MCID: HYP050
MIFTS: 56

Hyperinsulinemic Hypoglycemia malady

Categories: Metabolic diseases, Genetic diseases, Rare diseases, Endocrine diseases

Aliases & Classifications for Hyperinsulinemic Hypoglycemia

Aliases & Descriptions for Hyperinsulinemic Hypoglycemia:

Name: Hyperinsulinemic Hypoglycemia 12 29 14 69
Nesidioblastosis 12 52 42 69
Islet Cell Hyperplasia 12 29
Persistent Hyperinsulinemia Hypoglycemia of Infancy 12

Classifications:



External Ids:

Disease Ontology 12 DOID:13317
ICD10 33 E16.9
MeSH 42 D046768
NCIt 47 C4375
UMLS 69 C0027773

Summaries for Hyperinsulinemic Hypoglycemia

Disease Ontology : 12 A carbohydrate metabolic disorder that involves low blood glucose resulting from an excess of insulin.

MalaCards based summary : Hyperinsulinemic Hypoglycemia, also known as nesidioblastosis, is related to hyperinsulinemic hypoglycemia, familial, 5 and microcephaly, short stature, and impaired glucose metabolism. An important gene associated with Hyperinsulinemic Hypoglycemia is ABCC8 (ATP Binding Cassette Subfamily C Member 8), and among its related pathways/superpathways are AMP-activated Protein Kinase (AMPK) Signaling and Glucose / Energy Metabolism. The drugs Insulin Glargine and Zinc have been mentioned in the context of this disorder. Affiliated tissues include pancreas, brain and pancreatic islet, and related phenotypes are endocrine/exocrine gland and growth/size/body region

Wikipedia : 71 Hyperinsulinemic hypoglycemia describes the condition and effects of low blood glucose caused by... more...

Related Diseases for Hyperinsulinemic Hypoglycemia

Diseases in the Hyperinsulinemic Hypoglycemia family:

Hyperinsulinemic Hypoglycemia, Familial, 7 Hyperinsulinemic Hypoglycemia, Familial, 4
Hyperinsulinemic Hypoglycemia, Familial, 3 Hyperinsulinemic Hypoglycemia, Familial, 1
Hyperinsulinemic Hypoglycemia, Familial, 2 Hyperinsulinemic Hypoglycemia, Familial, 5

Diseases related to Hyperinsulinemic Hypoglycemia via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 103)
id Related Disease Score Top Affiliating Genes
1 hyperinsulinemic hypoglycemia, familial, 5 33.7 INS INSR
2 microcephaly, short stature, and impaired glucose metabolism 31.8 ABCC8 HADH
3 hyperinsulinemic hypoglycemia, familial, 2 12.5
4 hyperinsulinemic hypoglycemia, familial, 7 12.4
5 hyperinsulinemic hypoglycemia, familial, 1 12.4
6 hyperinsulinemic hypoglycemia, familial, 3 12.4
7 hyperinsulinemic hypoglycemia, familial, 4 12.4
8 adult-onset non-insulinoma persistent hyperinsulinemic hypoglycemia 12.0
9 hyperinsulinism-hyperammonemia syndrome 11.9
10 insr-related hyperinsulinism 11.1
11 hypoglycemia 11.0
12 hyperinsulinism due to short chain 3-hydroxylacyl-coa dehydrogenase deficiency 11.0
13 slc16a1-related hyperinsulinism 11.0
14 glud1-related hyperinsulinism 11.0
15 hyperinsulinism due to hnf4a deficiency 11.0
16 hyperinsulinism due to ucp2 deficiency 11.0
17 hyperinsulinism due to hnf1a deficiency 11.0
18 pancreatitis 10.4
19 noonan syndrome 3 10.3 ABCC8 KCNJ11
20 diaphanospondylodysostosis 10.2 ABCC8 GCK
21 ritscher-schinzel syndrome 2 10.2 ABCC8 KCNJ11
22 hypercholesterolemia, familial 10.2 ABCC8 INSR
23 insulinoma 10.2
24 image syndrome 10.2 GLUD1 INS
25 nosophobia 10.1 ABCC8 GCK KCNJ11
26 alopecia-mental retardation syndrome 2 10.1 ABCC8 INS
27 intellectual disability-developmental delay-contractures syndrome 10.1 INS INSR
28 fanconi anemia, complementation group f 10.1 ABCC8 KCNJ11
29 neutral lipid storage disease with myopathy 10.1 IGF2 INS
30 diarrhea 3, secretory sodium, congenital, syndromic 10.1 INS INSR
31 breast fibroadenoma 10.1 IGF2 INS
32 maple syrup urine disease, mild variant 10.1 GCK GLUD1 HADH
33 hemorrhagic fever 10.1 ABCC8 IGF2 KCNJ11
34 autoimmune disease 1 10.1 ABCC8 INS KCNJ11
35 deafness, autosomal recessive 66 10.1 ABCC8 INS KCNJ11
36 chudley-mccullough syndrome 10.0 GLUD1 INS
37 paranasal sinus cancer, adult 10.0 INS SST
38 choline deficiency disease 10.0 INS SST
39 shaken baby syndrome 10.0 INS SST
40 maturity-onset diabetes of the young 10.0
41 brain ependymoma 10.0 INS SST
42 femur bifid with monodactylous ectrodactyly 10.0 GLUD1 IGF2 INSR
43 peripheral degeneration of cornea 10.0 INS SST
44 spinal stenosis 10.0 INS SST
45 leg dermatosis 10.0 INS INSR
46 protein s deficiency 10.0 INS SST
47 blind loop syndrome 10.0 GCK INS INSR
48 photoparoxysmal response 3 10.0 INS SST
49 labyrinthine unilateral reactive loss 9.9 ABCC8 GCK INS KCNJ11
50 obesity 9.9

