Hyperinsulinemic Hypoglycemia malady

Genetics Home Reference: Familial hyperinsulinism is a condition that causes individuals to have abnormally high levels of insulin, which is a hormone that helps control blood sugar levels. People with this condition have frequent episodes of low blood sugar (hypoglycemia). In infants and young children, these episodes are characterized by a lack of energy (lethargy), irritability, and/or difficulty feeding. Repeated episodes of low blood sugar increase the risk for serious complications such as seizures, intellectual disability, breathing difficulties, and coma.¹⁷

MalaCards: Hyperinsulinemic Hypoglycemia, also known as nesidioblastosis, is related to persistent hyperinsulinemic hypoglycemia of infancy and hypoglycemia of infancy. An important gene associated with Hyperinsulinemic Hypoglycemia is KCNJ11 (potassium inwardly-rectifying channel, subfamily J, member 11), and among its related pathways are IRS activation and D-Glutamine and D-glutamate metabolism. The drugs glucose and glucagon and the compounds glipizide and preproinsulin have been mentioned in the context of this disorder. Affiliated tissues include pancreatic islet, and related mouse phenotypes are endocrine/exocrine gland and liver/biliary system.

Disease Ontology: A carbohydrate metabolic disorder that involves low blood glucose resulting from an excess of insulin.⁶

Wikipedia: Hyperinsulinemic hypoglycemia describes the condition and effects of low blood glucose caused by...⁴⁴ more...

Aliases & Descriptions:

hyperinsulinemic hypoglycemia 6 8 nesidioblastosis 6 32 43 persistent hyperinsulinemia hypoglycemia of infancy 6

hyperinsulinemic hypoglycemia, familial, 2 43 islet cell hyperplasia 6 hypoglycemia 43

Diseases related to hyperinsulinemic hypoglycemia by text searches and GeneDecks gene sharing:

(show top 50)    (show all 401)

