MCID: HRT040
MIFTS: 39

Hirata Disease

Categories: Endocrine diseases, Immune diseases, Rare diseases

Aliases & Classifications for Hirata Disease

MalaCards integrated aliases for Hirata Disease:

Name: Hirata Disease 12 53 59 15
Insulin Autoimmune Syndrome 12 53 59
Insulin Autoimmune Hypoglycemia 53

Characteristics:

Orphanet epidemiological data:

59
insulin autoimmune syndrome
Inheritance: Not applicable; Age of onset: Adult,Elderly;

Classifications:

Orphanet: 59  
Rare endocrine diseases


External Ids:

Disease Ontology 12 DOID:0040100
ICD10 via Orphanet 34 E16.1
UMLS via Orphanet 73 C0854359
Orphanet 59 ORPHA411593
UMLS 72 C0854359

Summaries for Hirata Disease

NIH Rare Diseases : 53 Insulin autoimmune syndrome is a rare condition that causes low blood sugar (hypoglycemia). This occurs because the body begins to make a specific kind of protein called antibodies to attack insulin. Insulin is a naturally occurring hormone that is responsible for keeping blood sugar at a normal level. When blood sugar levels get too high, insulin helps to store the sugar for future use. People affected by insulin autoimmune syndrome have antibodies that attack insulin, causing it to work too hard and the level of blood sugar to become too low. Insulin autoimmune syndrome most often begins during adulthood.

MalaCards based summary : Hirata Disease, also known as insulin autoimmune syndrome, is related to insulinoma and hyperinsulinism. An important gene associated with Hirata Disease is INS (Insulin), and among its related pathways/superpathways are Aldosterone synthesis and secretion and Integration of energy metabolism. Affiliated tissues include t cells, b cells and liver, and related phenotypes are hyperinsulinemic hypoglycemia and increased antibody level in blood

Disease Ontology : 12 An autoimmune disease of endocrine system characterized by hypoglycemia caused by antibodies to insulin.

Related Diseases for Hirata Disease

Diseases related to Hirata Disease via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 98)
# Related Disease Score Top Affiliating Genes
1 insulinoma 30.2 INS GCG ABCC8
2 hyperinsulinism 30.0 KCNJ11 INS ABCC8
3 hyperinsulinemic hypoglycemia 29.9 KCNJ11 INS ABCC8
4 hypoglycemia 29.7 KCNJ11 INS GCG ABCC8
5 hyperglycemia 29.1 KCNJ11 INS GCG ABCC8
6 diabetes mellitus 28.4 KCNJ11 INS HLA-DRB1 GCG ABCC8
7 type 1 diabetes mellitus 12 10.5 INS HLA-DRB1
8 type 1 diabetes mellitus 5 10.5 TNFRSF10A INS
9 neurodermatitis 10.5 SLC26A3 INS
10 central nervous system vasculitis 10.5 TNFRSF10A HLA-DRB1
11 pharyngitis 10.4 MYOM2 HLA-DRB1
12 type 1 diabetes mellitus 15 10.4 INS GCG
13 autoimmune disease of endocrine system 10.4 TNFRSF10A INS
14 hyperinsulinemic hypoglycemia, familial, 7 10.4 INS GCG
15 maturity-onset diabetes of the young, type 9 10.4 INS GCG
16 insulinomatosis and diabetes mellitus 10.3 INS ABCC8
17 fanconi-bickel syndrome 10.3 INS ABCC8
18 glycogen storage disease vii 10.3 MYOM2 MGAM
19 dumping syndrome 10.3 INS GCG
20 pancreatic cystadenoma 10.3 INS GCG
21 focal epithelial hyperplasia 10.3 TNFRSF10A HLA-DRB1
22 graves' disease 10.3
23 prediabetes syndrome 10.2 INS GCG
24 maturity-onset diabetes of the young, type 10 10.2 KCNJ11 INS
25 palindromic rheumatism 10.2 TNFRSF10A HLA-DRB1
26 fasting hypoglycemia 10.2
27 gastrointestinal system benign neoplasm 10.2 INS GCG
28 asphyxia neonatorum 10.1 MGAM ABCC8
29 hyperthyroidism 10.1
30 rheumatic fever 10.1 MYOM2 HLA-DRB1
31 autoimmune disease 10.1
32 cataract 10.1
33 diabetic neuropathy 10.1
34 ecthyma 10.1 MYOM2 KCNJ11
35 munchausen by proxy 10.0 KCNJ11 ABCC8
36 cardiomyopathy, dilated, 1o 10.0 KCNJ11 ABCC8
37 epiphyseal dysplasia, multiple, with early-onset diabetes mellitus 10.0 KCNJ11 ABCC8
38 ocular motor apraxia 10.0
39 hypoglycemic coma 10.0
40 diabetes mellitus, insulin-dependent 10.0 INS HLA-DRB1 GCG
41 usher syndrome, type ic 9.9 KCNJ11 ABCC8
42 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 9.9
43 liver cirrhosis 9.9
44 tremor 9.9
45 maturity-onset diabetes of the young, type 13 9.8 KCNJ11 INS ABCC8
46 endocrine pancreas disease 9.8 KCNJ11 INS ABCC8
47 pancreatic agenesis 9.8 KCNJ11 INS ABCC8
48 monogenic diabetes 9.8 KCNJ11 INS ABCC8
49 thiamine-responsive megaloblastic anemia syndrome 9.8 KCNJ11 INS ABCC8
50 diabetes mellitus, transient neonatal, 1 9.8 KCNJ11 INS ABCC8