Graphical network of the top 20 diseases related to Hyperinsulinemic Hypoglycemia:



Diseases related to Hyperinsulinemic Hypoglycemia

Symptoms & Phenotypes for Hyperinsulinemic Hypoglycemia

MGI Mouse Phenotypes related to Hyperinsulinemic Hypoglycemia:

44
id Description MGI Source Accession Score Top Affiliating Genes
1 endocrine/exocrine gland MP:0005379 9.97 ABCC8 GCK GLUD1 HADH IGF2 INS
2 growth/size/body region MP:0005378 9.97 GCK GLUD1 HADH HNF4A IGF2 INS
3 homeostasis/metabolism MP:0005376 9.96 ABCC8 GCK GLUD1 HADH HNF4A IGF2
4 liver/biliary system MP:0005370 9.55 GCK HNF4A IGF2 INS INSR
5 no phenotypic analysis MP:0003012 9.35 ABCC8 IGF2 INS KCNJ11 SST
6 renal/urinary system MP:0005367 9.02 GCK HADH IGF2 INS INSR

Drugs & Therapeutics for Hyperinsulinemic Hypoglycemia

Drugs for Hyperinsulinemic Hypoglycemia (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 119)
id Name Status Phase Clinical Trials Cas Number PubChem Id
1
Insulin Glargine Approved Phase 4 160337-95-1
2
Zinc Approved Phase 4,Early Phase 1 7440-66-6 32051 23994
3
Rosiglitazone Approved, Investigational Phase 4 122320-73-4 77999
4
Acarbose Approved, Investigational Phase 4,Phase 2 56180-94-0 441184
5
Metformin Approved Phase 4 657-24-9 14219 4091
6
Liraglutide Approved Phase 4 204656-20-2
7
Verapamil Approved Phase 4 52-53-9 2520
8 Hypoglycemic Agents Phase 4,Phase 2,Phase 3,Phase 1,Early Phase 1
9 insulin Phase 4,Phase 3,Phase 2,Phase 1,Early Phase 1
10 Insulin, Globin Zinc Phase 4,Phase 3,Phase 2,Phase 1,Early Phase 1
11 Gastrointestinal Agents Phase 4,Phase 2,Early Phase 1
12 glucagon Phase 4,Phase 2,Phase 3,Phase 1,Early Phase 1
13 Glucagon-Like Peptide 1 Phase 4,Phase 2,Phase 3,Phase 1,Early Phase 1
14 Hormone Antagonists Phase 4,Phase 3,Phase 2,Phase 1,Early Phase 1
15 Hormones Phase 4,Phase 3,Phase 2,Phase 1,Early Phase 1
16 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 4,Phase 3,Phase 2,Phase 1,Early Phase 1
17 Incretins Phase 4,Phase 3,Phase 2,Phase 1,Early Phase 1
18 Vasodilator Agents Phase 4,Phase 2,Phase 3
19 Dipeptidyl-Peptidase IV Inhibitors Phase 4,Phase 3
20 HIV Protease Inhibitors Phase 4,Phase 3
21
protease inhibitors Phase 4,Phase 3
22 Anti-Arrhythmia Agents Phase 4,Early Phase 1
23 Cardiac Glycosides Phase 4,Phase 2
24 Glycoside Hydrolase Inhibitors Phase 4,Phase 2
25 gastric inhibitory polypeptide Phase 4
26 Sitagliptin Phosphate Phase 4
27 calcium channel blockers Phase 4
28 Calcium, Dietary Phase 4
29
Diazoxide Approved Phase 2, Phase 3 364-98-7 3019
30
Exenatide Approved, Investigational Phase 2, Phase 3, Early Phase 1 141758-74-9 15991534
31
Saxagliptin Approved Phase 3 361442-04-8 11243969
32 Antihypertensive Agents Phase 2, Phase 3
33
Somatostatin Approved Phase 2,Phase 1 38916-34-6, 51110-01-1 53481605
34
Octreotide Approved, Investigational Phase 2,Phase 1 83150-76-9 383414 6400441
35
Acetylcysteine Approved, Investigational Phase 1, Phase 2 616-91-1 12035
36
Dopamine Approved Phase 2 51-61-6, 62-31-7 681
37
Pancrelipase Approved Phase 2 53608-75-6
38 Neurotransmitter Agents Phase 2,Early Phase 1
39 Antidotes Phase 1, Phase 2
40 Anti-Infective Agents Phase 1, Phase 2
41 Antioxidants Phase 1, Phase 2
42 Antiviral Agents Phase 1, Phase 2
43 Expectorants Phase 1, Phase 2
44 N-monoacetylcystine Phase 1, Phase 2
45 Protective Agents Phase 1, Phase 2
46 Respiratory System Agents Phase 1, Phase 2,Early Phase 1
47 Pharmaceutical Solutions Phase 2
48 Dihydroxyphenylalanine Phase 2
49 Dopamine Agents Phase 2
50 pancreatin Phase 2

Interventional clinical trials:

