Long-term non-surgical therapy of severe persistent congenital hyperinsulinism with glucagon

Hormone Research

Volumn 70, Issue 1, 2008, Pages 59-64

  Mohnike, K ^a   Blankenstein, O ^b   Pfuetzner, A ^c   Potzsch S ^a   Schober, E ^d   Steiner, S ^c   Hardy, O T ^e   Grimberg, A ^e   Van Waarde, W M ^f  

^a OTTO VON GUERICKE UNIVERSITY   (Germany)

^b CHARITÉ UNIVERSITÄTSMEDIZIN BERLIN   (Germany)

^c Pharmaceutical Discovery Corp   (United States)

^d UNIVERSITY OF VIENNA   (Austria)

^e UNIVERSITY OF PENNSYLVANIA   (United States)

^f UNIVERSITY MEDICAL CENTER GRONINGEN   (Netherlands)

Author keywords

Congenital hyperinsulinism; Continuous subcutaneous glucagon infusion; Diazoxide resistant congenital hyperinsulinism; Hypoglycemic episodes; Octreotide and subcutaneous glucagon infusion; Severe persistent congenital hyperinsulinism

Indexed keywords

GLUCAGON; OCTREOTIDE;

ARTICLE; CENTRAL VENOUS CATHETER; CLINICAL ARTICLE; CONTROLLED STUDY; DIABETES MELLITUS; DRUG EFFICACY; DRUG SAFETY; FEMALE; GLUCAGONOMA; GLYCOGENOLYSIS; HUMAN; HYPOGLYCEMIA; LONG TERM CARE; MALE; NEWBORN; PANCREAS RESECTION; PERSISTENT HYPERINSULINEMIC HYPOGLYCEMIA OF INFANCY; PRIORITY JOURNAL; RISK FACTOR; TREATMENT OUTCOME;

BLOOD GLUCOSE; BRAIN DISEASES, METABOLIC; FEMALE; GASTROINTESTINAL AGENTS; GLUCAGON; GLYCOGEN; HUMANS; HYPOGLYCEMIA; INFANT, NEWBORN; MALE; OCTREOTIDE; PANCREATECTOMY; PERSISTENT HYPERINSULINEMIA HYPOGLYCEMIA OF INFANCY; RETROSPECTIVE STUDIES;

EID: 46349099790     PISSN: 03010163     EISSN: None     Source Type: Journal    
DOI: 10.1159/000129680     Document Type: Article

References (40)

