Normal proinsulin processing despite beta-cell dysfunction in persistent hyperinsulinaemic hypoglycaemia of infancy (nesidioblastosis)

Diabetologia

Volumn 39, Issue 11, 1996, Pages 1338-1344

  Leibowitz, G ^a   Weintrob, N ^b   Pikarsky, A ^a   Josefsberg, Z ^b   Landau, H ^a   Glaser, B ^a   Hales, C N ^c   Cerasi, E ^a,d  

^a HEBREW UNIVERSITY OF JERUSALEM   (Israel)

^b SCHNEIDER CHILDREN S MEDICAL CENTER OF ISRAEL   (Israel)

^c ADDENBROOKE S HOSPITAL   (United Kingdom)

^d HADASSAH HEBREW UNIVERSITY MEDICAL CENTER   (Israel)

Author keywords

Hypoglycaemia; Infancy; Nesidioblastosis; Pancreatectomy; Proinsulin

Indexed keywords

INSULIN; PROINSULIN;

ADOLESCENT; ADULT; ARTICLE; CHILD; CLINICAL ARTICLE; CONTROLLED STUDY; FEMALE; HUMAN; HYPOGLYCEMIA; INSULINOMA; MALE; PANCREAS RESECTION; PRIORITY JOURNAL; PROTEIN PROCESSING;

ADOLESCENT; ADULT; CHILD; CHILD, PRESCHOOL; COHORT STUDIES; FASTING; FEMALE; HUMANS; HYPERINSULINISM; HYPOGLYCEMIA; INSULIN; MALE; PANCREATECTOMY; PANCREATIC DISEASES; PROINSULIN; PROTEIN PRECURSORS; REMISSION, SPONTANEOUS;

EID: 0029856724     PISSN: 0012186X     EISSN: None     Source Type: Journal    
DOI: 10.1007/s001250050580     Document Type: Article

References (28)