id	Related Disease	Score	Top Affiliating Genes
1	persistent hyperinsulinemic hypoglycemia of infancy	37.1	GCK, KCNJ11, GLUD1, ABCC8, HADH
2	hypoglycemia of infancy	34.2	GCK, SEL1L, VIM, KCNJ11, KCNJ5, INS
3	hyperinsulinism-hyperammonemia syndrome	31.7	KCNJ5, INS, GLUD1
4	leucine-sensitive hypoglycemia of infancy	31.2	GCK, KCNJ11, GLUD1, ABCC8
5	3-hydroxyacyl-coenzyme a dehydrogenase deficiency	30.3	HADH, HADHB
6	costello syndrome	29.6	INS, IGF2, IGF1
7	insulin autoimmune syndrome	29.4	INS, INSR
8	glycogen storage disease	29.4	GCG, INS, IGF2, IGF1
9	hemangiopericytoma	29.1	VIM, IGF1R, IGF2, IGF1, SST
10	gestational diabetes	28.9	GCK, GCG, KCNJ11, INS, INSR, HNF4A
11	lactic acidosis	28.9	KCNJ5, GLUD1, SLC16A1
12	type 1 diabetes mellitus	28.8	GCK, GCG, INS, INSR, ENO2, IGF2
13	beckwith-wiedemann syndrome	28.7	KCNJ11, INS, IGF1R, IGF2, SST, ABCC8
14	maturity-onset diabetes of the young	28.7	GCK, GCG, KCNJ11, FOXA2, INS, HNF4A
15	gastrointestinal stromal tumor	28.4	PCNA, VIM, ENO2, IGF1R, IGF2, IGF1
16	glucose intolerance	28.2	GCK, GCG, KCNJ11, INS, INSR, IGF1
17	hypoglycemia	27.2	PCNA, GCK, GCG, SEL1L, VIM, KCNJ11
18	histiocytoma	27.2	PCNA, VIM, ENO2, IGF2, IGF1
19	dumping syndrome	26.8	GCG, INS, SST
20	maturity-onset diabetes of the young, type 3	26.8	INS, HNF4A
21	insulinoma	26.7	GCK, GCG, KCNJ11, KCNJ5, INS, INSR
22	pancreatic islet cell tumors	26.6	SST, GAST
23	anorexia nervosa	26.6	GCG, VIM, INS, INSR, IGF2, IGF1
24	laron syndrome	26.5	INS, IGF2, IGF1
25	malignant fibrous histiocytoma	26.4	PCNA, VIM, ENO2, IGF2, IGF1
26	fibrous histiocytoma	26.4	PCNA, VIM, ENO2, IGF2, IGF1
27	insulin resistance	26.4	GCK, GCG, KCNJ11, FOXA2, INS, INSR
28	large cell carcinoma	26.3	ENO2, IGF2, IGF1, KRT19
29	hyperglycemia	26.2	GCK, GCG, KCNJ11, INS, INSR, MEN1
30	hyperprolactinemia	26.0	INS, IGF1, SST
31	neuroendocrine tumor	25.8	GCG, MEN1, ENO2, IGF1R, SST, KRT19
32	diabetic retinopathy	25.7	VIM, INS, INSR, IGF2, IGF1, SST
33	fibrosarcoma	25.7	VIM, INSR, IGF2, IGF1, PDX1
34	carcinoid syndrome	25.6	GCG, MEN1, ENO2, SST, GAST
35	silver-russell syndrome	25.6	IGF1R, IGF2, IGF1
36	acute myocardial infarction	25.6	GCG, KCNJ11, KCNJ5, INS, IGF1, GLUD1
37	hyperinsulinism	25.5	GCK, GCG, KCNJ11, KCNJ5, INS, INSR
