Practical management of hyperinsulinism in infancy

Archives of Disease in Childhood: Fetal and Neonatal Edition

Volumn 82, Issue 2, 2000, Pages

  Aynsley Green, A ^a,b   Lindley, K J ^a,b   Hussain, K ^a,b   Hall, J ^c   Saudubray, J M ^d   Nihoul Fekete C ^d   De Lonlay Debeney, P ^d   Brunelle, F ^d   Otonkoski, T ^e   Thornton, P ^f  

^a GREAT ORMOND STREET HOSPITAL   (United Kingdom)

^b UNIVERSITY COLLEGE LONDON   (United Kingdom)

^c UNIVERSITY OF SHEFFIELD   (United Kingdom)

^d HÔPITAL NECKER ENFANTS MALADES   (France)

^e UNIVERSITY OF HELSINKI   (Finland)

^f CHILDREN S UNIVERSITY HOSPITAL   (Ireland)

Author keywords

Cation channels; Hyperinsulinism; Hypoglycaemia; Neurological damage; Treatment

Indexed keywords

CATION CHANNEL; CHLOROTHIAZIDE; DIAZOXIDE; GLUCAGON; NIFEDIPINE; OCTREOTIDE;

ARTICLE; CLINICAL FEATURE; DRUG INDICATION; GENOTYPE; HUMAN; HYPERINSULINISM; HYPOGLYCEMIA; INFANT; MEDICAL SPECIALIST; NEUROLOGIC DISEASE; PANCREAS; PHENOTYPE; PRACTICE GUIDELINE; PRIORITY JOURNAL;

EID: 0034067782     PISSN: 13592998     EISSN: 14682052     Source Type: Journal    
DOI: 10.1136/fn.82.2.f98     Document Type: Article

References (55)

