MCID: HRT040
MIFTS: 41

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
Orphanet 59 ORPHA411593
ICD10 via Orphanet 34 E16.1
UMLS via Orphanet 74 C0854359
UMLS 73 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 hyperinsulinemic hypoglycemia and hypoglycemia. An important gene associated with Hirata Disease is INS (Insulin), and among its related pathways/superpathways are Allograft rejection and Influenza A. Affiliated tissues include bone, heart and pancreas, and related phenotypes are acanthosis nigricans and weight loss

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 65)
# Related Disease Score Top Affiliating Genes
1 hyperinsulinemic hypoglycemia 30.0 ABCC8 INS
2 hypoglycemia 30.0 ABCC8 GCG INS
3 autoimmune disease 29.8 HLA-DQA1 HLA-DRB1 INS
4 diabetes mellitus 29.0 ABCC8 GCG HLA-DQA1 HLA-DRB1 INS
5 type 1 diabetes mellitus 5 10.2 INS TNFRSF10A
6 autoimmune disease of endocrine system 10.1 INS TNFRSF10A
7 type 1 diabetes mellitus 15 10.1 GCG INS
8 type 1 diabetes mellitus 12 10.1 HLA-DRB1 INS
9 hyperinsulinemic hypoglycemia, familial, 7 10.1 GCG INS
10 maturity-onset diabetes of the young, type 9 10.1 GCG INS
11 neurodermatitis 10.1 INS SLC26A3
12 dumping syndrome 10.1 GCG INS
13 pancreatic cystadenoma 10.1 GCG INS
14 central nervous system vasculitis 10.1 HLA-DRB1 TNFRSF10A
15 prediabetes syndrome 10.1 GCG INS
16 gastrointestinal system benign neoplasm 10.1 GCG INS
17 keshan disease 10.1 HLA-DOA HLA-DQA1
18 palindromic rheumatism 10.1 HLA-DRB1 TNFRSF10A
19 autoimmune disease of gastrointestinal tract 10.0 HLA-DQA1 TNFRSF10A
20 beryllium disease 10.0 HLA-DQA1 HLA-DRB1
21 insulinomatosis and diabetes mellitus 10.0 ABCC8 INS
22 eosinophilia-myalgia syndrome 10.0 HLA-DQA1 HLA-DRB1
23 fanconi-bickel syndrome 10.0 ABCC8 INS
24 temporal arteritis 10.0 HLA-DRB1 TNFRSF10A
25 sympathetic ophthalmia 10.0 HLA-DQA1 HLA-DRB1
26 idiopathic bronchiectasis 10.0 HLA-DQA1 HLA-DRB1
27 graves' disease 10.0
28 histoplasmosis 10.0 HLA-DQA1 HLA-DRB1
29 maturity-onset diabetes of the young, type 13 10.0 ABCC8 INS
30 acute disseminated encephalomyelitis 10.0 HLA-DQA1 HLA-DRB1
31 oligoarticular juvenile idiopathic arthritis 10.0 HLA-DQA1 HLA-DRB1
32 endocrine pancreas disease 10.0 ABCC8 INS
33 hypoglycemic coma 10.0
34 pancreatic agenesis 10.0 ABCC8 INS
35 monogenic diabetes 9.9 ABCC8 INS
36 carbohydrate metabolic disorder 9.9 ABCC8 INS
37 thiamine-responsive megaloblastic anemia syndrome 9.9 ABCC8 INS
38 bone inflammation disease 9.9 HLA-DRB1 TNFRSF10A
39 diabetes mellitus, transient neonatal, 1 9.9 ABCC8 INS
40 focal epithelial hyperplasia 9.9 HLA-DQA1 HLA-DRB1 TNFRSF10A
41 vogt-koyanagi-harada disease 9.9 HLA-DQA1 HLA-DRB1
42 rheumatic fever 9.9 HLA-DQA1 HLA-DRB1 MYOM2
43 rheumatic heart disease 9.9 HLA-DQA1 HLA-DRB1 MYOM2
44 takayasu arteritis 9.9 HLA-DQA1 HLA-DRB1
45 acute insulin response 9.8 ABCC8 GCG INS
46 pancreas disease 9.8 ABCC8 GCG INS
47 glucose metabolism disease 9.8 ABCC8 GCG INS
48 acquired metabolic disease 9.8 ABCC8 GCG INS
49 insulinoma 9.8 ABCC8 GCG INS
50 hyperglycemia 9.8 ABCC8 GCG INS

