Clinical features of 52 neonates with hyperinsulinism

New England Journal of Medicine

Volumn 340, Issue 15, 1999, Pages 1169-1175

  De Lonlay Debeney, Pascale ^c   Poggi Travert, Florence ^c   Fournet, Jean Christophe ^c   Sempoux, Christine ^a   Vici, Carlo Dionisi ^c   Brunelle, Francis ^c   Touati, Guy ^c   Rahier, Jacques ^a   Junien, Claudine ^c   Nihoul Fékété, Claire ^c   Robert, Jean Jacques ^c   Saudubray, Jean Marie ^b  

^a CLINIQUES UNIVERSITAIRES SAINT LUC   (Belgium)

^b HÔPITAL NECKER ENFANTS MALADES   (France)

^c NONE

Author keywords

[No Author keywords available]

Indexed keywords

DIAZOXIDE; GLUCOCORTICOID; GLUCOSE; GLYCOSYLATED HEMOGLOBIN; INSULIN; OCTREOTIDE;

ARTICLE; CHILD; DIABETES MELLITUS; FEMALE; GLUCOSE BLOOD LEVEL; GLUCOSE TOLERANCE TEST; HUMAN; HYPERGLYCEMIA; HYPERINSULINISM; HYPOGLYCEMIA; INSULIN BLOOD LEVEL; INTRAMUSCULAR DRUG ADMINISTRATION; INTRAVENOUS DRUG ADMINISTRATION; MAJOR CLINICAL STUDY; MALE; NEWBORN; ORAL DRUG ADMINISTRATION; PANCREAS ISLET CELL HYPERPLASIA; PRIORITY JOURNAL; RISK ASSESSMENT; SUBCUTANEOUS DRUG ADMINISTRATION; SYMPTOMATOLOGY; TREATMENT FAILURE;

ATP-BINDING CASSETTE TRANSPORTERS; BLOOD GLUCOSE; GLUCOSE TOLERANCE TEST; HEMOGLOBIN A, GLYCOSYLATED; HUMANS; HYPERINSULINISM; HYPERPLASIA; HYPOGLYCEMIA; INFANT, NEWBORN; INSULIN; ISLETS OF LANGERHANS; MUTATION; PANCREATECTOMY; POTASSIUM CHANNELS; POTASSIUM CHANNELS, INWARDLY RECTIFYING; RECEPTORS, DRUG;

EID: 0033560687     PISSN: 00284793     EISSN: None     Source Type: Journal    
DOI: 10.1056/NEJM199904153401505     Document Type: Article

References (36)