(show top 50) (show all 53)
id Name Status NCT ID Phase
1 Prevention of Severe Hypoglycemia in Type 1 Diabetes Completed NCT00360984 Phase 4
2 Rosiglitazone and Insulin in T1DM Adolescents Completed NCT00372086 Phase 4
3 Sitagliptin and Glucagon Counterregulation Active, not recruiting NCT02256189 Phase 4
4 Treatment of Hypoglycemia Following Gastric Bypass Surgery Enrolling by invitation NCT02527993 Phase 4
5 Diazoxide In the Management Of Hypoglycemic Neonates Unknown status NCT00994149 Phase 2, Phase 3
6 DPP4inhibitors in Type 1 Diabetes Completed NCT01922817 Phase 3
7 New Imaging Procedure for the Localisation of Insulinoma and Transplanted Islet Cells Recruiting NCT02127541 Phase 2, Phase 3
8 Exenatide and Impaired Hypoglycaemic Awareness in Type 1 Diabetes Recruiting NCT02735031 Phase 2, Phase 3
9 Study to Assess Safety and Efficacy of sc Pasireotide in Patients With Dumping Syndrome Completed NCT01895296 Phase 2
10 A Pilot Study Evaluating Exenatide for the Treatment of Postprandial Hyperinsulinemic Hypoglycemia Post-RYGB Recruiting NCT02685852 Phase 2
11 Efficacy, Tolerability and Pharmacokinetics of Subcutaneous Exendin (9-39) in Patients With Post Bariatric Hypoglycemia Recruiting NCT02771574 Phase 2
12 A Randomized Double Blinded Study to Examine the Use of N-acetyl Cysteine for the Prevention and Treatment of HAAF in Patients With Type 1 Diabetes Recruiting NCT02206152 Phase 1, Phase 2
13 Phase II Safety and Efficacy Study of F-DOPA PET/CT in Children With Hyperinsulinemic Hypoglycemia Active, not recruiting NCT01468454 Phase 2
14 Utility of [F-18] fluoroDOPA for Neonatal Hyperinsulinism Active, not recruiting NCT00674440 Phase 2
15 Pasireotide in Hyperinsulinemic Hypoglycemia Not yet recruiting NCT03053284 Phase 2
16 Efficacy of Small Subcutaneous Glucagon Dose to Treat Hypoglycemia in Adults With Type 1 Diabetes Withdrawn NCT01828125 Phase 2
17 GLP1R-imaging in Hypoglycemia Recruiting NCT03182192 Phase 1
18 Treatment Plan for an Individual Patient With Pasireotide for Hyperinsulinemic Hypoglycemia Recruiting NCT03103009 Phase 1
19 Role of GLP-1 in Hyperinsulinemic Hypoglycemia Post-bariatric Surgery Recruiting NCT02550145 Phase 1
20 Evaluation of Single Ascending Doses of Subcutaneous Exendin 9-39 in Patients With Post-Bariatric Hypoglycemia Active, not recruiting NCT02996812 Phase 1
21 Effect of Cinnamon Extract on Insulin Resistance in Polycystic Ovary Syndrome Terminated NCT00970541 Phase 1
22 68Ga-NOTA-exendin-4 PET/CT for the Localization of Insulinoma and Diagnosis of Nesidioblastosis Unknown status NCT02560376 Early Phase 1
23 Etiology, Assessment and Treatment of Post-gastric Bypass Severe Hypoglycemia Unknown status NCT01865760