1. 1 Soltesz G, Aynsley-Grecn A. Hyperinsulinism in infancy and childhood. Ergeb Inn Med Kinderheilkd 1984;51:115-202.
2. 2 Aynsley-Green A, Soltesz G, Jenkins PA. Hyperinsulinaemic hypoglycaemia in infancy and childhood-a practical approach to diagnosis and treatment based on experience of 18 cases. Acta Paediatr Hung 1984;25:319-32.
3. 3 Meissner T, Brune W, Mayatepak E. Persistent hyperinsulinaemic hypoglycaemic of infancy: therapy, clinical outcome and mutational analysis. Eur J Pediatr 1997;156:754-7.
4. 4 Milner RDG. Nesidoblastosis unravelled. Arch Dis Child 1996;74:369-72.
5. atp channels in B-cells causes persistent hyperinsulinaemic hypoglycaemia on infancy. Nat Med 1996;2:1344-7.
6. 6 Aynsley-Green A, Dunne MJ, James RFL, Lindley KJ. Ions and genes in persistent hyperinsulinaemic hypoglycaemia in infancy: a commentary on the implications for tailoring treatment to disease pathogenesis. J Pediatr Endocrinol Metab 1998;11:121-9.
7. 7 Kane C, Lindley KJ, Johnstone PRV, et al. Therapy for persistent hyperinsulinaemic hypoglycaemia of infancy. J Clin Invest 1997;100:1888-93.
8. 8 Dunne MJ, Peterson OH. Potassium selective ion channels in insulin secreting cells: physiology, pharmacology and their role in stimulus secretion coupling. Biochem Biophys Acta 1991;1071:67-82.
9. 9 Bruninig GJ. Recent advances in hyperinsulinism and the pathogenesis of diabetes mellitus. Curr Opin Pediatr 1990;2:758-65.
10. 10 Otonkoski T, Ammala C, Huopio H, et al. A point mutation inactivating the sulphonylurea receptor causes the severe form of persistent hyperinsulinaemic hypoglycaemia of infancy in Finland. Diabetes 1999;48:408-15.
11. 11 Mathew PM, Young JM, Abu YK, et al. Persistent neonatal hyperinsulinism. Clin Pediatr (Phila) 1988;27:148-51.
12. 12 Aynsley-Green A. Hyperinsulinism in infancy and chidhood from genotype to clinical management. In: S O'Rahilly, D Dunger, eds. Genetic insights into paediatric endocrinology and metabolism. Bristol: Bioscientifica [In press.]
13. 13 Collins JE, Leonard JVL. Hyperinsulinism in asphyxiated and small for dates infants with hypoglycaemia. Lancet, 1984;ii:311-13.
14. 14 Brown G, Brown R, Hey E. Foetal hyperinsulinism in rhesus isoimmunisation. Am J Obstet Gynecol 1978;131:682-6.
15. 15 Lucas A, Adrian TE, Bloom SR, Aynsley-Green A. Tatrogenic hyperinsulinism at birth. Lancet 80;i:144-5.
16. 16 Moncrieff MM, Lacey KA, Malleson PN. Management of prolonged hypoglycaemia in Beckwith syndrome. Postgrad Med 1977;53:159-61.
17. 17 Lindley KJ, Dunne MJ, Kane C, et al. Ionic control of the B-cell function in nesidioblastosis, a possible role for calcium channel blockade. Arch Dis Child 1996;74:373-8.
18. 18 Aynsley-Green A, Pollak JM, Keeling J, Gough MH, Baum JD. Averted neonatal death due to pancreatic nesidioblastosis [letter]. Lancet 1978;i:550-1.
19. 19 Koh THH, Eyre JA, Aynsley-Green A. Neonatal hypoglycaemia - the controversy regarding definition. Arch Dis Child 1988;63:1386-9.
20. 20 Koh THHG, Eyre JA, Aynsley-Green A. Neural dysfunction during hypoglycaemia. Arch Dis Child 1988;63:1353-8.
21. 21 Cornblath M, Schwanz R, Aynsley-Green A, Lloyd JK. Hypoglycaemia in infancy: the need for a rational definition. Pediatrics 1990;85:834-7.
22. 22 Shepherd RM, Cosgrove KE, O'Brien RE, et al. Hyperinsulinism in infancy: towards an understanding of unregulated insulin release. Arch Dis Child Fetal Neonatal Ed 2000;82:F87-97.
23. 23 Hawdon JM, Aynsley-Green A, Alberti KGMM, Ward Platt MP. The role of pancreatic insulin secretion in neonatal glucoregulation. 1. Healthy term and preterm infants. Arch Dis Child 1993;68:274-9.
24. 24 Hawdon JM, Aynsley-Green A, Ward Platt MP. The role of pancreatic insulin secretion in neonatal glucoregulation. 2. Infants with disordered glucose homeostasis. Arch Dis Child 1993;68:280-5.
25. 25 Aynsley-Green A, Jenkins PA, Tronier B, Heding LG. Plasma proinsulin and C-peptide concentrations in children with hyperinsulinaemic hypoglycaemia. Acta Paediatr Scand 1984;73:359-63.
26. 26 Finegold DN, Stanley CA, Baker L. Glycaemic response to glucagon during fasting hypoglycaemia: an aid in the diagnosis of hyperinsulinism. J Pediatr 1980;96:257.
27. 27 Levitt-Katz LE, Satin-Smith M, Collett-Solberg P, et al. Insulin like growth factor binding protein-1 levels in the diagnosis of hypoglycaemia caused by hyperinsulinism. J Pediatr 1997;131:193-9.
28. 28 Stanley CA, Lieu YK, Hsu BY, et al. Hyperinsulinism and hyperammonaemia in infants with regulatory mutations of the glutamate dehydrogenase gene. N Engl J Med 1998;338:1352-7.
29. 29 Sener A, Malaisse-Lagae F, Malassie WJ. Stimulation of pancreatic islet metabolism and insulin release by non-metabolizable amino acid. Proc Natl Acad Sci USA 1981;78:5460-4.
30. 30 Morris AAM, Thekekara A, Wilks Z, Clayton P, Leonard JVL, Aynsley-Green A. Evaluation of fasts for investigating hypoglycaemia or suspected metabolic disease. Arch Dis Child 1996;75:115-19.
31. + channels. Endocr Rev 1999;20:101-35.
