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Serum glucagon counterregulatory hormonal response to hypoglycemia is blunted in congenital hyperinsulinism.

Hussain K, Bryan J, Christesen HT, Brusgaard K, Aguilar-Bryan L.

Diabetes. 2005 Oct;54(10):2946-51.PMID: 16186397 [PubMed - indexed for MEDLINE]Related articlesFree article

Suppression of insulin oversecretion by subcutaneous recombinant human insulin-like growth factor I in children with congenital hyperinsulinism due to defective beta-cell sulfonylurea receptor.

Katz LE, Ferry RJ Jr, Stanley CA, Collett-Solberg PF, Baker L, Cohen P.

J Clin Endocrinol Metab. 1999 Sep;84(9):3117-24.PMID: 10487673 [PubMed - indexed for MEDLINE]Related articlesFree article

Dysregulation of insulin secretion in children with congenital hyperinsulinism due to sulfonylurea receptor mutations.

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.

Diabetes. 2001 Feb;50(2):322-8.PMID: 11272143 [PubMed - indexed for MEDLINE]Related articlesFree article

Regulation of glucagon secretion at low glucose concentrations: evidence for adenosine triphosphate-sensitive potassium channel involvement.

Muñoz A, Hu M, Hussain K, Bryan J, Aguilar-Bryan L, Rajan AS.

Endocrinology. 2005 Dec;146(12):5514-21. Epub 2005 Aug 25.PMID: 16123162 [PubMed - indexed for MEDLINE]Related articlesFree article

Protein-sensitive hypoglycemia without leucine sensitivity in hyperinsulinism caused by K(ATP) channel mutations.

Fourtner SH, Stanley CA, Kelly A.

J Pediatr. 2006 Jul;149(1):47-52.PMID: 16860127 [PubMed - indexed for MEDLINE]Related articles

Carriers of an inactivating beta-cell ATP-sensitive K(+) channel mutation have normal glucose tolerance and insulin sensitivity and appropriate insulin secretion.

Huopio H, Vauhkonen I, Komulainen J, Niskanen L, Otonkoski T, Laakso M.

Diabetes Care. 2002 Jan;25(1):101-6.PMID: 11772909 [PubMed - indexed for MEDLINE]Related articlesFree article

Intraislet hyperinsulinemia prevents the glucagon response to hypoglycemia despite an intact autonomic response.

Banarer S, McGregor VP, Cryer PE.

Diabetes. 2002 Apr;51(4):958-65.PMID: 11916913 [PubMed - indexed for MEDLINE]Related articlesFree article

Characterization of genes encoding the pancreatic beta-cell ATP-sensitive K+ channel in persistent hyperinsulinemic hypoglycemia of infancy in Japanese patients.

Someya T, Miki T, Sugihara S, Minagawa M, Yasuda T, Kohno Y, Seino S.

Endocr J. 2000 Dec;47(6):715-22.PMID: 11228046 [PubMed - indexed for MEDLINE]Related articles

ABCC8 (SUR1) and KCNJ11 (KIR6.2) mutations in persistent hyperinsulinemic hypoglycemia of infancy and evaluation of different therapeutic measures.

Darendeliler F, Fournet JC, Baş F, Junien C, Gross MS, Bundak R, Saka N, Günöz H.

J Pediatr Endocrinol Metab. 2002 Jul-Aug;15(7):993-1000.PMID: 12199344 [PubMed - indexed for MEDLINE]Related articles

Engineering a glucose-responsive human insulin-secreting cell line from islets of Langerhans isolated from a patient with persistent hyperinsulinemic hypoglycemia of infancy.

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.

J Biol Chem. 1999 Nov 26;274(48):34059-66.PMID: 10567373 [PubMed - indexed for MEDLINE]Related articlesFree article

Genotype-phenotype correlations in children with congenital hyperinsulinism due to recessive mutations of the adenosine triphosphate-sensitive potassium channel genes.

Henwood MJ, Kelly A, Macmullen C, Bhatia P, Ganguly A, Thornton PS, Stanley CA.