Graphical network of the top 20 diseases related to Hirata Disease:



Diseases related to Hirata Disease

Symptoms & Phenotypes for Hirata Disease

Human phenotypes related to Hirata Disease:

59 32 (show all 13)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 hyperinsulinemic hypoglycemia 59 32 obligate (100%) Obligate (100%) HP:0000825
2 increased antibody level in blood 59 32 hallmark (90%) Very frequent (99-80%) HP:0010702
3 insulin-resistant diabetes mellitus 59 32 hallmark (90%) Very frequent (99-80%) HP:0000831
4 systemic lupus erythematosus 59 32 hallmark (90%) Very frequent (99-80%) HP:0002725
5 autoimmune antibody positivity 59 32 hallmark (90%) Very frequent (99-80%) HP:0030057
6 acanthosis nigricans 59 32 frequent (33%) Frequent (79-30%) HP:0000956
7 weight loss 59 32 frequent (33%) Frequent (79-30%) HP:0001824
8 arthralgia/arthritis 59 32 frequent (33%) Frequent (79-30%) HP:0005059
9 reactive hypoglycemia 59 32 frequent (33%) Frequent (79-30%) HP:0012051
10 fasting hypoglycemia 59 32 frequent (33%) Frequent (79-30%) HP:0003162
11 nonketotic hypoglycemia 59 32 frequent (33%) Frequent (79-30%) HP:0001958
12 autoimmunity 59 Frequent (79-30%)
13 insulin resistance 59 Very frequent (99-80%)

Drugs & Therapeutics for Hirata Disease

Search Clinical Trials , NIH Clinical Center for Hirata Disease

Genetic Tests for Hirata Disease

Anatomical Context for Hirata Disease

MalaCards organs/tissues related to Hirata Disease:

41
T Cells, B Cells, Liver, Skin, Testes, Pancreatic Islet

Publications for Hirata Disease

Articles related to Hirata Disease:

(show top 50) (show all 191)
# Title Authors PMID Year
1
Diagnosis of insulin autoimmune syndrome using polyethylene glycol precipitation and gel filtration chromatography with ex vivo insulin exchange. 38 6
27588366 2017
2
Use of the continuous glucose monitoring system to treat insulin autoimmune syndrome: quantification of glucose excursions and evaluation of treatment efficacy. 38 6
25077287 2014
3
An uncommon cause of hypoglycemia: insulin autoimmune syndrome. 38 6
25060673 2014
4
Drug-induced insulin autoimmune syndrome. 38 6
19070385 2009
5
Autoimmune insulin syndrome. 6
2698754 1989
6
Rituximab for treatment of refractory insulin autoimmune syndrome associated with non-Hodgkin B-cell lymphoma. 38
31009087 2019
7
Recurrent hypoglycemia, a rare case of insulin autoimmune syndrome in a young African American male. 38
31235174 2019
8
Insulin Autoimmune Syndrome Diagnosis and Therapy in a Single Chinese Center. 38
30992145 2019
9
Insulin autoimmune syndrome possibly caused by coenzyme Q10. 38
31191778 2019
10
Pitfalls in the diagnosis of insulin autoimmune syndrome (Hirata's disease) in a hypoglycemic child: a case report and review of the literature. 38
30862762 2019
11
A novel treatment of hyperinsulinemic hypoglycemia induced by insulin antibodies with alkali administration: a case report. 38
30853027 2019
12
Enhancement of postprandial endogenous insulin secretion rather than exogenous insulin injection ameliorated insulin antibody-induced unstable diabetes: a case report. 38
30621663 2019
13
Potential cause-effect relationship between insulin autoimmune syndrome and alpha lipoic acid: Two case reports. 38
30086435 2019
14
Insulin autoimmune syndrome as a cause of recurrent hypoglycemia in a carbimazole user: a case report from Nepal. 38
30799961 2019
15
Insulin autoimmune syndrome: Three case reports. 38
30572448 2018
16
Autoimmune Hypoglycemia Relapse on Glucocorticoids, Effectively Treated with Azathioprine. 38
31313558 2018
17
Insulin autoimmune syndrome in an occidental woman: a case report and literature review. 38
30462811 2018
18
Assessment and Management of Anti-Insulin Autoantibodies in Varying Presentations of Insulin Autoimmune Syndrome. 38
30085133 2018
19
Insulin autoimmune syndrome: from diagnosis to clinical management. 38
30306074 2018
20
Insulin autoimmune syndrome (Hirata's disease) in an Italian patient: a case report and review of the literature. 38
28917079 2018
21
A case report of insulin autoimmune syndrome in a Central European individual. 38
29683798 2018
22
Immunoadsorption Followed by Rituximab as a Definitive Treatment for Insulin Autoimmune Syndrome (Hirata Syndrome): A Case Report. 38
29282202 2018
23
Therapeutic plasma exchange normalizes insulin-mediated response in a child with type 1 diabetes and insulin autoimmune syndrome. 38
28370959 2018
24
[Current Status of Health Foods Including Their Interactions with Drugs and Adverse Events]. 38
30504666 2018
25
Autoimmune hypoglycaemia caused by alpha-lipoic acid: a rare condition in Caucasian patients. 38
30532998 2018
26
Insulin Autoimmune Syndrome: a rare case of hypoglycaemia resolving with immunosuppression. 38
29588555 2018
27
Insulin autoimmune syndrome in a pregnant female: A rare case report: Erratum. 38
29369220 2018
28
Insulin autoimmune syndrome in an Argentine woman taking α-lipoic acid: A case report and review of the literature. 38
30627435 2018
29
Spontaneously remitting insulin autoimmune syndrome in a patient taking alpha-lipoic acid. 38
30532997 2018
30
Insulin autoimmune syndrome in a pregnant female: A rare case report. 38
29390469 2017
31
Clopidogrel-Induced Insulin Autoimmune Syndrome: A Newly Recognized Cause of Hypoglycemia in a Patient Without Diabetes. 38
29264578 2017
32
Resolution of Hypoglycemia and Cardiovascular Dysfunction After Rituximab Treatment of Insulin Autoimmune Syndrome. 38
28461453 2017
33
High glycated albumin (GA) levels and the GA/HbA1c ratio in patients with insulin autoimmune syndrome. 38
30603322 2017
34
Rare Cause of Recurrent Hypoglycemia: Insulin Autoimmune Syndrome. 38
29318060 2017
35
Human serum albumin: Possible cause of insulin autoimmune syndrome. 38
27804283 2016
36
The insulin autoimmune syndrome (IAS) as a cause of hypoglycaemia: an update on the pathophysiology, biochemical investigations and diagnosis. 38
27071154 2016
37
Insulin-induced autoimmune syndrome: A case report. 38
27882163 2016
38
High fasting serum insulin level due to autoantibody interference in insulin immunoassay discloses autoimmune insulin syndrome: a case report. 38
27492703 2016
39
Methimazole-induced insulin autoimmune syndrome. 38
27540463 2016
40
Insulin autoimmune syndrome during the administration of clopidogrel. 38
26847582 2016
41
Spontaneous hypoglycemia: diagnostic evaluation and management. 38
26951054 2016
42
Novel Management of Insulin Autoimmune Syndrome with Rituximab and Continuous Glucose Monitoring. 38
26982011 2016
43
[Insulin autoimmune syndrome]. 38
27266161 2016
44
Spontaneous hypoglycaemia in a patient with Graves' disease. 38
27005798 2016
45
Hyperinsulinemic hypoglycemia associated with insulin antibodies caused by exogenous insulin analog. 38
27933175 2016
46
Insulin Autoimmune Syndrome: a rare cause of postprandial hypoglycemia. 38
27855237 2016
47
Insulin Autoimmune Syndrome Accompanied by Multiple Myeloma. 38
27522998 2016
48
Gliclazide-Induced Insulin Autoimmune Syndrome: A Rare Case Report and Review on Literature. 38
28017142 2016
49
Two Sisters with Graves' Disease and Similar Clinical Features who Tested Positive for Anti-insulin Antibodies after Thiamazole Treatment. 38
27150866 2016
50
Recurrent Hypoglycaemia due to Insulin Autoimmune Disease (Hirata Disease) Associated with Monoclonal Gammopathy of Unknown Significance. 38
27608696 2015