1. Cresto JC, Abdenur JP, Bergada I, Martino R: Long-term follow up of persistent hyperinsulinaemic hypoglycaemia of infancy. Arch Dis Child 1998;79:440-444.
2. Dunne MJ, Cosgrove KE, Shepherd RM, Aynsley-Green A, Lindley KJ: Hyperinsulinism in infancy: from basic science to clinical disease. Physiol Rev 2004;84:239-275.
3. Izumi T, Takeshige H, Arai T, Sugama M, Mizushima M, Fukuyama Y, Mabuchi G: Prospective study of nesidioblastosis in newborns and infants: hypoglycemic seizures, epileptogenesis and the significance of the C-peptide suppression test in pancreatectomy. Acta Paediatr Jpn 1997;39:10-17.
4. Koh TH, Eyre JA, Aynsley-Green A: Neonatal hypoglycaemia - the controversy regarding definition. Arch Dis Child 1988;63:1386-1388.
5. Menni F, de Lonlay P, Sevin C, Touati G, Peigne C, Barbier V, Nihoul-Fekete C, Saudubray JM, Robert JJ: Neurologic outcomes of 90 neonates and infants with persistent hyperinsulinemic hypoglycemia. Pediatrics 2001;107:476-479.
6. Murakami Y, Yamashita Y, Matsuishi T, Utsunomiya H, Okudera T, Hashimoto T: Cranial MRI of neurologically impaired children suffering from neonatal hypoglycaemia. Pediatr Radiol 1999;29:23-27.
7. Touati G, Poggi-Travert F, Ogier dB, Rahier J, Brunelle F, Nihoul-Fekete C, Czernichow P, Saudubray JM: 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;157:628-633.
8. Daneman D, Ehrlich RM: The enigma of persistent hyperinsulinemic hypoglycemia of infancy. J Pediatr 1993;123:573-575.
9. Kane C, Lindley KJ, Johnson PR, James RF, Milla PJ, Aynsley-Green A, Dunne MJ: Therapy for persistent hyperinsulinemic hypoglycemia of infancy. Understanding the responsiveness of β cells to diazoxide and somatostatin. J Clin Invest 1997;100:1888-1893.
10. Lonlay-Debeney P, Poggi-Travert F, Fournet JC, Sempoux C, Vici CD, Brunelle F, Touati G, Rahier J, Junien C, Nihoul-Fekete C, Robert JJ, Saudubray JM: Clinical features of 52 neonates with hyperinsulinism. N Engl J Med 1999;340:1169-1175.
11. Mohnike K, Blankenstein O, Christesen HT, De Lonlay J, Hussain K, Koopmans KP, Minn H, Mohnike W, Mutair A, Otonkoski T, Rahier J, Ribeiro M, Schoenle E, Fekete CN: Proposal for a standardized protocol for 18 F-DOPA-PET (PET/CT) in congenital hyperinsulinism. Horm Res 2006;66:40-42.
12. Aynsley-Green A, Hussain K, Hall J, Saudubray JM, Nihoul-Fekete C, Lonlay-Debeney P, Brunelle F, Otonkoski T, Thornton P, Lindley KJ: Practical management of hyperinsulinism in infancy. Arch Dis Child Fetal Neonatal Ed 2000;82:F98-F107.
13. Bougneres PF, Landier F, Garnier P, Job JC, Chaussain JL: Treatment of insulin excess by continuous subcutaneous infusion of somatostatin and glucagon in an infant. J Pediatr 1985;106:792-794.
14. Glaser B, Hirsch HJ, Landau H: Persistent hyperinsulinemic hypoglycemia of infancy: long-term octreotide treatment without pancreatectomy. J Pediatr 1993;123:644-650.
15. Lindley K, Knafelz D, St Louis D, Dunne M, Aynsley-Green A, Milla PJ: Surface electrogastrographic evidence of foregut dysmotility in persistent hyperinsulinaemic hypoglycaemia of infancy (abstract). J Physiol 1997;499:108.
16. Glaser B, Landau H, Permutt MA: Neonatal hyperinsulinism. Trends Endocrinol Metab 1999;10:55-61.
17. Stanley CA, Baker L: Hyperinsulinism in infants and children: diagnosis and therapy. Adv Pediatr 1976;23:315-355.
18. 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;123:637-643.
19. De Lonlay P, Fournet JC, Rahier J, Gross-Morand MS, Poggi-Travert F, Foussier V, Bonnefont JP, Brusset MC, Brunelle F, Robert JJ, Nihoul-Fekete C, Saudubray JM, Junien C: 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;100:802-807.
20. Eichmann D, Hufnagel M, Quick P, Santer R: Treatment of hyperinsulinaemic hypoglycaemia with nifedipine. Eur J Pediatr 1999;158:204-206.
21. Cederblad F, Ewald U, Gustafsson J: Effect of glucagon on glucose production, lipolysis, and gluconeogenesis in familial hyperinsulinism. Horm Res 1998;50:94-98.