32. 32 Thornton PS, Martha S, Smith S, et al. Familial hyperinsulinism with apparent autosomal dominant inheritance: clinical and genetic differences from the autosomal recessive variant. J Pediatr 1998;132:9-14.
33. 33 Glaser B, Thornton PS, Herold K, Stanley CA. Clinical and molecular heterogeneity of familial hyperinsulinism. J Pediatr 1998;133:801.
34. 34 Delonlay P, Fournet JC, Rashier J, et al. Somatic deletion of the imprinted 11p15.1 region in sporadic persistent hyperinsulinaemic hypoglycaemia of infancy is specific of focal adenomatous hyperplasia and endorses partial pancreatectomy. J Clin Invest 1997;100:802-7.
35. 35 Ryan F, Devaney D, Joyce C, et al. Hyperinsulinism: the molecular aetiology of focal disease. Arch Dis Child 1998;79:445-7.
36. 36 Glaser B, Kesavan P, Heyman M, et al. Familial hyperinsulinism caused by activating glucokinase mutation. N Engl J Med 1998;338:226-30.
37. 37 Aynsley-Green A, Wiendling AM, Soltesz G, Jenkins PA. Transient lactic acidaemia and hyperalaninaemia associated with neonatal hyperinsulinaemic hypoglycaemia: effect of dichloroacetate. Eur J Pediatr 1983;141:114-17.
38. 38 Cosgrove KE, Shepherd RM, Hashmi MN, et al. The role of calcium ions in determining insulin hypersecretion in patients with PHHI [abstract]. Horm Res 1999;51(suppl 2):29.
39. 39 Glaser B, Thornton P, Otonkoski T, Junien C. Genetics of neonatal hyperinsulinism. Arch Dis Child Fetal Neonatal Ed 2000;82:F79-86.
40. 40 Glaser B, Furth J, Stanley CA, et al. Intragenic single nucleotide polymorphism haplorype analysis of SUR I mutations in familial hyperinsulinism. Hum Mutat 1999;14: 23-9.
41. 41 DeLonlay-Debeney P, Poggi-Trovert F, Fournet JC, et al. Clinical features of 52 neonates with hyperinsulinism. N Engl J Med 1999;340:1169-75.
42. 42 Abernethy LJ, Davidson DC, Lamont GL, Shepherd RM, Dunne MJ. Intra-arterial calcium stimulation test in the investigation of hyperinsulinaemic hypoglycaemia. Arch Dis Child 1998;78:359-63.
43. 43 Nihoul-Fekete C, Delonlay-Debenay P, Brunelle F, Rahier J, Saudubray JM. Partial pancreatectomy as a curative treatment in neonatal persistent hyperinsulinaemic hypoglycaemia of infancy. In: Herausgegeben von, Wolfgang Rabl, K Mohnike, eds. Nesidoblastose, von der molekularen, Pathophysiologie zur Therapie. Heidelberg: Johann Ambrious Barth 1998:64-9.
44. 44 Touati G, Poggi-Travaer P, Ogier de Baulney 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.
45. 45 Aynsley-Green A, Barnes ND, Adrian TE, et al. Effect of somatostatin infusion on intermediary metabolism and entero-insular hormone release in infants with hyperinsulinaemic hypoglycaemia. Acta Paediatr Scand 1981;70:889-95.
46. 46 Glaser B, Hirsch HJ, Landau H. Persistent hyperinsulinaemic hypoglycaemia of infancy: long term treatment without pancreatectomy. J Pediatr 1993;123:644-50.
47. 47 Rahier J, Falt K, Muntefering H, Becker K, Gepts W, Falkmer S. The basic structural lesion in persistent neonatal hypoglycaemia with hyperinsulinism: deficiency of pancreatic D cells or hyperactivity of B-cells? Diabetelogia 1984;26:282-9.
48. 48 Landau H, Glaser B. Conservative treatment and long term follow-up of persistent hyperinsulinism of infancy (HI). Second international conference on ATP sensitive potassium channels and disease, St Charles, USA, 1998. Houston: Giovanni Lorenzini Medical Foundation:33-8.
49. 49 Leibowitz G, Glaser B, Higazi AA, Salamah M, Cesari E, Landau H. Hyperinsulinaemic hypoglycaemia of infancy (nesidioblastosis) in clinical remission: high incidence of diabetes mellitus and persistent B-cell dysfunction at long term follow up. J Clin Endocrinol Metab 1995;80:386-92.
50. 50 Labrune P, Lechevallie S, Rault M, Odievre M. Diabetes mellitus 14 years after subtotal pancreatectomy for neonatal hyperinsulinism. J Pediatr Surg 1990;25:1246-7.
51. 51 Greene SA, Aynsley-Green A, Soltesz G, Baum JD. Management of secondary diabetes mellitus after pancreatectomy in infancy. Arch Dis Child 1984;59:356-9.
52. 52 Lindley KJ, Knafelz D, St Louis D, Dunne MJ, Aynsley-Green A, Milla PJ. Surface electrogastrographic evidence of forgut dysmotility in persistent hyperinsulinaemic hypoglycaemia of infancy (PHHI). J Physiol (Lond) 1997;499:108p.
53. 53 Aynsley-Green A, Dunne MJ, James RF, Lindley KJ, Docherty K. Persistent hyperinsulinaemic hypoglycaemia of infancy - an enigma only now being unravelled. In: Herausgegeben von, Wolfgang Rabl, Klaus Mohnike, eds. Nesidioblastose, von der molekularen, Palhophysiologie zur therapie. Heidelberg: Johann Ambrosius Banh, 1998:57-65.
54. 54 Gross-Tsur V, Shalev RS, Wertman-Elad R, Landau H, Amir N. Neurobehavioural profile of children with persistent hyperinsulinaemic hypoglycaemia of infancy. Dev Neuropsychol 1994;10:153-63.
55. 55 Macfarlane WM, Chapman JC, Shepherd RM, et al. Engineering a glucose-responsive human insulin-secreting cell line from islets of Langerhans from a patient with persistent hyperinsulinaemic hypoglycaemia of infancy (PHHI). J Biol Chem 1999;274:34059-66.