24 Hypoglycemia and Autonomic Nervous System Function Unknown status NCT01816893
25 Post-Gastric Bypass Hypoglycemia Completed NCT01933490
26 Metabolic Causes of Thrombosis in Type 2 Diabetes - Question 4 Completed NCT00574340 Early Phase 1
27 Sympathetic Nerve Activity During Hypoglycemia and Exercise Completed NCT00608348
28 The Effects of Hypoglycaemia in People With Type 2 Diabetes Completed NCT02205996
29 Counter-Regulatory Impairment and the Effect of Microvascular Insulin Transfer in Type 1 Diabetes Mellitus Completed NCT00943787
30 Hypoglycemia Associated Autonomic Failure in Type 1 DM, Q2 Completed NCT00592332
31 The Impact of Renin-angiotensin System on Brain Activation During Hypoglycaemia in Healthy Men, a PET Study Completed NCT00264641
32 Mechanisms of Hypoglycemia Associated Autonomic Dysfunction, Q.3 Completed NCT00742521
33 Metabolic Adaptations to Diabetes in African Americans Completed NCT00780195
34 Mechanisms of Hypoglycemia Associated Autonomic Dysfunction Question 2 Completed NCT00574639 Early Phase 1
35 Glimepiride Induced Insulin Secretion Will be Inhibited by Hypoglycemia Completed NCT00608179
36 Metabolic Adaptation to Diabetes Completed NCT00732862
37 Non Invasive Assessment of Liver Glycogen Kinetics and ATP Synthesis in Type1 Diabetics Completed NCT00481598
38 Adrenergic System in Islet Transplantation Recruiting NCT03079921
39 Role of Neural and Hormonal Regulation Factors on Insulin Secretion After Gastric Bypass Surgery Recruiting NCT00992901 Early Phase 1
40 Hypoglycemia Associated Autonomic Failure in Type 1 Diabetes Mellitus Recruiting NCT00607646 Early Phase 1
41 Comparison Between Hypoglycemic Counter Regulatory Responses in Type 1 Diabetics vs Control Subjects Recruiting NCT03034226
42 Brain Ketone Metabolism in Type 1 Diabetes Recruiting NCT02471040
43 Dose Response of Epinephrine Recruiting NCT02692313 Early Phase 1
44 Study of the Use of [18F]-DOPA in Hyperinsulinemic Hypoglycemia Available NCT02533219
45 18F-L-Fluoro-DOPA PET/CT Scan Localization of Focal Pancreatic Lesions in Subjects With Hyperinsulinemic Hypoglycemia Available NCT01916148
46 Compassionate Use of SOM230 for Hyperinsulinemic/Hypoglycemia Available NCT02835131
47 Evaluation of the Effect of Hypoglycemia With PET and a Norepinephrine Transporter Ligand Enrolling by invitation NCT02056249
48 Adverse Effects of Systemic Hypoglycemia Exposure on Endothelial Function in Humans Terminated NCT01660009
49 Tight Glycaemic Control During Cardiac Surgery Terminated NCT01225159
50 The Effects of Antecedent Hypoglycemia or Exercise on the Response of the Brain to Subsequent Hypoglycemia Withdrawn NCT00600730

