maximum dose of 20 mg/kg/day. Requirement of a higher dose was considered as a failure of medical treatment and an indication for surgery. Sixteen children responded to diazoxide; 10 failed to respond and underwent pancreatic resection. Six of the latter group started with symptoms in the neonatal period. Eleven of the 26 children have neurological sequelae. Head growth and neurological outcome correlated well. Additionally, non-specific electroencephalogram abnormalities (slow waves) appear to be indicative of subclinical hypoglycaemia during follow up.



Full Text

The Full Text of this article is available as a PDF (107.1 KB).

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Auer R. N., Siesjö B. K. Hypoglycaemia: brain neurochemistry and neuropathology. Baillieres Clin Endocrinol Metab. 1993 Jul;7(3):611–625. doi: 10.1016/s0950-351x(05)80210-1. [DOI] [PubMed] [Google Scholar]
  2. Baker L., Thornton P. S., Stanley C. A. Management of hyperinsulinism in infants. J Pediatr. 1991 Nov;119(5):755–757. doi: 10.1016/s0022-3476(05)80291-4. [DOI] [PubMed] [Google Scholar]
  3. Bergstedt K., Hu B. R., Wieloch T. Initiation of protein synthesis and heat-shock protein-72 expression in the rat brain following severe insulin-induced hypoglycemia. Acta Neuropathol. 1993;86(2):145–153. doi: 10.1007/BF00334881. [DOI] [PubMed] [Google Scholar]
  4. Bier D. M., Leake R. D., Haymond M. W., Arnold K. J., Gruenke L. D., Sperling M. A., Kipnis D. M. Measurement of "true" glucose production rates in infancy and childhood with 6,6-dideuteroglucose. Diabetes. 1977 Nov;26(11):1016–1023. doi: 10.2337/diab.26.11.1016. [DOI] [PubMed] [Google Scholar]
  5. Bjerke H. S., Kelly R. E., Jr, Geffner M. E., Fonkalsrud E. W. Surgical management of islet cell dysmaturation syndrome in young children. Surg Gynecol Obstet. 1990 Oct;171(4):321–325. [PubMed] [Google Scholar]
  6. Bonnefont J. P., Specola N. B., Vassault A., Lombes A., Ogier H., de Klerk J. B., Munnich A., Coude M., Paturneau-Jouas M., Saudubray J. M. The fasting test in paediatrics: application to the diagnosis of pathological hypo- and hyperketotic states. Eur J Pediatr. 1990 Dec;150(2):80–85. doi: 10.1007/BF02072043. [DOI] [PubMed] [Google Scholar]
  7. Buttery J. E., Chamberlain B. R., Pannall P. R. Colorimetric measurement of abnormal levels of 3-hydroxybutyrate in plasma. Clin Chim Acta. 1984 May 30;139(2):167–171. doi: 10.1016/0009-8981(84)90325-5. [DOI] [PubMed] [Google Scholar]
  8. Cresto J. C., Dujovne I. L., Castellani P. I., Mitta E. A., De Majo S. F., Foglia V. G. Insulin radioimmunoassay by the charcoal-dextran technique. Diabetologia. 1972 Aug;8(4):292–295. doi: 10.1007/BF01225574. [DOI] [PubMed] [Google Scholar]
  9. Daneman D., Ehrlich R. M. The enigma of persistent hyperinsulinemic hypoglycemia of infancy. J Pediatr. 1993 Oct;123(4):573–575. doi: 10.1016/s0022-3476(05)80952-7. [DOI] [PubMed] [Google Scholar]
  10. Duncombe W. G. The colorimetric micro-determination of long-chain fatty acids. Biochem J. 1963 Jul;88(1):7–10. doi: 10.1042/bj0880007. [DOI] [PMC free article] [PubMed] [Google Scholar]
  11. Glaser B., Chiu K. C., Liu L., Anker R., Nestorowicz A., Cox N. J., Landau H., Kaiser N., Thornton P. S., Stanley C. A. Recombinant mapping of the familial hyperinsulinism gene to an 0.8 cM region on chromosome 11p15.1 and demonstration of a founder effect in Ashkenazi Jews. Hum Mol Genet. 1995 May;4(5):879–886. doi: 10.1093/hmg/4.5.879. [DOI] [PubMed] [Google Scholar]
  12. Glaser B., Hirsch H. J., Landau H. Persistent hyperinsulinemic hypoglycemia of infancy: long-term octreotide treatment without pancreatectomy. J Pediatr. 1993 Oct;123(4):644–650. doi: 10.1016/s0022-3476(05)80970-9. [DOI] [PubMed] [Google Scholar]
  13. Gottschalk M. E., Geffner M. E., Yasuda P. M., Shields W. D. Reversal of microcephaly and developmental delay after cure of hyperinsulinemic hypoglycemia. J Pediatr. 1990 Sep;117(3):432–434. doi: 10.1016/s0022-3476(05)81088-1. [DOI] [PubMed] [Google Scholar]