38	myotonic dystrophy	25.5	GCG, VIM, INS, INSR, ENO2, IGF1
39	adrenocortical carcinoma	25.4	MEN1, IGF1R, IGF2, HNF4A, SST
40	colon adenocarcinoma	25.4	INS, ENO2, IGF1R, IGF2, KRT19, SLC16A1
41	somatostatinoma	25.3	GCG, MEN1, ENO2, SST, PPY, GAST
42	teratoma	25.3	PCNA, GCG, VIM, ENO2, SST
43	short stature	25.2	INS, IGF1R, IGF2, IGF1, SST
44	growth hormone deficiency	25.1	INS, INSR, IGF1R, IGF2, IGF1, SST
45	myocardial infarction	25.1	GCG, KCNJ11, KCNJ5, INS, INSR, IGF1
46	leiomyosarcoma	24.8	PCNA, VIM, INSR, ENO2, IGF2, IGF1
47	arthritis	24.4	PCNA, VIM, INS, INSR, ENO2, IGF1R
48	craniopharyngioma	24.4	PCNA, VIM, INS, IGF1R, IGF1
49	acromegaly	24.3	GCG, INS, INSR, MEN1, IGF2, IGF1
50	type 2 diabetes mellitus	24.3	GCK, GCG, KCNJ11, KCNJ5, INS, INSR

Approved drugs:

Drug clinical trials:

Inferred drug relations via UMLS/NDF-RT:

^43 28 dextrose, dextrose 100% pwdr [va product], glucagon, glucose, glucose liquid

MalaCards organs/tissues related to hyperinsulinemic hypoglycemia:

²²

MGI Mouse Phenotypes related to hyperinsulinemic hypoglycemia:

²⁵ (show all 13)

id	Description	MGI Source Accession	Score	Top Affiliating Genes
1	endocrine/exocrine gland phenotype	MP:0005379	9.8	SEL1L, KCNJ11, MEN1, GLUD1, ABCC8
2	liver/biliary system phenotype	MP:0005370	9.1	HADHB, PDX1, MEN1, INS, FOXA2, SEL1L
3	reproductive system phenotype	MP:0005389	9.0	TGFBR3, KRT19, NRK, MEN1, FOXA2
4	nervous system phenotype	MP:0003631	8.3	KRT19, HNF4A, IGF2, MEN1, INSR, INS
5	adipose tissue phenotype	MP:0005375	8.0	HADHB, HADH, IGF1, IGF1R, INSR, INS
6	muscle phenotype	MP:0005369	7.2	HADHB, TGFBR3, PDX1, IGF1, IGF2, IGF1R
7	digestive/alimentary phenotype	MP:0005381	7.1	GAST, KRT19, PDX1, SST, IGF2, IGF1R
8	mortality/aging	MP:0010768	7.0	PDX1, KRT19, TGFBR3, HADHB, GAST, NRK
9	growth/size phenotype	MP:0005378	6.8	SST, PPY, PDX1, KRT19, HADH, HADHB
10	cardiovascular system phenotype	MP:0005385	6.4	IGF1R, IGF2, IGF1, KRT19, TGFBR3, HADHB
11	immune system phenotype	MP:0005387	6.1	TGFBR3, PDX1, SST, IGF1, IGF2, IGF1R
12	cellular phenotype	MP:0005384	5.7	IGF2, IGF1, HNF4A, PDX1, KRT19, TGFBR3
13	homeostasis/metabolism phenotype	MP:0005376	5.3	GLUD1, HNF4A, SST, PDX1, ABCC8, HADH