Search NIH Clinical Center for Hyperinsulinemic Hypoglycemia

Cochrane evidence based reviews: nesidioblastosis

Genetic Tests for Hyperinsulinemic Hypoglycemia

Genetic tests related to Hyperinsulinemic Hypoglycemia:

id Genetic test Affiliating Genes
1 Islet Cell Hyperplasia 29
2 Hyperinsulinemic Hypoglycemia 29

Anatomical Context for Hyperinsulinemic Hypoglycemia

MalaCards organs/tissues related to Hyperinsulinemic Hypoglycemia:

39
Pancreas, Brain, Pancreatic Islet, B Cells, Liver, Spleen

Publications for Hyperinsulinemic Hypoglycemia

Articles related to Hyperinsulinemic Hypoglycemia:

(show top 50) (show all 262)
id Title Authors Year
1
Single incision laparoscopic 90A % pancreatectomy for the treatment of persistent hyperinsulinemic hypoglycemia of infancy. ( 27473008 )
2016
2
Synchronous Nesidioblastosis, Endocrine Microadenoma, and Intraductal Papillary Mucinous Neoplasia in a Man Presenting With Hyperinsulinemic Hypoglycemia. ( 26658039 )
2016
3
Assessment of Nifedipine therapy in Hyperinsulinemic Hypoglycemia due to mutations in the ABCC8 gene. ( 27898257 )
2016
4
Safety and tolerability of diazoxide in Japanese patients with hyperinsulinemic hypoglycemia. ( 26598136 )
2016
5
Hyperinsulinemic Hypoglycemia of Infancy due to Novel HADH Mutation in Two Siblings. ( 27771675 )
2016
6
Current Status of Childhood Hyperinsulinemic Hypoglycemia in Turkey. ( 27181376 )
2016
7
Hyperinsulinemic Hypoglycemia - The Molecular Mechanisms. ( 27065949 )
2016
8
Incidence and Predictive Factors of Postprandial Hyperinsulinemic Hypoglycemia After Roux-en-Y Gastric Bypass: A Five year Longitudinal Study. ( 27560624 )
2016
9
Hyperinsulinemic hypoglycemia associated with insulin antibodies caused by exogenous insulin analog. ( 27933175 )
2016
10
Hyperinsulinemic Hypoglycemia after Bariatric Surgery: Diagnosis and Management Experience from a Spanish Multicenter Registry. ( 26901345 )
2016
11
Postprandial hyperinsulinemic hypoglycemia after Roux-en-Y gastric bypass: an update. ( 27865808 )
2016
12
Conditional Tissue-Specific Foxa2 Ablation in Mouse Pancreas Causes Hyperinsulinemic Hypoglycemia. ( 26882312 )
2016
13
One-Anastomosis Jejunal Interposition with Gastric Remnant Resection (Branco-Zorron Switch) for Severe Recurrent Hyperinsulinemic Hypoglycemia after Gastric Bypass for Morbid Obesity. ( 27738969 )
2016
14
Pathological aspects underlying pancreatogenous hyperinsulinemic hypoglycemia - report of three cases. ( 25826512 )
2015
15
Hyperinsulinemic hypoglycemia syndrome associated with mutations in the human insulin receptor gene: Report of two cases. ( 25753915 )
2015
16
Clinical features and causes of endogenous hyperinsulinemic hypoglycemia in Korea. ( 25922806 )
2015
17
Recognition and management of hyperinsulinemic hypoglycemia after bariatric surgery. ( 26522879 )
2015
18
Persistent hyperinsulinemic hypoglycemia of infancy: a clinical and pathological study of 19 cases in a single institution. ( 26823758 )
2015
19
Screening for Mutations in ABCC8 and KCNJ11 Genes in Saudi Persistent Hyperinsulinemic Hypoglycemia of Infancy (PHHI) Patients. ( 25871929 )
2015
20
Hyperinsulinemic Hypoglycemia. ( 26210630 )
2015
21
Severe Hyperinsulinemic Hypoglycemia in a Neonate: Response to Sirolimus Therapy. ( 26504129 )
2015
22
A Deep Intronic HADH Splicing Mutation (c.636+471G>T) in a Congenital Hyperinsulinemic Hypoglycemia Case: Long Term Clinical Course. ( 26316438 )
2015
23
Minimally invasive surgical interventions in the treatment of primary persistent hyperinsulinemic hypoglycemia of infancy. ( 26331229 )
2015
24