1. Stanley CA. Hyperinsulinism in infants and children. Pediatr Clin North Am 1997;44:363-74.
2. Bruining GJ. Recent advances in hyperinsulinism and the pathogenesis of diabetes mellitus. Curr Opin Pediatr 1990;2:758-65.
3. Thomas CG Jr, Underwood LE, Carney CN, Dolcourt JL, Whitt JJ. Neonatal and infantile hypoglycemia due to insulin excess: new aspects of diagnosis and surgical management. Ann Surg 1977;185:505-17.
4. Touati G, Poggi-Travert F, Ogier de Baulny H, et al. 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 1998;157:628-33.
5. Shilyanski J, Fisher S, Cutz E, Perlman K, Filler RM. Is 95% pancreatectomy the procedure of choice for treatment of persistent hyperinsulinemic hypoglycemia of the neonate? J Pediatr Surg 1997;32:342-6.
6. Hyperinsulinismes de l'enfant: à propos d'une série de 56 cas (1984-1994). In: Poggi-Travert F, Rahier J, Brunelle F, Fékété C, Saudubray JM. Journées Parisiennes de pédiatrie. Paris: Flammarion Médecine-Sciences, 1994:29-42.
7. Thornton PS, Alter CA, Katz LE, Baker L, Stanley CA. Short-and long-term use of octreotide in the treatment of congenital hyperinsulinism. J Pediatr 1993;123:637-43.
8. Spitz L, Bhargava RK, Grant DB, Leonard JV. Surgical treatment of hyperinsulinaemic hypoglycaemia in infancy and childhood. Arch Dis Child 1992;67:201-5.
9. Sempoux C, Guiot Y, Lefevre A, et al. Neonatal hyperinsulinemic hypoglycemia: heterogeneity of the syndrome and keys for differential diagnosis. J Clin Endocrinol Metab 1998;83:1455-61.
10. Nesidioblastosis, In: Solcia E, Capella C, Klöppel G. Tumors of the pancreas. Atlas of tumor pathology. 3rd series. Fascicle 20. Washington, D.C.: Armed Forces Institute of Pathology, 1997:238-43.
11. Goossens A, Gepts W, Saudubray JM, et al. Diffuse and focal nesidioblastosis: a clinicopathological study of 24 patients with persistent neonatal hyperinsulinemic hypoglycemia. Am J Surg Pathol 1989;3:766-75.
12. Goudswaard WB, Houthoff HJ, Koudstaal J, Zwierstra RP. Nesidioblastosis and endocrine hyperplasia of the pancreas: a secondary phenomenon. Hum Pathol 1986;17:46-54.
13. Rahier J, Fält K, Müntefering H, Becker K, Gepts W, Falkmer S. The basic structural lesion of persistent neonatal hypoglycaemia with hyperinsulinism: deficiency of pancreatic D cells or hyperactivity of B cells? Diabetologia 1984;26:282-9.
14. Jaffé R, Hashida Y, Yunis EJ. Pancreatic pathology in hyperinsulinemic hypoglycemia of infancy. Lab Invest 1980;42:356-65.
15. de Lonlay P, Fournet JC, Rahier J, et al. Somatic deletion of the imprinted 11p15 region in sporadic persistent hyperinsulinemic hypoglycemia of infancy is specific of focal adenomatous hyperplasia and endorses partial pancreatectomy. J Clin Invest 1997;100:802-7
16. Verkarre V, Fournet JC, de Lonlay P, et al. Paternal mutation of the sulfonylurea receptor (SUR1) gene and maternal loss of 11p15 imprinted genes lead to persistent hyperinsulinism in focal adenomatous hyperplasia. J Clin Invest 1998;102:1286-91.
17. Thomas PM, Cote GJ, Wohllk N, et al. Mutations in the sulfonylurea receptor gene in familial persistent hyperinsulinemic hypoglycemia of infancy. Science 1995;268:426-9.
18. Nestorowicz A, Wilson BA, Schoor KP, et al. Mutations in the sulfonylurea receptor gene are associated with familial hyperinsulinism in Ashkenazi Jews. Hum Mol Genet 1996;5:1813-22.
19. Thomas P, Ye Y, Lightner E. Mutation of the pancreatic islet inward rectifier Kir6.2 also leads to familial persistent hyperinsulinemic hypoglycemia of infancy. Hum Mol Genet 1996;5:1809-12.
20. Nestorowicz A, Inagaki N, Gonoi T, et al. A nonsense mutation in the inward rectifier potassium channel gene, Kir6.2, is associated with familial hyperinsulinism. Diabetes 1997;46:1743-8.
21. Thornton PS, Sumner AE, Ruchelli ED, Spielman RS, Baker L, Stanley CA. Familial and sporadic hyperinsulinism: histopathologic findings and segregation analysis support a single autosomal recessive disorder. J Pediatr 1991;199:721-4.
22. Glaser B, Kesavan P, Heyman M, et al. Familial hyperinsulinism caused by an activating glucokínase mutatíon. N Engl J Med 1998;338:226-30.
23. Kukuvitis A, Deal C, Arbour L, Polychronakos C. An autosomal dominant form of familial persistent hyperinsulinemic hypoglycemia of infancy, not linked to the sulfonylurea receptor locus. J Clin Endocrinol Metab 1997;82:1192-4.
24. Stanley CA, Lieu YK, Hsu BLY, et al. Hyperinsulinemia and hyper-ammonemia in infants with regulatory mutations of the glutamate dehydrogenase gene. N Engl J Med 1998;338:1352-7.
25. Leibowitz G, Glaser B, Higazi AA, Salameh M, Cerasi E, Landau H. Hyperinsulinemic hypoglycemia of infancy (nesidioblastosis) in clinical remission: high incidence of diabetes mellitus and persistent β-cell dysfunction at long-term follow-up. J Clin Endocrinol Metab 1995;80:386-92.
26. Labrune P, Lechevallier S, Rault M, Odièvre M. Diabetes mellitus 14 years after subtotal pancreatectomy for neonatal hyperinsulinism. J Pediatr Surg 1990;25:1246-7.
27. Lyonnet S, Bonnefont JP, Saudubray JM, Nihoule-Fekete C, Brunelle F. Localisation of focal lesion permitting partial pancreatectomy in infants. Lancet 1989;2:671.
28. Rahier J, Sempoux C, Fournet JC, et al. Partial or near-total pancreatectomy for persistent neonatal hyperinsulinaemic hypoglycaemia: the pathologist's role. Histopathology 1998;32:15-9.
29. Brunelle F, Negre V, Barth MO, et al. Pancreatic venous samplings in infants and children with primary hyperinsulinism. Pediatr Radiol 1989;19: 100-3.
30. Dubois J, Brunelle F, Touati G, et al. Hyperinsulinism in children: diagnostic value of pancreatic venous sampling correlated with clinical, pathological and surgical outcome in 25 cases. Pediatr Radiol 1995;25: 512-6.
31. The Expert Committee on the Diagnosis and Classification of Diabetes Mellitus. Report of the Expert Committee on the Diagnosis and Classification of Diabetes Mellitus. Diabetes Care 1998;21:Suppl 1:S5-S19.
32. Glaser B, Hirsch HJ, Landau H. Persistent hyperinsulinemic hypoglycemia of infancy: long-term octreotide treatment without pancreatectomy. J Pediatr 1993;123:644-50.
33. Horev Z, Ipp M, Levey P, Daneman D. Familial hyperinsulinism: successful conservative management. J Pediatr 1991;119:717-20.
34. Bordi C, Ravazzola M, Pollak A, Lubec G, Orci L. Neonatal islet cell adenoma: a distinct type of islet cell tumor? Diabetes Care 1982;5:122-5.
35. Craver RD, Hill CB. Cure of hypoglycemic hyperinsulinism by enucleation of a focal islet cell adenomatous hyperplasia. J Pediatr Surg 1997; 32:1526-7.
36. Scarlett JA, Mako ME, Rubenstein AH, et al. Factitious hypoglycemia: diagnosis by measurement of serum C-peptide immunoreactivity and insulin-binding antibodies. N Engl J Med 1977;297:1029-32.