Articles related to hyperinsulinemic hypoglycemia:

(show top 50)    (show all 59)

id	Title	Authors	Year	Affiliating Genes
1	Genome-wide homozygosity analysis reveals HADH mutati ons as a common cause of diazoxide-responsive hyperinsulinemic-hypoglycemia in consanguineous pedigrees. (21252247)	Flanagan S.E.... Ellard S.	2011	HADH
2	ABCC8 mutation allele frequency in the Ashkenazi Jewi sh population and risk of focal hyperinsulinemic hypoglycemia. (21716120)	Glaser B.... Abeliovich D.	2011	ABCC8
3	Diazoxide-responsive hyperinsulinemic hypoglycemia ca used by HNF4A gene mutations. (20164212)	Flanagan S.E.... Ellard S.	2010	GCK, GLUD1, ABCC8
4	Hyperinsulinemic hypoglycemia evolving to gestational diabetes and diabetes mellitus in a family carrying the inactivating ABCC8 E15 06K mutation. (20042013)	Vieira T.C.... MoisAcs R.S.	2010	ABCC8
5	Advances in the diagnosis and management of hyperinsulinemic hypoglycemia. (19165222)	Kapoor R.R.... Hussain K.	2009	ABCC8, KCNJ11
6	3-Hydroxyacyl-coenzyme A dehydrogenase deficiency and hyperinsulinemic hypoglycemia: characterization of a novel mutation and severe dietary protein sensitivity. (19417036)	Kapoor R.R.... Hussain K.	2009	HADHB
7	Identification of a diffuse form of hyperinsulinemic hypoglycemia by 18-fluoro-L-3,4 dihydroxyphenylalanine positron emission tomography/CT in a patient carrying a novel mutation of the HADH gene. (19318379)	Di Candia S.... Mora S.	2009	INS, HADH
8	Hyperinsulinemic hypoglycemia with nesidioblastosis: histologic features and growth factor expression. (18931647)	Rumilla K.M.... Lloyd R.V.	2009	IGF1R, IGF2, TGFBR3
9	Hyperinsulinemic hypoglycemia in adults. Case reports and a short review (18713705)	KovA!cs E.... Pfliegler G.	2008	INS
10	Insulinomatosis: A Multicentric Insulinoma Disease that Frequently Causes Early Recurrent Hyperinsulinemic Hypoglycemia. (19011561)	Anlauf M.... Kloppel G.	2008	MEN1
11	Persistent hyperinsulinemic hypoglycemia and maturity-onset diabetes of the young due to heterozygous HNF4A mutations. (18268044)	Kapoor R.R.... Hussain K.	2008	HNF4A
12	Glucose intolerance and diabetes are observed in the long-term follow-up of nonpancreatectomized patients with persistent hyperinsulinemic hypoglycemia of infancy due to mutations in the ABCC8 gene. (18339976)	Gussinyer M.... Carrascosa A.	2008	ABCC8
13	Non-hyperinsulinemic hypoglycemia in a patient with a gastrointestinal stromal tumor. (16490691)	Singh R.... Van Doorn J.	2006	IGF2
14	Hyperinsulinemic hypoglycemia due to adult nesidioblastosis in insulin-dependent diabetes. (17131493)	Raffel A.... Knoefel W.T.	2006	INS
15	Hyperinsulinemic hypoglycemia after gastric bypass surgery is not accompanied by islet hyperplasia or increased beta-cell turnover. (16801578)	Meier J.J.... Butler P.C.	2006	INS
16	Growth restriction and exendin 4 promote endocrine expression in cultured islet cells derived from patients with persistent hyperinsulinemic hypoglycemia of infancy (PHHI). (16353670)	Webb M.... James R.F.	2005	PDX1
17	Hyperinsulinemic hypoglycemia precipitated by weight loss. (16108243)	Prelipcean M.S.... Bell D.S.	2005	INS
18	Congenital disorder of glycosylation Id presenting with hyperinsulinemic hypoglycemia and islet cell hyperplasia. (15840742)	Sun L.... Freeze H.H.	2005	ALG3
19	Costello syndrome and hyperinsulinemic hypoglycemia. (16278907)	Alexander S.... Hussain K.	2005	INS
20	Hyperinsulinemic hypoglycemia in Beckwith-Wiedemann syndrome due to defects in the function of pancreatic beta-cell adenosine triphosphate-sensitive potassium channels. (15811927)	Hussain K.... Dunne M.J.	2005	ABCC8
21	A novel syndrome of autosomal-dominant hyperinsulinemic hypoglycemia linked to a mutation in the human insulin receptor gene. (15161766)	Hoejlund K.... Bech-Nielsen H.	2004	INSR
22	Severe persistent hyperinsulinemic hypoglycemia due to a de novo glucokinase mutation. (15277402)	Cuesta-Munoz A.L.... Laakso M.	2004	GCK
23	Familial hyperinsulinemic hypoglycemia caused by a defect in the SCHAD enzyme of mitochondrial fatty acid oxidation. (14693719)	Molven A.... Sovik O.	2004	GLUD1, INS, HADH
24	Identification of a novel polymorphism in the fibronectin type II domain of the SEL1L gene and possible relation to the persistent hyperinsulinemic hypoglycemia of infancy. (15450414)	Saltini G.... Biunno I.	2004	SEL1L
25	Hyperinsulinemic hypoglycemia of infancy: the challenge continues. (15133749)	Dekelbab B.H.... Sperling M.A.	2004	GCK, GLUD1
26	Persistent hyperinsulinemic hypoglycemia of the neon ate (15615477)	MaglajliA8 S.... VujoviA8 D.	2004	INS