Biomarkers of Insulin for the Diagnosis of Hyperinsulinemic Hypoglycemia in Infants and Children. ( 26490124 )
2015
25
Hyperinsulinemic hypoglycemia: think of hyperinsulinism/hyperammonemia (HI/HA) syndrome caused by mutations in the GLUD1 gene. ( 25781533 )
2015
26
Post-Gastric Bypass Hyperinsulinemic Hypoglycemia: Fructose is a Carbohydrate Which Can Be Safely Consumed. ( 26037514 )
2015
27
Paternal uniparental disomy 11p15.5 in the pancreatic nodule of an infant with Costello syndrome: Shared mechanism for hyperinsulinemic hypoglycemia in neonates with Costello and Beckwith-Wiedemann syndrome and somatic loss of heterozygosity in Costello syndrome driving clonal expansion. ( 26572961 )
2015
28
Experience of Octreotide Therapy for Hyperinsulinemic Hypoglycemia in Neonates Born Small for Gestational Age: A Case Series. ( 26448570 )
2015
29
Clinical and molecular data from 61 Brazilian cases of Congenital Hyperinsulinemic Hypoglycemia. ( 25972930 )
2015
30
Hyperinsulinemic Hypoglycemia in Infancy: Current Concepts in Diagnosis and Management. ( 26713990 )
2015
31
A case with neonatal hyperinsulinemic hypoglycemia: It is a characteristic complication of sotos syndrome. ( 25712828 )
2015
32
Hyperinsulinemic hypoglycemia in a patient with an intragenic NSD1 mutation. ( 26487424 )
2015
33
Effect of Carbohydrate Restriction in Patients with Hyperinsulinemic Hypoglycemia after Roux-en-Y Gastric Bypass. ( 24902654 )
2014
34
Dynamic 18F-FDOPA PET Findings After Carbidopa Premedication in 2 Adult Patients With Insulinoma-Related Hyperinsulinemic Hypoglycemia. ( 25549347 )
2014
35
Endogenous hyperinsulinemic hypoglycemia syndrome: surgical treatment. ( 24491350 )
2014
36
Sirolimus therapy in infants with severe hyperinsulinemic hypoglycemia. ( 24645945 )
2014
37
Inhibition of insulin receptor function by a human, allosteric monoclonal antibody: A potential new approach for the treatment of hyperinsulinemic hypoglycemia. ( 24423625 )
2014
38
Poor pregnancy outcome after octreotide treatment during pregnancy for familial hyperinsulinemic hypoglycemia: a case report. ( 25404386 )
2014
39
Novel ABCC8 (SUR1) gene mutations in Asian Indian children with congenital hyperinsulinemic hypoglycemia. ( 25117148 )
2014
40
Treatment of severe postRYGB hyperinsulinemic hypoglycemia with pasireotide: a comparison with octreotide on insulin, glucagon, and GLP-1. ( 24448101 )
2014
41
Hyperinsulinemic hypoglycemia syndrome in 2 dogs with bartonellosis. ( 24934703 )
2014
42
Sirolimus in severe hyperinsulinemic hypoglycemia. ( 24941187 )
2014
43
Sirolimus in severe hyperinsulinemic hypoglycemia. ( 24941188 )
2014
44
Fetal Macrosomia and Neonatal Hyperinsulinemic Hypoglycemia Associated With Transplacental Transfer of Sulfonylurea in a Mother With KCNJ11-Related Neonatal Diabetes. ( 25231897 )
2014
45
Using miglitol at 30 min before meal is effective in hyperinsulinemic hypoglycemia after a total gastrectomy. ( 25142087 )
2014
46
Postprandial hyperinsulinemic hypoglycemia after gastric bypass surgical treatment. ( 25002326 )
2014
47
Evaluation, Medical Therapy, and Course of Adult Persistent Hyperinsulinemic Hypoglycemia After Roux-en-Y Gastric Bypass Surgery: A Case Series. ( 25100376 )
2014
48
Monogenic hyperinsulinemic hypoglycemia: current insights into the pathogenesis and management. ( 23384201 )
2013
49
Determination of insulin for the diagnosis of hyperinsulinemic hypoglycemia. ( 24275188 )
2013
50
Mutations in the genes encoding the transcription factors hepatocyte nuclear factor 1 alpha and 4 alpha in maturity-onset diabetes of the young and hyperinsulinemic hypoglycemia. ( 23348805 )
2013