1. Kaiser N, Corcos AP, Tur-Sinai A et al. (1990) Regulation of insulin release in persistent hyperinsulinaemic hypoglycaemia of infancy studied in long-term culture of pancreatic tissue. Diabetologia 33: 482-488
2. Thomas PM, Cote GJ, Wohlk N et al. (1995) Mutations in the sulphonylurea receptor gene in familial persistent hyperinsulinemic hypoglycemia of infancy. Science 268: 426-429
3. Nestorowicz A, Wilson BA, Schoor KP et al. (1996) Hyperinsulinism: identification of two common mutations in the sulphonylurea receptor gene in Ashkenazi Jews. Diabetes 45 [Suppl 2]:229 (Abstract)
4. Stanley CA, Baker L (1976) Hyperinsulinism in infants and children: diagnosis and therapy. Adv Pediatr 23: 315-355
5. Glaser B, Hirsch HJ, Landau H (1993) Persistent hyperinsulinemic hypoglycemia of infancy: long term octreotide treatment without pancreatectomy. J Pediatr 123: 644-650
6. Horev Z, Ipp M, Levey P, Daneman D (1991) Familial hyperinsulinism: successful conservative management. J Pediatr 119: 717-720
7. Leibowitz G, Glaser B, Higazi AA, Salameh M, Cerasi E, Landau H (1995) Hyperinsulinemic hypoglycemia of infancy (nesidioblastosis) in clinical remission: high incidence of diabetes mellitus and persistent beta-cell dysfunction at long-term follow-up. J Clin Endocrinol Metab 80: 386-392
8. Gorden P, Hendricks CM, Roth J (1974) Circulating proinsulin-like component in man: increased proportion in hypoinsulinaemic states. Diabetologia 10: 469-474
9. Melani F, Ryan WG, Rubenstein AH, Steiner DF (1970) Proinsulin secretion by pancreatic beta-cell adenoma: proinsulin and C-peptide secretion. New Engl J Med 283: 713-719
10. Aynsley-Green A, Polak JM, Bloom SR, Gough M, Keeling J, Ashcroft SJ (1981) Nesidioblastosis of the pancreas: definition of the syndrome and the management of the severe neonatal hyperinsulinaemic hypoglycaemia. Arch Dis Child 56: 496-508
11. Landau H, Perlman M, Meyer S et al. (1982) Persistent neonatal hypoglycemia due to hyperinsulinemia: medical aspects. Pediatrics 70: 440-446
12. Cole TJ, Freeman JV, Preece MA (1995) Body mass index reference curves for the UK, 1990. Arch Dis Childhood 73: 25-29
13. Sobey WJ, Beer SF, Carrington CA et al. (1989) Sensitive and specific two-site immunoradiometric assays for human insulin, proinsulin, 65-66 split and 32-33 split proinsulins. Biochem J 260: 535-541
14. Ostrega D, Polonsky K, Nagi D et al. (1995) Measurement of proinsulin and intermediates. Validation of immunoassay methods by high-performance liquid chromatography. Diabetes 437: 437-440
15. Seaquist ER, Kahn SE, Clark PM, Hales CN, Porte DJ, Robertson RP (1996) Hyperproinsulinemia is associated with increased beta cell demand following hemipancreatectomy in humans. J Clin Invest 97: 455-460
16. Haffner SM, Mykkanen L, Valdez RA et al. (1994) Disproportionately increased proinsulin levels are associated with the insulin resistance syndrome. J Clin Endocrinol Metabol 79: 1806-1810
17. Gadot M, Ariav Y, Cerasi E, Kaiser N, Gross DJ (1995) Hyperproinsulinemia in the diabetic Psammomys obesus is a result of increased secretory demand on the B-cell. Endocrinology 136: 4218-4223
18. Roder ME, Vaag A, Hartling SG, Dinesen B, Lanng S, Beck-Nielsen H, Binder C (1995) Proinsulin immunoreactivity in identical twins discordant for noninsulin-dependent diabetes mellitus. J Clin Endocrinol Metabol 80: 2359-2363
19. Davies MJ, Metcalfe J, Day JL, Grenfell A, Hales CN, Gray IP (1994) Improved beta cell function, with reduction in secretion of intact and 32/33 split proinsulin, after dietary intervention in subjects with type 2 diabetes mellitus. Diabet Med 11:71-78
20. Glaser B, Leibovich G, Nesher R, Hartling S, Binder C, Cerasi E (1988) Improved beta-cell function after intensive insulin treatment in severe non-insulin dependent diabetes. Acta Endocrinol 118: 365-373
21. Yoshioka N, Kuzuya T, Matsuda A, Taniguchi M, Iwamoto Y (1988) Serum proinsulin levels at fasting and after oral glucose load in patients with type 2 (non-insulin-dependent) diabetes mellitus. Diabetologia 31: 355-360
22. Haffner SM, Bowsher RR, Mykkanen Let al. (1994) Proinsulin and specific insulin concentration in high- and low-risk populations for NIDDM. Diabetes 43: 1490-1493
23. Davies MJ, Rayman G, Gray IP, Day JL, Hales CN (1993) Insulin deficiency and increased plasma concentration of intact and 32/33 split proinsulin in subjects with impaired glucose tolerance. Diahet Med 10: 313-320
24. Haffner SM, Stern MP, Miettinen H, Gingerich R, Bowsher RR (1995) Higher proinsulin and specific insulin are both associated with a parental history of diabetes in non-diabetic Mexican-American subjects. Diabetes 44: 1156-1160
25. Swinn RA, Wareham NJ, Gregory R et al. (1995) Excessive secretion of insulin precursors characterizes and predicts gestational diabetes. Diabetes 44: 911-915
26. Rhodes CJ, Alarcon C (1994) What beta-cell defect could lead to hyperproinsulinemia in NIDDM? Diabetes 43: 511-517
27. Davies MJ, Metcalfe J, Day JL, Gray IP, Hales CN (1993) Insulin deficiency rather than hyperinsulinaemia in newly diagnosed type 2 diabetes mellitus. Diabet Med 10: 305-312
28. Nagi DK, Hendra TJ, Ryle AJ et al. (1990) The relationships of concentrations of insulin, intact proinsulin and 32-33 split proinsulin with cardiovascular risk factors in type 2 (non-insulin-dependent) diabetic subjects. Diabetologia 33: 532-537