27	The focal form of persistent hyperinsulinemic hypoglycemia of infancy: morphological and molecular studies show structural and functional differences with insulinoma. (12606521)	Sempoux C.... Rahier J.	2003	IGF2, ABCC8
28	Mutations in the sulfonylurea receptor gene in relation to the long-term outcome of persistent hyperinsulinemic hypoglycemia of infancy. (11912517)	Taguchi T.... Ono J.	2002	ABCC8
29	ABCC8 (SUR1) and KCNJ11 (KIR6.2) mutations in persistent hyperinsulinemic hypoglycemia of infancy and evaluation of different therapeutic measures. (12199344)	Darendeliler F.... Gunoz H.	2002	ABCC8, KCNJ11, KCNJ5
30	Facial appearance in persistent hyperinsulinemic hypoglycemia. (12210338)	de Lonlay P.... Saudubray J.M.	2002	GLUD1, INS
31	Tissue-specific deletion of Foxa2 in pancreatic beta cells results in hyperinsulinemic hypoglycemia. (11445544)	Sund N.J.... Kaestner K.H.	2001	FOXA2, INS
32	Effects of oral glucose on systemic glucose metabolism during hyperinsulinemic hypoglycemia in normal man. (11145123)	Poulsen P.L.... ller N.	2000	GCG
33	Characterization of genes encoding the pancreatic beta-cell ATP-sensitive K+ channel in persistent hyperinsulinemic hypoglycemia of infancy in Japanese patients. (11228046)	Someya T.... Seino S.	2000	ABCC8, KCNJ11
34	Genetic analysis of Japanese patients with persistent hyperinsulinemic hypoglycemia of infancy: nucleotide-binding fold-2 mutation impairs cooperative binding of adenine nucleotides to sulfonylurea receptor 1. (10615958)	Tanizawa Y.... Oka Y.	2000	ABCC8, KCNJ11
35	Engineering a glucose-responsive human insulin-secreting cell line from islets of Langerhans isolated from a patient with persistent hyperinsulinemic hypoglycemia of infancy. (10567373)	Macfarlane W.M.... Dunne M.J.	1999	KCNJ11, PDX1
36	A point mutation inactivating the sulfonylurea receptor causes the severe form of persistent hyperinsulinemic hypoglycemia of infancy in Finland. (10334322)	Otonkoski T.... Thomas P.M.	1999	ABCC8, KCNJ11
37	Noninsulinoma pancreatogenous hypoglycemia: a novel syndrome of hyperinsulinemic hypoglycemia in adults independent of mutations in Kir6.2 and SUR1 genes. (10323384)	Service F.J.... Lloyd R.V.	1999	ABCC8, KCNJ11
38	Hyperinsulinemic hypoglycemia of infancy. Recent insights into ATP-sensitive potassium channels, sulfonylurea receptors, molecular mechanisms, and treatment. (10609115)	Sperling M.A.... Menon R.K.	1999	ABCC8, KCNJ5
39	Functional analyses of novel mutations in the sulfonylurea receptor 1 associated with persistent hyperinsulinemic hypoglycemia of infancy. (9648840)	Shyng S.-L.... Nichols C.G.	1998	ABCC8, KCNJ11, KCNJ5
40	Somatic deletion of the imprinted 11p15 region in sporadic persistent hyperinsulinemic hypoglycemia of infancy is specific of focal adenomatous hyperplasia and endorses partial pancreatectomy. (9259578)	de Lonlay P.... Junien C.	1997	INS
41	Long-term diazoxide treatment in persistent hyperinsulinemic hypoglycemia of infancy: a patient report. (9364348)	Darendeliler F.... GA1nAPz H.	1997	SST
42	Therapy for persistent hyperinsulinemic hypoglycemia of infancy. Understanding the responsiveness of beta cells to diazoxide and som atostatin. (9312191)	Kane C.... Dunne M.J.	1997	SST, INS
43	An autosomal dominant form of familial persistent hyperinsulinemic hypoglycemia of infancy, not linked to the sulfonylurea receptor locus. (9100595)	Kukuvitis A.... Polychronakos C.	1997	ABCC8
44	The molecular basis for familial persistent hyperinsulinemic hypoglycemia of infancy. (8834059)	Thomas P.M.... Gagel R.F.	1996	ABCC8, INS
45	Mutation of the pancreatic islet inward rectifier Kir6.2 also leads to familial persistent hyperinsulinemic hypoglycemia of infancy. (8923010)	Thomas P.... Lightner E.	1996	ABCC8, KCNJ11, KCNJ5
46	Inactivation of the first nucleotide-binding fold of the sulfonylurea receptor, and familial persistent hyperinsulinemic hypoglycemia of infancy. (8751851)	Thomas P.M.... Cote G.J.	1996	ABCC8
47	Nesidioblastosis causing reversal of insulin-dependent diabetes and development of hyperinsulinemic hypoglycemia. (8721942)	Bell D.S.... Dunlap N.E.	1995	INS
48	Mutations in the sulfonylurea receptor gene in familial persistent hyperinsulinemic hypoglycemia of infancy. (7716548)	Thomas P.M.... Bryan J.	1995	ABCC8
49	Homozygosity mapping, to chromosome 11p, of the gene for familial persistent hyperinsulinemic hypoglycemia of infancy. (7847376)	Thomas P.M.... Mathew P.M.	1995	ABCC8, KCNJ11
50	Somatostatin regulation of beta-cell function in the normal human fetuses and in neonates with persistent hyperinsulinemic hypoglycemia. (8093619)	Otonkoski T.... Simell O.	1993	SST

