Paradoxical hypoglycaemia associated with diazoxide therapy for hyperinsulinaemic hypoglycaemia

Hormone Research in Paediatrics

Volumn 80, Issue 2, 2013, Pages 129-133

  Ponmani, Caroline ^a   Gannon, Hannah ^a   Hussain, Khalid ^a,b   Senniappan, Senthil ^a,b  

^a GREAT ORMOND STREET HOSPITAL   (United Kingdom)

^b UNIVERSITY COLLEGE LONDON   (United Kingdom)

Author keywords

Diazoxide; Glucose; Hyperinsulinaemic hypoglycaemia; Insulin

Indexed keywords

ANTIDIABETIC AGENT; DIAZOXIDE; DIURETIC AGENT; FUROSEMIDE; GLUCAGON; OCTREOTIDE; SPIRONOLACTONE;

ARTICLE; CASE REPORT; DRUG DOSE INCREASE; DRUG WITHDRAWAL; FEMALE; GENE MUTATION; GLUCOSE BLOOD LEVEL; GLUCOSE INFUSION; HUMAN; HUMAN TISSUE; HYPERINSULINEMIA; HYPERINSULINEMIC HYPOGLYCEMIA; HYPOGLYCEMIA; IN VITRO STUDY; INFANCY; INFANT; INSULIN RELEASE; JAUNDICE; NEWBORN PERIOD; OBSERVATIONAL STUDY; PANCREAS RESECTION; PREGNANCY DIABETES MELLITUS; PRIORITY JOURNAL; SEIZURE;

CONGENITAL HYPERINSULINISM; DIAZOXIDE; FEMALE; GLUCAGON; GLUCOSE; HUMANS; HYPOGLYCEMIA; INFANT; OCTREOTIDE; SULFONYLUREA RECEPTORS;

EID: 84884288207     PISSN: 16632818     EISSN: 16632826     Source Type: Journal    
DOI: 10.1159/000353773     Document Type: Article

References (24)

1. Aynsley-Green A, Hussain K, Hall J, Saudubray JM, Nihoul-Fekete C, De 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.
2. Senniappan S, Shanti B, James C, Hussain K: Hyperinsulinaemic hypoglycaemia: genetic mechanisms, diagnosis and management. J Inherit Metab Dis 2012; 35: 589-601.
3. Rahier J, Guiot Y, Sempoux C: Persistent hyperinsulinaemic hypoglycaemia of infancy: a heterogeneous syndrome unrelated to nesidioblastosis. Arch Dis Child Fetal Neonatal Ed 2000; 82:F108-F112.
4. 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: 1809-1812.
5. Flanagan SE, Clauin S, Bellanne-Chantelot C, de Lonlay P, Harries LW, Gloyn AL, Ellard S: Update of mutations in the genes encoding the pancreatic beta-cell K(ATP) channel subunits Kir6.2 (KCNJ11) and sulfonylurea receptor
6. (ABCC8) in diabetes mellitus and hyperinsulinism. Hum Mutat 2009; 30: 170-180.
7. Bellanne-Chantelot C, Saint-Martin C, Ribeiro MJ, Vaury C, Verkarre V, Arnoux JB, Valayannopoulos V, Gobrecht S, Sempoux C, Rahier J, Fournet JC, Jaubert F, Aigrain Y, Nihoul-Fekete C, de Lonlay P: ABCC8 and KCNJ11 molecular spectrum of 109 patients with diazoxide-unresponsive congenital hyperinsulinism. J Med Genet 2010; 47: 752-759.
8. James C, Kapoor RR, Ismail D, Hussain K: The genetic basis of congenital hyperinsulinism. J Med Genet 2009, vol 46, pp 289-299.
9. Stanescu DE, Hughes N, Kaplan B, Stanley CA, De Leon DD: Novel presentations of congenital hyperinsulinism due to mutations in the MODY genes: HNF1A and HNF4A. J Clin Endocrinol Metab 2012; 97:E2026-E2030.
10. Ashcroft FM, Harrison DE, Ashcroft SJ: Glucose induces closure of single potassium channels in isolated rat pancreatic beta-cells. Nature 1984; 312: 446-448.
11. Akrouh A, Halcomb SE, Nichols CG, Sala-Rabanal M: Molecular biology of K(ATP) channels and implications for health and disease. IUBMB Life 2009; 61: 971-978.
12. Kane C, Shepherd RM, Squires PE, Johnson PR, James RF, Milla PJ, Aynsley-Green A, Lindley KJ, Dunne MJ: Loss of functional KATP channels in pancreatic beta-cells causes persistent hyperinsulinemic hypoglycemia of infancy. Nat Med 1996; 2: 1344-1347.
13. Garrino MG, Plant TD, Henquin JC: Effects of putative activators of K+ channels in mouse pancreatic beta-cells. Br J Pharmacol 1989; 98: 957-965.
14. Pruitt AW, Dayton PG, Patterson JH: Disposition of diazoxide in children. Clin Pharmacol Ther 1973; 14: 73-82.
15. Alexander S, Anazodo A, Hussain K: Interactions of diazoxide with frusemide, spironolactone, and acetylsalicylic acid in a patient with hyperinsulinism of infancy and fallot tetralogy. Eur J Pediatr 2003; 162: 806-807.
16. Henquin JC, Nenquin M, Sempoux C, Guiot Y, Bellanne-Chantelot C, Otonkoski T, de Lonlay P, Nihoul-Fekete C, Rahier J: In vitro insulin secretion by pancreatic tissue from infants with diazoxide-resistant congenital hyperinsulinism deviates from model predictions. J Clin Invest 2011; 121: 3932-3942.
17. Miki T, Nagashima K, Tashiro F, Kotake K, Yoshitomi H, Tamamoto A, Gonoi T, Iwanaga T, Miyazaki J, Seino S: Defective insulin secretion and enhanced insulin action in KATP channel-deficient mice. Proc Natl Acad Sci USA 1998; 95: 10402-10406.
18. Seghers V, Nakazaki M, DeMayo F, Aguilar-Bryan L, Bryan J: Sur1 knockout mice. A model for K(ATP) channel-independent regulation of insulin secretion. J Biol Chem 2000; 275: 9270-9277.
19. Hauge-Evans AC, King AJ, Carmignac D, Richardson CC, Robinson IC, Low MJ, Christie MR, Persaud SJ, Jones PM: Somatostatin secreted by islet delta-cells fulfills multiple roles as a paracrine regulator of islet function. Diabetes 2009; 58: 403-411.
20. Grimmsmann T, Rustenbeck I: Direct effects of diazoxide on mitochondria in pancreatic B-cells and on isolated liver mitochondria. Br J Pharmacol 1998; 123: 781-788.
21. 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.
22. Nenquin M, Szollosi A, Aguilar-Bryan L, Bryan J, Henquin JC: Both triggering and amplifying pathways contribute to fuel-induced insulin secretion in the absence of sulfonylurea receptor-1 in pancreatic beta-cells. J Biol Chem 2004; 279: 32316-32324.
23. Szollosi A, Nenquin M, Aguilar-Bryan L, Bryan J, Henquin JC: Glucose stimulates Ca2+ influx and insulin secretion in 2-week-old beta-cells lacking ATP-sensitive K+ channels. J Biol Chem 2007; 282: 1747-1756.
24. Mourad NI, Nenquin M, Henquin JC: Metabolic amplifying pathway increases both phases of insulin secretion independently of beta-cell actin microfilaments. Am J Physiol Cell Physiol 2010; 299:C389-C398.