MCID: HRT040
MIFTS: 37

Hirata Disease

Categories: Endocrine diseases, Immune diseases, Rare diseases

Aliases & Classifications for Hirata Disease

MalaCards integrated aliases for Hirata Disease:

Name: Hirata Disease 12 54 60 15
Insulin Autoimmune Syndrome 12 54 60
Insulin Autoimmune Hypoglycemia 54

Characteristics:

Orphanet epidemiological data:

60
insulin autoimmune syndrome
Inheritance: Not applicable; Age of onset: Adult,Elderly;

Classifications:

Orphanet: 60  
Rare endocrine diseases


External Ids:

Disease Ontology 12 DOID:0040100
ICD10 via Orphanet 35 E16.1
UMLS via Orphanet 75 C0854359
Orphanet 60 ORPHA411593
UMLS 74 C0854359

Summaries for Hirata Disease

NIH Rare Diseases : 54 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 Aldosterone synthesis and secretion and Integration of energy metabolism. 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 66)
# Related Disease Score Top Affiliating Genes
1 hyperinsulinemic hypoglycemia 29.6 ABCC8 INS KCNJ11
2 hypoglycemia 29.4 ABCC8 GCG INS KCNJ11
3 diabetes mellitus, noninsulin-dependent 28.8 ABCC8 GCG INS KCNJ11
4 diabetes mellitus 28.6 ABCC8 GCG HLA-DRB1 INS KCNJ11
5 type 1 diabetes mellitus 12 10.3 HLA-DRB1 INS
6 type 1 diabetes mellitus 5 10.3 INS TNFRSF10A
7 central nervous system vasculitis 10.2 HLA-DRB1 TNFRSF10A
8 autoimmune disease of endocrine system 10.2 INS TNFRSF10A
9 type 1 diabetes mellitus 15 10.2 GCG INS
10 hyperinsulinemic hypoglycemia, familial, 7 10.2 GCG INS
11 maturity-onset diabetes of the young, type 9 10.2 GCG INS
12 dumping syndrome 10.2 GCG INS
13 pharyngitis 10.2 HLA-DRB1 MYOM2
14 pancreatic cystadenoma 10.2 GCG INS
15 palindromic rheumatism 10.1 HLA-DRB1 TNFRSF10A
16 focal epithelial hyperplasia 10.1 HLA-DRB1 TNFRSF10A
17 prediabetes syndrome 10.1 GCG INS
18 insulinomatosis and diabetes mellitus 10.1 ABCC8 INS
19 fanconi-bickel syndrome 10.1 ABCC8 INS
20 gastrointestinal system benign neoplasm 10.1 GCG INS
21 rheumatic fever 10.1 HLA-DRB1 MYOM2
22 autoimmune disease 10.0
23 graves' disease 10.0
24 distal trisomy 3p 10.0
25 maturity-onset diabetes of the young, type 10 10.0 INS KCNJ11
26 rheumatic heart disease 10.0 HLA-DRB1 MYOM2
27 hypoglycemic coma 10.0
28 carbohydrate metabolic disorder 10.0 ABCC8 INS
29 diabetes mellitus, insulin-dependent 9.9 GCG HLA-DRB1 INS
30 ecthyma 9.9 KCNJ11 MYOM2
31 alstrom syndrome 9.9 INS KCNJ11
32 temporal arteritis 9.9 HLA-DRB1 TNFRSF10A
33 munchausen by proxy 9.8 ABCC8 KCNJ11
34 insulinoma 9.8 ABCC8 GCG INS
35 cardiomyopathy, dilated, 1o 9.8 ABCC8 KCNJ11
36 epiphyseal dysplasia, multiple, with early-onset diabetes mellitus 9.8 ABCC8 KCNJ11
37 cantu syndrome 9.8 ABCC8 KCNJ11
38 usher syndrome, type ic 9.8 ABCC8 KCNJ11
39 spondyloarthropathy 1 9.7
40 rheumatoid arthritis 9.7
41 myeloma, multiple 9.7
42 arthritis 9.7
43 glomerulonephritis 9.7
44 hepatitis 9.7
45 hepatitis c 9.7
46 viral hepatitis 9.7
47 spondylitis 9.7
48 hyperthyroidism 9.7
49 brittle diabetes 9.7
50 maturity-onset diabetes of the young, type 13 9.7 ABCC8 INS KCNJ11

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:

60 33 (show all 13)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 hyperinsulinemic hypoglycemia 60 33 obligate (100%) Obligate (100%) HP:0000825
2 increased antibody level in blood 60 33 hallmark (90%) Very frequent (99-80%) HP:0010702
3 insulin-resistant diabetes mellitus 60 33 hallmark (90%) Very frequent (99-80%) HP:0000831
4 systemic lupus erythematosus 60 33 hallmark (90%) Very frequent (99-80%) HP:0002725
5 autoimmune antibody positivity 60 33 hallmark (90%) Very frequent (99-80%) HP:0030057
6 acanthosis nigricans 60 33 frequent (33%) Frequent (79-30%) HP:0000956
7 weight loss 60 33 frequent (33%) Frequent (79-30%) HP:0001824
8 arthralgia/arthritis 60 33 frequent (33%) Frequent (79-30%) HP:0005059
9 fasting hypoglycemia 60 33 frequent (33%) Frequent (79-30%) HP:0003162
10 nonketotic hypoglycemia 60 33 frequent (33%) Frequent (79-30%) HP:0001958
11 reactive hypoglycemia 60 33 frequent (33%) Frequent (79-30%) HP:0012051
12 autoimmunity 60 Frequent (79-30%)
13 insulin resistance 60 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

Publications for Hirata Disease

Articles related to Hirata Disease:

(show all 20)
# Title Authors Year
1
Insulin autoimmune syndrome as a cause of recurrent hypoglycemia in a carbimazole user: a case report from Nepal. ( 30799961 )
2019
2
Potential cause-effect relationship between insulin autoimmune syndrome and alpha lipoic acid: Two case reports. ( 30086435 )
2019
3
Pitfalls in the diagnosis of insulin autoimmune syndrome (Hirata's disease) in a hypoglycemic child: a case report and review of the literature. ( 30862762 )
2019
4
Assessment and Management of Anti-Insulin Autoantibodies in Varying Presentations of Insulin Autoimmune Syndrome. ( 30085133 )
2018
5
Insulin autoimmune syndrome: from diagnosis to clinical management. ( 30306074 )
2018
6
Insulin autoimmune syndrome in an occidental woman: a case report and literature review. ( 30462811 )
2018
7
Spontaneously remitting insulin autoimmune syndrome in a patient taking alpha-lipoic acid. ( 30532997 )
2018
8
Insulin autoimmune syndrome: Three case reports. ( 30572448 )
2018
9
Insulin autoimmune syndrome in an Argentine woman taking α-lipoic acid: A case report and review of the literature. ( 30627435 )
2018
10
High glycated albumin (GA) levels and the GA/HbA1c ratio in patients with insulin autoimmune syndrome. ( 30603322 )
2017
11
Recurrent Hypoglycaemia due to Insulin Autoimmune Disease (Hirata Disease) Associated with Monoclonal Gammopathy of Unknown Significance. ( 27608696 )
2015
12
Insulin autoimmune syndrome (Hirata Disease) in European Caucasians taking I+-lipoic acid. ( 24111525 )
2013
13
Insulin autoimmune syndrome (Hirata disease) as differential diagnosis in patients with hyperinsulinemic hypoglycemia. ( 16670629 )
2006
14
Worldwide differences in the incidence of insulin autoimmune syndrome (Hirata disease) with respect to the evolution of HLA-DR4 alleles. ( 10717808 )
2000
15
Insulin autoimmune syndrome (IAS, Hirata disease). ( 10445096 )
1999
16
Differential immunogenetic determinants of polyclonal insulin autoimmune syndrome (Hirata's disease) and monoclonal insulin autoimmune syndrome. ( 7556962 )
1995
17
Insulin autoimmune syndrome (Hirata disease): clinical features and epidemiology in Japan. ( 8200300 )
1994
18
Patients with Graves' disease who developed insulin autoimmune syndrome (Hirata disease) possess HLA-Bw62/Cw4/DR4 carrying DRB1*0406. ( 8325948 )
1993
19
The immunoglobulin class, the subclass and the ratio of kappa:lambda light chain of autoantibodies to human insulin in insulin autoimmune syndrome. ( 2518827 )
1989
20
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:

# Super pathways Score Top Affiliating Genes
1
Show member pathways
12.42 ABCC8 GCG INS KCNJ11
2
Show member pathways
11.62 ABCC8 GCG INS KCNJ11
3
Show member pathways
11.5 HLA-DOA HLA-DRB1 PTK2B
4 11.48 HLA-DOA HLA-DRB1 PTK2B
5 11.31 HLA-DOA HLA-DRB1
6
Show member pathways
11.31 ABCC8 INS KCNJ11
7 11.2 ABCC8 KCNJ11
8 11.15 ABCC8 KCNJ11
9
Show member pathways
11.07 GCG INS
10 10.44 ABCC8 INS KCNJ11

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 MHC class II protein complex GO:0042613 8.96 HLA-DOA HLA-DRB1
2 inward rectifying potassium channel GO:0008282 8.62 ABCC8 KCNJ11

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

# Name GO ID Score Top Affiliating Genes
1 response to drug GO:0042493 9.63 ABCC8 KCNJ11 PTK2B
2 regulation of synaptic plasticity GO:0048167 9.43 INS PTK2B
3 antigen processing and presentation GO:0019882 9.4 HLA-DOA HLA-DRB1
4 positive regulation of insulin secretion involved in cellular response to glucose stimulus GO:0035774 9.37 GCG HLA-DRB1
5 negative regulation of insulin secretion GO:0046676 9.32 ABCC8 KCNJ11
6 positive regulation of nitric-oxide synthase activity GO:0051000 9.26 INS PTK2B
7 positive regulation of cellular protein metabolic process GO:0032270 9.16 INS PTK2B
8 antigen processing and presentation of peptide or polysaccharide antigen via MHC class II GO:0002504 8.96 HLA-DOA HLA-DRB1
9 regulation of insulin secretion GO:0050796 8.8 ABCC8 GCG KCNJ11

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

# Name GO ID Score Top Affiliating Genes
1 MHC class II protein complex binding GO:0023026 9.16 HLA-DOA HLA-DRB1
2 MHC class II receptor activity GO:0032395 8.96 HLA-DOA HLA-DRB1
3 ATP-activated inward rectifier potassium channel activity GO:0015272 8.62 ABCC8 KCNJ11

Sources for Hirata Disease

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
20 FMA
29 GO
30 GTR
31 HGMD
32 HMDB
33 HPO
34 ICD10
35 ICD10 via Orphanet
36 ICD9CM
37 IUPHAR
38 KEGG
39 LifeMap
41 LOVD
43 MedGen
45 MeSH
46 MESH via Orphanet
47 MGI
50 NCI
51 NCIt
52 NDF-RT
55 NINDS
56 Novoseek
58 OMIM
59 OMIM via Orphanet
63 PubMed
65 QIAGEN
70 SNOMED-CT via HPO
71 SNOMED-CT via Orphanet
72 TGDB
73 Tocris
74 UMLS
75 UMLS via Orphanet
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