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 acanthosis nigricans 59 32 frequent (33%) Frequent (79-30%) HP:0000956
2 weight loss 59 32 frequent (33%) Frequent (79-30%) HP:0001824
3 arthralgia/arthritis 59 32 frequent (33%) Frequent (79-30%) HP:0005059
4 hyperinsulinemic hypoglycemia 59 32 obligate (100%) Obligate (100%) HP:0000825
5 increased antibody level in blood 59 32 hallmark (90%) Very frequent (99-80%) HP:0010702
6 insulin-resistant diabetes mellitus 59 32 hallmark (90%) Very frequent (99-80%) HP:0000831
7 systemic lupus erythematosus 59 32 hallmark (90%) Very frequent (99-80%) HP:0002725
8 autoimmune antibody positivity 59 32 hallmark (90%) Very frequent (99-80%) HP:0030057
9 fasting hypoglycemia 59 32 frequent (33%) Frequent (79-30%) HP:0003162
10 nonketotic hypoglycemia 59 32 frequent (33%) Frequent (79-30%) HP:0001958
11 reactive hypoglycemia 59 32 frequent (33%) Frequent (79-30%) HP:0012051
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
Bone, Heart, Pancreas

Publications for Hirata Disease

Articles related to Hirata Disease:

(show all 19)
# Title Authors Year
1
Potential cause-effect relationship between insulin autoimmune syndrome and alpha lipoic acid: Two case reports. ( 30086435 )
2019
2
Assessment and Management of Anti-Insulin Autoantibodies in Varying Presentations of Insulin Autoimmune Syndrome. ( 30085133 )
2018
3
Insulin autoimmune syndrome: from diagnosis to clinical management. ( 30306074 )
2018
4
Insulin autoimmune syndrome in an occidental woman: a case report and literature review. ( 30462811 )
2018
5
Spontaneously remitting insulin autoimmune syndrome in a patient taking alpha-lipoic acid. ( 30532997 )
2018
6
Insulin autoimmune syndrome in a pregnant female: A rare case report. ( 29390469 )
2017
7
Recurrent Hypoglycaemia due to Insulin Autoimmune Disease (Hirata Disease) Associated with Monoclonal Gammopathy of Unknown Significance. ( 27608696 )
2015
8
Insulin autoimmune syndrome (Hirata Disease) in European Caucasians taking I+-lipoic acid. ( 24111525 )
2013
9
Two cases of methimazole-induced insulin autoimmune syndrome in graves' disease. ( 24396652 )
2013
10
Insulin autoimmune syndrome (Hirata disease) as differential diagnosis in patients with hyperinsulinemic hypoglycemia. ( 16670629 )
2006
11
Insulin autoimmune syndrome due to IgG kappa paraprotein. ( 14757564 )
2004
12
Worldwide differences in the incidence of insulin autoimmune syndrome (Hirata disease) with respect to the evolution of HLA-DR4 alleles. ( 10717808 )
2000
13
Insulin autoimmune syndrome (IAS, Hirata disease). ( 10445096 )
1999
14
Differential immunogenetic determinants of polyclonal insulin autoimmune syndrome (Hirata's disease) and monoclonal insulin autoimmune syndrome. ( 7556962 )
1995
15
Insulin autoimmune syndrome (Hirata disease): clinical features and epidemiology in Japan. ( 8200300 )
1994
16
Patients with Graves' disease who developed insulin autoimmune syndrome (Hirata disease) possess HLA-Bw62/Cw4/DR4 carrying DRB1*0406. ( 8325948 )
1993
17
Strong association of insulin autoimmune syndrome with HLA-DR4. ( 1346661 )
1992
18
The immunoglobulin class, the subclass and the ratio of kappa:lambda light chain of autoantibodies to human insulin in insulin autoimmune syndrome. ( 2518827 )
1989
19
Insulin autoimmune syndrome with insulin-resistant diabetes at the incipient stage prior to hypoglycemic attacks. ( 3571854 )
1986

Variations for Hirata Disease

Expression for Hirata Disease

Search GEO for disease gene expression data for Hirata Disease.