Variations for Hyperinsulinemic Hypoglycemia

ClinVar genetic disease variations for Hyperinsulinemic Hypoglycemia:

6
id Gene Variation Type Significance SNP ID Assembly Location
1 KCNJ11 NM_000525.3(KCNJ11): c.440T> C (p.Leu147Pro) single nucleotide variant Pathogenic rs28936678 GRCh37 Chromosome 11, 17409199: 17409199
2 KCNJ11 NM_000525.3(KCNJ11): c.36C> A (p.Tyr12Ter) single nucleotide variant Pathogenic rs104894236 GRCh37 Chromosome 11, 17409603: 17409603
3 KCNJ11 NM_000525.3(KCNJ11): c.-134G> T single nucleotide variant Pathogenic rs387906398 GRCh37 Chromosome 11, 17409772: 17409772
4 KCNJ11 NM_000525.3(KCNJ11): c.761C> T (p.Pro254Leu) single nucleotide variant Pathogenic rs104894237 GRCh37 Chromosome 11, 17408878: 17408878
5 KCNJ11 NM_000525.3(KCNJ11): c.776A> G (p.His259Arg) single nucleotide variant Pathogenic rs104894248 GRCh37 Chromosome 11, 17408863: 17408863
6 KCNJ11 NM_000525.3(KCNJ11): c.902G> A (p.Arg301His) single nucleotide variant Pathogenic rs74339576 GRCh37 Chromosome 11, 17408737: 17408737
7 KCNJ11 KCNJ11, GLY156ARG undetermined variant Pathogenic
8 KCNJ11 NM_000525.3(KCNJ11): c.844G> A (p.Glu282Lys) single nucleotide variant Pathogenic rs267607196 GRCh37 Chromosome 11, 17408795: 17408795
9 KCNJ11 NM_000525.3(KCNJ11): c.881C> T (p.Thr294Met) single nucleotide variant Pathogenic rs780957825 GRCh37 Chromosome 11, 17408758: 17408758

Expression for Hyperinsulinemic Hypoglycemia

Search GEO for disease gene expression data for Hyperinsulinemic Hypoglycemia.