Variations for Hirata Disease

Expression for Hirata Disease

Search GEO for disease gene expression data for Hirata Disease.

Pathways for Hirata Disease

GO Terms for Hirata Disease

Cellular components related to Hirata Disease according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 plasma membrane GO:0005886 9.65 TNFRSF10A SLC26A3 SI PTK2B MGAM KCNJ11
2 MHC class II protein complex GO:0042613 9.16 HLA-DRB1 HLA-DOA
3 inward rectifying potassium channel GO:0008282 8.62 KCNJ11 ABCC8

Biological processes related to Hirata Disease according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 carbohydrate metabolic process GO:0005975 9.61 SI MGAM INS
2 positive regulation of insulin secretion involved in cellular response to glucose stimulus GO:0035774 9.4 HLA-DRB1 GCG
3 negative regulation of insulin secretion GO:0046676 9.37 KCNJ11 ABCC8
4 positive regulation of nitric-oxide synthase activity GO:0051000 9.32 PTK2B INS
5 positive regulation of cellular protein metabolic process GO:0032270 9.26 PTK2B INS
6 antigen processing and presentation of peptide or polysaccharide antigen via MHC class II GO:0002504 9.16 HLA-DRB1 HLA-DOA
7 regulation of insulin secretion GO:0050796 9.13 KCNJ11 GCG ABCC8
8 polysaccharide digestion GO:0044245 8.62 SI MGAM

Molecular functions related to Hirata Disease according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 hydrolase activity, hydrolyzing O-glycosyl compounds GO:0004553 9.32 SI MGAM
2 MHC class II protein complex binding GO:0023026 9.26 HLA-DRB1 HLA-DOA
3 MHC class II receptor activity GO:0032395 9.16 HLA-DRB1 HLA-DOA
4 ATP-activated inward rectifier potassium channel activity GO:0015272 8.65 KCNJ11
5 alpha-1,4-glucosidase activity GO:0004558 8.32 MGAM

Sources for Hirata Disease

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 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 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 TGDB
71 Tocris
72 UMLS
73 UMLS via Orphanet
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