Pathways for Hirata Disease

Pathways related to Hirata Disease according to GeneCards Suite gene sharing:

(show all 21)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
12.59 HLA-DOA HLA-DQA1 HLA-DRB1 INS
2
Show member pathways
12.39 HLA-DOA HLA-DQA1 HLA-DRB1 TNFRSF10A
3
Show member pathways
12.31 HLA-DOA HLA-DQA1 HLA-DRB1
4 12.16 HLA-DOA HLA-DQA1 HLA-DRB1
5
Show member pathways
12.12 HLA-DOA HLA-DQA1 HLA-DRB1
6
Show member pathways
12.1 ABCC8 GCG INS
7 12.02 HLA-DOA HLA-DQA1 HLA-DRB1
8
Show member pathways
11.97 HLA-DOA HLA-DQA1 HLA-DRB1
9 11.91 HLA-DOA HLA-DQA1 HLA-DRB1
10 11.81 HLA-DOA HLA-DQA1 HLA-DRB1
11 11.7 HLA-DOA HLA-DQA1 HLA-DRB1
12
Show member pathways
11.65 HLA-DOA HLA-DQA1 HLA-DRB1
13 11.59 HLA-DOA HLA-DQA1 HLA-DRB1
14 11.51 HLA-DOA HLA-DQA1 HLA-DRB1
15 11.42 HLA-DOA HLA-DQA1 HLA-DRB1
16 11.29 HLA-DOA HLA-DQA1 HLA-DRB1
17 11.27 HLA-DOA HLA-DQA1 HLA-DRB1
18
Show member pathways
11.25 GCG INS
19 11.23 ABCC8 INS
20 10.98 HLA-DOA HLA-DQA1 HLA-DRB1
21 10.55 HLA-DOA HLA-DQA1 HLA-DRB1

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 lysosome GO:0005764 9.61 HLA-DOA HLA-DQA1 HLA-DRB1
2 lysosomal membrane GO:0005765 9.5 HLA-DOA HLA-DQA1 HLA-DRB1
3 endocytic vesicle membrane GO:0030666 9.46 HLA-DQA1 HLA-DRB1
4 ER to Golgi transport vesicle membrane GO:0012507 9.4 HLA-DQA1 HLA-DRB1
5 transport vesicle membrane GO:0030658 9.37 HLA-DQA1 HLA-DRB1
6 endosome membrane GO:0010008 9.33 HLA-DOA HLA-DQA1 HLA-DRB1
7 clathrin-coated endocytic vesicle membrane GO:0030669 9.26 HLA-DQA1 HLA-DRB1
8 integral component of lumenal side of endoplasmic reticulum membrane GO:0071556 8.96 HLA-DQA1 HLA-DRB1
9 MHC class II protein complex GO:0042613 8.8 HLA-DOA HLA-DQA1 HLA-DRB1

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

# Name GO ID Score Top Affiliating Genes
1 antigen processing and presentation of exogenous peptide antigen via MHC class II GO:0019886 9.33 HLA-DOA HLA-DQA1 HLA-DRB1
2 regulation of insulin secretion GO:0050796 9.32 ABCC8 GCG
3 positive regulation of insulin secretion involved in cellular response to glucose stimulus GO:0035774 9.26 GCG HLA-DRB1
4 antigen processing and presentation GO:0019882 9.13 HLA-DOA HLA-DQA1 HLA-DRB1
5 antigen processing and presentation of peptide or polysaccharide antigen via MHC class II GO:0002504 8.8 HLA-DOA HLA-DQA1 HLA-DRB1

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

# Name GO ID Score Top Affiliating Genes
1 peptide antigen binding GO:0042605 9.16 HLA-DQA1 HLA-DRB1
2 MHC class II protein complex binding GO:0023026 8.96 HLA-DOA HLA-DRB1
3 MHC class II receptor activity GO:0032395 8.8 HLA-DOA HLA-DQA1 HLA-DRB1

Sources for Hirata Disease

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 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 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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