Pathways for Hyperinsulinemic Hypoglycemia

Pathways related to Hyperinsulinemic Hypoglycemia according to GeneCards Suite gene sharing:

(show all 12)
id Super pathways Score Top Affiliating Genes
1
Show member pathways
12.1 HNF4A INS INSR
2 12.09 GLUD1 HNF4A INS INSR
3
Show member pathways
11.97 ABCC8 INS KCNJ11
4
Show member pathways
11.96 ABCC8 GCK INS KCNJ11
5
Show member pathways
11.55 GCK HNF4A INS INSR
6
Show member pathways
11.54 GCK HNF4A INS
7
Show member pathways
11.42 ABCC8 GCK HNF4A INS INSR KCNJ11
8 11.13 INS INSR
9
Show member pathways
11.1 INS INSR
10 11.02 INS INSR
11 10.87 INS INSR
12 10.74 ABCC8 GCK HADH HNF4A INS KCNJ11

GO Terms for Hyperinsulinemic Hypoglycemia

Cellular components related to Hyperinsulinemic Hypoglycemia according to GeneCards Suite gene sharing:

id Name GO ID Score Top Affiliating Genes
1 ATP-sensitive potassium channel complex GO:0008282 8.62 ABCC8 KCNJ11

Biological processes related to Hyperinsulinemic Hypoglycemia according to GeneCards Suite gene sharing:

(show all 22)
id Name GO ID Score Top Affiliating Genes
1 response to drug GO:0042493 9.81 ABCC8 HADH KCNJ11 SST
2 carbohydrate metabolic process GO:0005975 9.71 GCK IGF2 INS INSR
3 positive regulation of MAPK cascade GO:0043410 9.67 IGF2 INS INSR
4 positive regulation of protein kinase B signaling GO:0051897 9.65 IGF2 INS INSR
5 insulin receptor signaling pathway GO:0008286 9.63 IGF2 INS INSR
6 positive regulation of insulin secretion GO:0032024 9.61 GCK GLUD1
7 positive regulation of nitric oxide biosynthetic process GO:0045429 9.6 INS INSR
8 positive regulation of DNA replication GO:0045740 9.59 INS INSR
9 glucose transport GO:0015758 9.58 GCK INS
10 positive regulation of glucose import GO:0046326 9.58 INS INSR
11 activation of protein kinase B activity GO:0032148 9.57 INS INSR
12 cellular glucose homeostasis GO:0001678 9.56 ABCC8 GCK
13 glucose homeostasis GO:0042593 9.56 GCK HNF4A INS INSR
14 positive regulation of glycolytic process GO:0045821 9.55 INS INSR
15 negative regulation of gluconeogenesis GO:0045721 9.54 GCK INS
16 negative regulation of insulin secretion GO:0046676 9.54 ABCC8 HADH KCNJ11
17 positive regulation of insulin receptor signaling pathway GO:0046628 9.52 IGF2 INS
18 positive regulation of mitotic nuclear division GO:0045840 9.5 IGF2 INS INSR
19 regulation of insulin secretion GO:0050796 9.46 ABCC8 GCK HNF4A KCNJ11
20 positive regulation of respiratory burst GO:0060267 9.43 INS INSR
21 glucose metabolic process GO:0006006 9.26 GCK IGF2 INS KCNJ11
22 positive regulation of glycogen biosynthetic process GO:0045725 8.92 GCK IGF2 INS INSR

Molecular functions related to Hyperinsulinemic Hypoglycemia according to GeneCards Suite gene sharing:

id Name GO ID Score Top Affiliating Genes
1 hormone activity GO:0005179 9.33 IGF2 INS SST
2 insulin receptor binding GO:0005158 9.32 IGF2 INS
3 NAD+ binding GO:0070403 9.26 GLUD1 HADH
4 ATP-activated inward rectifier potassium channel activity GO:0015272 8.96 ABCC8 KCNJ11
5 insulin-like growth factor receptor binding GO:0005159 8.8 IGF2 INS INSR

Sources for Hyperinsulinemic Hypoglycemia

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
16 ExPASy
18 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 MedGen
42 MeSH
43 MESH via Orphanet
44 MGI
46 NCI
47 NCIt
48 NDF-RT
51 NINDS
52 Novoseek
54 OMIM
55 OMIM via Orphanet
59 PubMed
60 QIAGEN
65 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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