J Clin Endocrinol Metab. 2005 Feb;90(2):789-94. Epub 2004 Nov 23.PMID: 15562009 [PubMed - indexed for MEDLINE]Related articlesFree article

Severe congenital hyperinsulinism caused by a mutation in the Kir6.2 subunit of the adenosine triphosphate-sensitive potassium channel impairing trafficking and function.

Marthinet E, Bloc A, Oka Y, Tanizawa Y, Wehrle-Haller B, Bancila V, Dubuis JM, Philippe J, Schwitzgebel VM.

J Clin Endocrinol Metab. 2005 Sep;90(9):5401-6. Epub 2005 Jul 5.PMID: 15998776 [PubMed - indexed for MEDLINE]Related articlesFree article

Congenital hyperinsulinism associated ABCC8 mutations that cause defective trafficking of ATP-sensitive K+ channels: identification and rescue.

Yan FF, Lin YW, MacMullen C, Ganguly A, Stanley CA, Shyng SL.

Diabetes. 2007 Sep;56(9):2339-48. Epub 2007 Jun 15.PMID: 17575084 [PubMed - indexed for MEDLINE]Related articlesFree article

Effect of physiological hyperinsulinemia on counterregulatory hormone responses during hypoglycemia in humans.

Mellman MJ, Davis MR, Shamoon H.

J Clin Endocrinol Metab. 1992 Nov;75(5):1293-7.PMID: 1430091 [PubMed - indexed for MEDLINE]Related articles

Paternal mutation of the sulfonylurea receptor (SUR1) gene and maternal loss of 11p15 imprinted genes lead to persistent hyperinsulinism in focal adenomatous hyperplasia.

Verkarre V, Fournet JC, de Lonlay P, Gross-Morand MS, Devillers M, Rahier J, Brunelle F, Robert JJ, Nihoul-Fékété C, Saudubray JM, Junien C.

J Clin Invest. 1998 Oct 1;102(7):1286-91.PMID: 9769320 [PubMed - indexed for MEDLINE]Related articlesFree article

[Congenital hyperinsulinism in newborn and infant]

Giurgea I, Ribeiro MJ, Boddaert N, Touati G, Robert JJ, Saudubray JM, Jaubert F, Bellanné-Chantelot C, Brunelle F, Nihoul-Fékété C, de Lonlay P.

Arch Pediatr. 2005 Nov;12(11):1628-35. Epub 2005 Sep 28. Review. French. PMID: 16198094 [PubMed - indexed for MEDLINE]Related articles

Loss of imprinted genes and paternal SUR1 mutations lead to hyperinsulinism in focal adenomatous hyperplasia.

Fournet JC, Verkarre V, De Lonlay P, Rahier J, Brunelle F, Robert JJ, Nihoul-Fékété C, Saudubray JM, Junien C.

Ann Endocrinol (Paris). 1998;59(6):485-91.PMID: 10189991 [PubMed - indexed for MEDLINE]Related articles

Dominantly inherited hyperinsulinism caused by a mutation in the sulfonylurea receptor type 1.

Huopio H, Reimann F, Ashfield R, Komulainen J, Lenko HL, Rahier J, Vauhkonen I, Kere J, Laakso M, Ashcroft F, Otonkoski T.

J Clin Invest. 2000 Oct;106(7):897-906.PMID: 11018078 [PubMed - indexed for MEDLINE]Related articlesFree article

Diabetes and hypoglycaemia in young children and mutations in the Kir6.2 subunit of the potassium channel: therapeutic consequences.

Flechtner I, de Lonlay P, Polak M.

Diabetes Metab. 2006 Dec;32(6):569-80. Review.PMID: 17296510 [PubMed - indexed for MEDLINE]Related articlesFree article

Hyperinsulinism of infancy: the regulated release of insulin by KATP channel-independent pathways.

Straub SG, Cosgrove KE, Ammälä C, Shepherd RM, O'Brien RE, Barnes PD, Kuchinski N, Chapman JC, Schaeppi M, Glaser B, Lindley KJ, Sharp GW, Aynsley-Green A, Dunne MJ.

Diabetes. 2001 Feb;50(2):329-39.PMID: 11272144 [PubMed - indexed for MEDLINE]Related articlesFree article