Genes related to hyperinsulinemic hypoglycemia according to GeneCards:

(show all 31)

id	Symbol	Description	Score	GeneCards Section Context
1	KCNJ11	potassium inwardly-rectifying channel, subfamily J, member 11	11.1	Summaries, Publications
2	HADH	hydroxyacyl-CoA dehydrogenase	11.1	Publications, Summaries
3	ABCC8	ATP-binding cassette, sub-family C (CFTR/MRP), member 8	11.1	Publications, Summaries
4	GCK	glucokinase (hexokinase 4)	11.1	Publications, Summaries
5	SLC16A1	solute carrier family 16, member 1 (monocarboxylic acid transporter 1)	11.0
6	INSR	insulin receptor	11.0	Publications
7	GLUD1	glutamate dehydrogenase 1	11.0	Publications, Orthologs
8	SEL1L	sel-1 suppressor of lin-12-like (C. elegans)	11.0	Publications
9	KCNJ5	potassium inwardly-rectifying channel, subfamily J, member 5	11.0	Publications
10	PDX1	pancreatic and duodenal homeobox 1	11.0	Publications
11	SST	somatostatin	11.0	Publications
12	ENO2	enolase 2 (gamma, neuronal)	11.0	Publications
13	KRT19	keratin 19	11.0	Publications
14	VIM	vimentin	11.0	Publications
15	IGF2	insulin-like growth factor 2 (somatomedin A)	11.0	Publications
16	INS	insulin	11.0	Publications
17	ALG3	asparagine-linked glycosylation 3, alpha-1,3- mannosyltransferase homolog (S. cerevisiae)	11.0	Publications
18	GLUD2	glutamate dehydrogenase 2	11.0	Orthologs
19	PPY	pancreatic polypeptide	11.0
20	FOXA2	forkhead box A2	11.0	Publications
21	HADHB	hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), beta subunit	11.0	Publications
22	NRK	Nik related kinase	11.0	Disorders
23	MEN1	multiple endocrine neoplasia I	11.0	Publications
24	TGFBR3	transforming growth factor, beta receptor III	11.0	Publications
25	GCG	glucagon	11.0	Publications
26	HNF4A	hepatocyte nuclear factor 4, alpha	11.0	Publications
27	KCNJ12	potassium inwardly-rectifying channel, subfamily J, member 12	11.0
28	PCNA	proliferating cell nuclear antigen	11.0	Publications
29	GAST	gastrin	11.0
30	IGF1R	insulin-like growth factor 1 receptor	11.0	Publications
31	IGF1	insulin-like growth factor 1 (somatomedin C)	11.0	Publications

Pathways related to hyperinsulinemic hypoglycemia according to GeneDecks:

(show all 19)

id	Pathway	Score	Top Affiliating Genes
1	IRS activation38	10.3	INS, INSR
2	D-Glutamine and D-glutamate metabolism20	10.3	GLUD1, GLUD2
3	Inwardly rectifying K+ channels38	10.1	KCNJ11, ABCC8
4	Type II diabetes mellitus20	9.8	ABCC8, PDX1, INSR, INS, KCNJ11, GCK
5	Potassium transporters- outward current10	9.8	ABCC8, KCNJ5, KCNJ12, KCNJ11
6	Aldosterone-regulated sodium reabsorption20	9.6	IGF1, INSR, INS
7	Maturity onset diabetes of the young20	9.6	PDX1, HNF4A, INS, FOXA2, GCK
8	Anti-diabetic Drug Potassium Channel Inhibitors Pathway, Pharmacodynamics34	9.4	GCK, ABCC8, PDX1, HNF4A, INSR, INS
9	Potassium transporters- inward current10	9.4	KCNJ5, KCNJ12, KCNJ11
10	14-3-3 Induced Intracellular Signaling36	9.1	INSR, IGF1R, IGF1
11	Transcription_Receptor-mediated HIF regulation41	9.0	IGF1, IGF1R, INSR, INS
12	Transcription Receptor-mediated HIF regulation10	9.0	IGF1, IGF1R, INSR, INS
13	MODY (Maturity-Onset Diabetes of Young)36	8.8	HNF4A, IGF1R, INSR, INS, FOXA2, GCK
14	Apoptotic Pathways in Synovial Fibroblasts36	8.6	IGF1R, IGF2, IGF1, TGFBR3
15	eIF2 Pathway36	8.5	TGFBR3, IGF1, IGF2, IGF1R, INSR, INS
16	p70S6K Signaling36	8.4	TGFBR3, IGF1, IGF2, IGF1R, INSR, INS
17	ILK Signaling36	8.3	HNF4A, IGF1, IGF2, IGF1R, INSR, VIM
18	MAPK Signaling36	8.1	TGFBR3, HNF4A, IGF1, IGF2, IGF1R, INSR
19	Glucose / Energy Metabolism3	8.0	PDX1, HNF4A, GLUD1, IGF1R, ENO2, INSR

Compounds related to hyperinsulinemic hypoglycemia according to GeneDecks:

(show top 50)    (show all 166)

id	Compound	Score	Top Affiliating Genes
1	glipizide32 9 9	12.0	SST, KCNJ5, ABCC8, INS
2	preproinsulin32	10.0	PDX1, INS, INSR, GCK
3	repaglinide32 42 9 9	12.8	INS, ABCC8, KCNJ5, KCNJ11, GCG
4	pyruvate32	9.7	GCK, GLUD1, HNF4A, KRT19
5	nateglinide32 42 9 9	12.7	SLC16A1, ABCC8, INS, KCNJ5, KCNJ11, GCG
6	glimepiride32 34 9 9	12.7	KCNJ5, GCG, KCNJ11, ABCC8, INSR, INS
7	incretin32	9.7	INS, PDX1, GCG, GCK
8	diazoxide32 42 9 9	12.7	INS, KCNJ5, KCNJ11, GCK, HADH, ABCC8
9	glibenclamide32 34	10.6	ABCC8, INSR, INS, KCNJ5, KCNJ11, GCG
10	2-deoxyglucose32	9.6	IGF1, IGF2, INSR, INS, GCK
11	gnrh32	9.5	IGF1, GCG, VIM, INS
12	streptozotocin32	9.4	SST, PDX1, INSR, INS, GCK, VIM
13	sulfonylurea32	9.4	ABCC8, PDX1, GLUD1, INS, KCNJ11, GCG
14	tolbutamide32 34 9 9	12.4	GCG, KCNJ5, INS, KCNJ11, GCK, SST
15	i-app32	9.3	PDX1, GAST, INS, GCG, GCK
16	metformin32 34 9 9	12.2	IGF1, KCNJ11, GCK, INS, INSR, GCG
17	mecasermin32 9 9	11.1	IGF1, INS, INSR, IGF1R
18	acipimox32	9.1	SST, IGF1, GCG, INS
19	c-peptide32	9.1	PDX1, HNF4A, IGF2, INSR, FOXA2, KCNJ11
20	katp32	9.0	HADH, FOXA2, GCK, GCG, KCNJ11, ABCC8
21	ag 102432	9.0	IGF1R, IGF2, INSR, IGF1
22	aspartate32	9.0	ABCC8, KRT19, VIM, GLUD2, PCNA, KCNJ11
23	arginine32	8.9	HNF4A, GCG, GCK, IGF2, GLUD1, SST
24	leucine32	8.7	ABCC8, ENO2, VIM, KCNJ11, GLUD2, GLUD1
25	glycerol32 9 18 9	11.7	GCK, PCNA, INS, INSR, GLUD1, HNF4A
26	hydrocortisone32 9 9	10.7	SST, IGF1, INSR, INS, GAST, IGF2
27	nicotinamide32 9 9	10.6	GAST, GCG, PDX1, INS, IGF2
28	fatty acid32	8.5	FOXA2, INS, GCG, HNF4A, ABCC8, HADH
29	rapamycin32 42	9.5	IGF1R, GCG, GLUD1, IGF2, SST, INS
30	thyroxine32 18	9.3	PCNA, INS, INSR, GAST, SST, IGF1
31	ibmx32	8.2	INS, GCG, SST, IGF1, ENO2, IGF1R
32	glucose32	8.1	GLUD1, HADHB, GAST, HADH, SLC16A1, KRT19
33	bromodeoxyuridine32	8.0	PDX1, IGF1R, ENO2, VIM, PCNA, GAST
34	acetylcholine32 9 18 9	10.9	GLUD1, ENO2, IGF1R, SST, GAST, KCNJ5
35	butyrate32	7.8	SLC16A1, PCNA, HNF4A, KRT19, IGF2, IGF1R
36	creatinine32	7.7	PCNA, GCK, GCG, ENO2, IGF1R, IGF1
37	octreotide32 42 9 9	10.6	ENO2, GAST, IGF1R, IGF2, IGF1, SST
38	glycogen32 18	8.6	FOXA2, VIM, INS, ENO2, IGF1R, IGF2
39	adenylate32	7.6	IGF1R, SST, GCG, VIM, KCNJ5, ENO2
40	vitamin d32	7.4	VIM, INSR, IGF1R, IGF2, GAST, IGF1
41	dexamethasone32 42 34 9 9	11.3	PCNA, GAST, SST, HNF4A, GLUD1, IGF2
42	lactate32	7.1	KRT19, SST, GLUD1, VIM, PCNA, GCK
43	glutamate32	6.7	GLUD1, IGF2, IGF1R, INSR, INS, KCNJ5
44	thymidine32 18	7.7	PCNA, MEN1, GAST, ENO2, KRT19, PDX1
45	alanine32	6.6	ENO2, GCG, VIM, INS, GLUD2, PCNA
46	glutamine32	6.5	HNF4A, GLUD1, VIM, IGF2, IGF1R, ENO2
47	vegf32	6.4	PCNA, GAST, VIM, KRT19, PDX1, SST
48	testosterone32 9 18 9	9.4	INSR, IGF1R, IGF2, IGF1, HNF4A, KRT19
49	serine32	6.1	GCK, VIM, INS, INSR, KRT19, MEN1
50	estrogen32	5.9	GAST, INS, KCNJ5, VIM, GCG, INSR