22. Finegold DN, Stanley CA, Baker L: Glycemic response to glucagon during fasting hypoglycemia: an aid in the diagnosis of hyperinsulinism. J Pediatr 1980;96:257-259.
23. Rose SR, Chrousos G, Cornblath M, Sidbury J: Management of postoperative nesidioblastosis with zinc protamine glucagon and oral starch. J Pediatr 1986;108:97-100.
24. Hussain K, Bryan J, Christesen HT, Brusgaard K, Aguilar-Bryan L: Serum glucagon counterregulatory hormonal response to hypoglycemia is blunted in congenital hyperinsulinism. Diabetes 2005;54:2946-2951.
25. Wald M, Lawrenz K, Luckner D, Seimann R, Mohnike K, Schober E: Glucagon therapy as a possible cause of erythema necrolyticum migrans in two neonates with persistent hyperinsulinaemic hypoglycaemia. Eur J Pediatr 2002;161:600-603.
26. Grimberg A, Ferry RJ Jr, Kelly A, Koo-McCoy S, Polonsky K, Glaser B, Permutt MA, Aguilar-Bryan L, Stafford D, Thornton PS, Baker L, Stanley CA: Dysregulation of insulin secretion in children with congenital hyperinsulinism due to sulfonylurea receptor mutations. Diabetes 2001;50:322-328.
27. Haymond MW: Hypoglycemia in infants and children. Endocrinol Metab Clin North Am 1989;18:211-252.
28. Macfarlane WM, Chapman JC, Shepherd RM, Hashmi MN, Kamimura N, Cosgrove KE, O'Brien RE, Barnes PD, Hart AW, Docherty HM, Lindley KJ, Aynsley-Green A, James RF, Docherty K, Dunne MJ: Engineering a glucose-responsive human insulin-secreting cell line from islets of Langerhans isolated from a patient with persistent hyperinsulinemic hypoglycemia of infancy. J Biol Chem 1999;274:34059-34066.
29. Glaser B, Thornton P, Otonkoski T, Junien C: Genetics of neonatal hyperinsulinism. Arch Dis Child Fetal Neonatal Ed 2000;82:F79-F86.
30. Magge SN, Shyng SL, MacMullen C, Steinkrauss L, Ganguly A, Katz LE, Stanley CA: Familial leucine-sensitive hypoglycemia of infancy due to a dominant mutation of the β-cell sulfonylurea receptor. J Clin Endocrinol Metab 2004;89:4450-4456.
31. De Leon DD, Stanley CA: Mechanisms of disease: advances in diagnosis and treatment of hyperinsulinism in neonates. Nat Clin Pract Endocrinol Metab 2007;3:57-68.
32. Katz LE, Ferry RJ Jr, Stanley CA, Collett-Solberg PF, Baker L, Cohen P: Suppression of insulin oversecretion by subcutaneous recombinant human insulin-like growth factor I in children with congenital hyperinsulinism due to defective β-cell sulfonylurea receptor. J Clin Endocrinol Metab 1999;84:3117-3124.
33. Shepherd RM, Cosgrove KE, O'Brien RE, Barnes PD, Ammala C, Dunne MJ: Hyperinsulinism of infancy: towards an understanding of unregulated insulin release. European Network for Research into Hyperinsulinism in Infancy. Arch Dis Child Fetal Neonatal Ed 2000;82:F87-F97.
34. Tauber MT, Harris AG, Rochiccioli P: Clinical use of the long acting somatostatin analogue octreotide in pediatrics. Eur J Pediatr 1994;153:304-310.
35. Alexopoulou O, Abrams P, Verhelst J, Poppe K, Velkeniers B, Abs R, Maiter D: Efficacy and tolerability of lanreotide Autogel therapy in acromegalic patients previously treated with octreotide LAR. Eur J Endocrinol 2004;151:317-324.
36. Aynsley-Green A: Nesidioblastosis of the pancreas in infancy. Dev Med Child Neurol 1981;23:372-379.
37. Kuhl C, Hvorslev V, Tygstrup I, Lindkaer JS: Elevated serum proinsulin in β-cell nesidioblastosis. Report of a case in a newborn. Scand J Gastroenterol Suppl 1979;53:49-52.
38. Pfutzner A, Mann AE, Steiner SS: Technosphere/insulin - a new approach for effective delivery of human insulin via the pulmonary route. Diabetes Technol Ther 2002;4:589-594.
39. Pfutzner A, Flacke F, Pohl R, Linkie D, Engelbach M, Woods R, Forst T, Beyer J, Steiner SS: Pilot study with Technosphere/PTH - a new approach for effective pulmonary delivery of parathyroid hormone (1-34). Horm Metab Res 2003;35:319-323.
40. Pfutzner A, Forst T: Pulmonary insulin delivery by means of the Technosphere drug carrier mechanism. Expert Opin Drug Deliv 2005;2:1097-1106.