  14. Grant D. B., Dunger D. B., Burns E. C. Long-term treatment with diazoxide in childhood hyperinsulinism. Acta Endocrinol Suppl (Copenh) 1986;279:340–345. doi: 10.1530/acta.0.112s340. [DOI] [PubMed] [Google Scholar]
  15. Heding L. G. Radioimmunological determination of human C-peptide in serum. Diabetologia. 1975 Dec;11(6):541–548. doi: 10.1007/BF01222104. [DOI] [PubMed] [Google Scholar]
  16. Heitz P. U., Klöppel G., Häcki W. H., Polak J. M., Pearse A. G. Nesidioblastosis: the pathologic basis of persistent hyperinsulinemic hypoglycemia in infants. Morphologic and quantitative analysis of seven cases based on specific immunostaining and electron microscopy. Diabetes. 1977 Jul;26(7):632–642. doi: 10.2337/diab.26.7.632. [DOI] [PubMed] [Google Scholar]
  17. Koh T. H., Aynsley-Green A., Tarbit M., Eyre J. A. Neural dysfunction during hypoglycaemia. Arch Dis Child. 1988 Nov;63(11):1353–1358. doi: 10.1136/adc.63.11.1353. [DOI] [PMC free article] [PubMed] [Google Scholar]
  18. Labrune P., Bonnefont J. P., Nihoul-Fékété C., Nezeloff C., Gepts W., Czernichow P., Rappaport R., Saudubray J. M. Evaluation des méthodes diagnostiques et thérapeutiques de l'hyperinsulinisme du nouveau-né et du nourrisson. A propos de l'étude rétrospective de 26 enfants. Arch Fr Pediatr. 1989 Mar;46(3):167–173. [PubMed] [Google Scholar]
  19. Landau H., Perlman M., Meyer S., Isacsohn M., Krausz M., Mayan H., Lijovetzky G., Schiller M. Persistent neonatal hypoglycemia due to hyperinsulinism: medical aspects. Pediatrics. 1982 Sep;70(3):440–446. [PubMed] [Google Scholar]
  20. Lewis L. D., Ljunggren B., Ratcheson R. A., Siesjö B. K. Cerebral energy state in insulin-induced hypoglycemia, related to blood glucose and to EEG. J Neurochem. 1974 Oct;23(4):673–679. doi: 10.1111/j.1471-4159.1974.tb04390.x. [DOI] [PubMed] [Google Scholar]
  21. Meissner T., Brune W., Mayatepek E. Persistent hyperinsulinaemic hypoglycaemia of infancy: therapy, clinical outcome and mutational analysis. Eur J Pediatr. 1997 Oct;156(10):754–757. doi: 10.1007/s004310050706. [DOI] [PubMed] [Google Scholar]
  22. Otonkoski T., Andersson S., Simell O. Somatostatin regulation of beta-cell function in the normal human fetuses and in neonates with persistent hyperinsulinemic hypoglycemia. J Clin Endocrinol Metab. 1993 Jan;76(1):184–188. doi: 10.1210/jcem.76.1.8093619. [DOI] [PubMed] [Google Scholar]
  23. Polonsky K. S., Rubenstein A. H. C-peptide as a measure of the secretion and hepatic extraction of insulin. Pitfalls and limitations. Diabetes. 1984 May;33(5):486–494. doi: 10.2337/diab.33.5.486. [DOI] [PubMed] [Google Scholar]
  24. Rindi G., Terenghi G., Westermark G., Westermark P., Moscoso G., Polak J. M. Islet amyloid polypeptide in proliferating pancreatic B cells during development, hyperplasia, and neoplasia in humans and mice. Am J Pathol. 1991 Jun;138(6):1321–1334. [PMC free article] [PubMed] [Google Scholar]
  25. Stanley C. A. Hyperinsulinism in infants and children. Pediatr Clin North Am. 1997 Apr;44(2):363–374. doi: 10.1016/s0031-3955(05)70481-8. [DOI] [PubMed] [Google Scholar]
  26. Thomas C. G., Jr, Underwood L. E., Carney C. N., Dolcourt J. L., Whitt J. J. Neonatal and infantile hypoglycemia due to insulin excess: new aspects of diagnosis and surgical management. Ann Surg. 1977 May;185(5):505–517. doi: 10.1097/00000658-197705000-00002. [DOI] [PMC free article] [PubMed] [Google Scholar]
  27. Thornton P. S., Alter C. A., Katz L. E., Baker L., Stanley C. A. Short- and long-term use of octreotide in the treatment of congenital hyperinsulinism. J Pediatr. 1993 Oct;123(4):637–643. doi: 10.1016/s0022-3476(05)80969-2. [DOI] [PubMed] [Google Scholar]

Articles from Archives of Disease in Childhood are provided here courtesy of BMJ Publishing Group

RESOURCES