Biological processes related to hyperinsulinemic hypoglycemia according to GeneDecks:

(show all 23)

id	Name	GO ID	Score	Top Affiliating Genes
1	detection of glucose	GO:051594	10.3	PDX1, GCK
2	positive regulation of respiratory burst	GO:060267	10.3	INSR, INS
3	glutamate catabolic process	GO:006538	10.2	GLUD1, GLUD2
4	glutamate biosynthetic process	GO:006537	10.1	GLUD2, GLUD1
5	positive regulation of insulin receptor signaling pathway	GO:046628	9.9	INS, IGF2
6	exocrine pancreas development	GO:031017	9.9	INSR, IGF1, PDX1
7	glucose metabolic process	GO:006006	9.9	PDX1, IGF2, ENO2, INS, KCNJ11
8	positive regulation of glucose import	GO:046326	9.8	IGF1, INSR, INS
9	negative regulation of gluconeogenesis	GO:045721	9.8	INS, GCK
10	positive regulation of glycolysis	GO:045821	9.8	IGF1, INSR, INS, GCK
11	energy reserve metabolic process	GO:006112	9.8	ABCC8, INS, KCNJ11, GCG
12	glucose homeostasis	GO:042593	9.7	PDX1, HNF4A, INSR, INS, GCK
13	positive regulation of mitosis	GO:045840	9.7	IGF1, IGF2, INSR, INS
14	positive regulation of protein kinase B signaling cascade	GO:051897	9.7	INS, INSR, IGF2, IGF1
15	positive regulation of MAPK cascade	GO:043410	9.6	IGF1, IGF2, INSR, INS
16	endocrine pancreas development	GO:031018	9.6	GCK, FOXA2, INS, HNF4A, PDX1
17	positive regulation of glycogen biosynthetic process	GO:045725	9.6	GCK, INS, INSR, IGF2, IGF1
18	response to drug	GO:042493	9.5	HADH, SST, HNF4A, ENO2, KCNJ11
19	regulation of insulin secretion	GO:050796	9.4	ABCC8, HNF4A, INS, KCNJ11, GCG, GCK
20	insulin receptor signaling pathway	GO:008286	9.1	INS, INSR, IGF1R, IGF2
21	positive regulation of DNA replication	GO:045740	9.1	IGF1, IGF1R, INSR, INS
22	small molecule metabolic process	GO:044281	8.8	HADHB, HADH, SLC16A1, ABCC8, GLUD1, ENO2
23	positive regulation of cell proliferation	GO:008284	8.6	PDX1, IGF1, IGF2, IGF1R, INSR, INS

Molecular functions related to hyperinsulinemic hypoglycemia according to GeneDecks:

(show all 8)

id	Name	GO ID	Score	Top Affiliating Genes
1	glutamate dehydrogenase (NAD+) activity	GO:004352	10.1	GLUD1, GLUD2
2	glutamate dehydrogenase [NAD(P)+] activity	GO:004353	10.1	GLUD1, GLUD2
3	ADP binding	GO:043531	9.8	GLUD2, GLUD1, GCK
4	leucine binding	GO:070728	9.8	GLUD1, GLUD2
5	insulin-like growth factor receptor binding	GO:005159	9.5	INS, INSR, IGF2, IGF1
6	insulin binding	GO:043559	9.5	INSR, IGF1R
7	insulin receptor binding	GO:005158	8.8	IGF1, IGF2, IGF1R, INS
8	hormone activity	GO:005179	8.3	GCG, GAST, PPY, SST, IGF1, IGF2