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 52 58 15
Insulin Autoimmune Syndrome 12 52 58
Insulin Autoimmune Hypoglycemia 52

Characteristics:

Orphanet epidemiological data:

58
insulin autoimmune syndrome
Inheritance: Not applicable; Age of onset: Adult,Elderly;

Classifications:

Orphanet: 58  
Rare endocrine diseases


External Ids:

Disease Ontology 12 DOID:0040100
ICD10 via Orphanet 33 E16.1
UMLS via Orphanet 72 C0854359
Orphanet 58 ORPHA411593
UMLS 71 C0854359

Summaries for Hirata Disease

NIH Rare Diseases : 52 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 hypoglycemic coma and diabetes mellitus, ketosis-prone. An important gene associated with Hirata Disease is INS (Insulin), and among its related pathways/superpathways are Peptide hormone metabolism and G-protein signaling N-RAS regulation pathway. Affiliated tissues include t cells, liver and b cells, 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 hypoglycemic coma 30.2 INS GCG
2 diabetes mellitus, ketosis-prone 28.6 SLC30A8 SLC30A10 INS GCG
3 hypoglycemia 10.5
4 hyperinsulinism 10.3
5 type 1 diabetes mellitus 12 10.3 TNFRSF10A INS
6 type 1 diabetes mellitus 2 10.3 TNFRSF10A INS
7 graves' disease 10.3
8 insulinoma 10.3
9 fasting hypoglycemia 10.2
10 pemphigoid gestationis 10.2 TNFRSF10A HLA-DRB1
11 erythematosquamous dermatosis 10.2 INS GCG
12 amelogenesis imperfecta, type ie 10.2 TNFRSF10A HLA-DRB1
13 postgastrectomy syndrome 10.2 INS GCG
14 central nervous system vasculitis 10.2 TNFRSF10A HLA-DRB1
15 acute insulin response 10.1 INS GCG
16 hyperthyroidism 10.1
17 alzheimer disease 6 10.1 INS GCG
18 pancreatic endocrine carcinoma 10.1 INS GCG
19 autoimmune disease 10.1
20 hyperinsulinemic hypoglycemia 10.1
21 cataract 10.1
22 diabetic neuropathy 10.1
23 nasopharyngitis 10.1 INS GCG
24 hyperglycemia 10.1
25 diabetic autonomic neuropathy 10.1 INS GCG
26 panuveitis 10.1 TNFRSF10A HLA-DRB1
27 dumping syndrome 10.1 INS GCG
28 somatostatinoma 10.1 INS GCG
29 maturity-onset diabetes of the young, type 1 10.1 INS GCG
30 functional gastric disease 10.1 INS GCG
31 autonomic nervous system disease 10.1 INS GCG
32 apnea, obstructive sleep 10.1 INS GCG
33 lipodystrophy, familial partial, type 2 10.0 INS GCG
34 endocrine pancreas disease 10.0 INS GCG
35 ocular motor apraxia 10.0
36 carbohydrate metabolic disorder 10.0 INS GCG
37 temporal arteritis 10.0 TNFRSF10A HLA-DRB1
38 islet cell tumor 10.0 INS GCG
39 autoimmune disease of gastrointestinal tract 10.0 TNFRSF10A INS HLA-DRB1
40 wolfram syndrome 10.0 INS HLA-DRB1
41 type 1 diabetes mellitus 3 9.9 SLC30A8 SLC30A10
42 autoimmune polyendocrine syndrome, type ii 9.9 NUDT10 INS
43 alpha/beta t-cell lymphopenia with gamma/delta t-cell expansion, severe cytomegalovirus infection, and autoimmunity 9.9
44 liver cirrhosis 9.9
45 diabetes mellitus 9.9
46 tremor 9.9
47 abdominal obesity-metabolic syndrome 1 9.9 INS GCG
48 adrenal cortex disease 9.8 NUDT10 INS
49 adrenal gland disease 9.8 NUDT10 INS
50 pituitary gland disease 9.8 INS GCG

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:

58 31 (show all 13)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 hyperinsulinemic hypoglycemia 58 31 obligate (100%) Obligate (100%) HP:0000825
2 increased antibody level in blood 58 31 hallmark (90%) Very frequent (99-80%) HP:0010702
3 insulin-resistant diabetes mellitus 58 31 hallmark (90%) Very frequent (99-80%) HP:0000831
4 systemic lupus erythematosus 58 31 hallmark (90%) Very frequent (99-80%) HP:0002725
5 autoimmune antibody positivity 58 31 hallmark (90%) Very frequent (99-80%) HP:0030057
6 acanthosis nigricans 58 31 frequent (33%) Frequent (79-30%) HP:0000956
7 weight loss 58 31 frequent (33%) Frequent (79-30%) HP:0001824
8 arthralgia/arthritis 58 31 frequent (33%) Frequent (79-30%) HP:0005059
9 reactive hypoglycemia 58 31 frequent (33%) Frequent (79-30%) HP:0012051
10 fasting hypoglycemia 58 31 frequent (33%) Frequent (79-30%) HP:0003162
11 nonketotic hypoglycemia 58 31 frequent (33%) Frequent (79-30%) HP:0001958
12 autoimmunity 58 Frequent (79-30%)
13 insulin resistance 58 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:

40
T Cells, Liver, B Cells, Skin, Testes, Pancreatic Islet, Cortex

Publications for Hirata Disease

Articles related to Hirata Disease:

(show top 50) (show all 200)
# Title Authors PMID Year
1
Diagnosis of insulin autoimmune syndrome using polyethylene glycol precipitation and gel filtration chromatography with ex vivo insulin exchange. 61 52
27588366 2017
2
Use of the continuous glucose monitoring system to treat insulin autoimmune syndrome: quantification of glucose excursions and evaluation of treatment efficacy. 61 52
25077287 2014
3
An uncommon cause of hypoglycemia: insulin autoimmune syndrome. 61 52
25060673 2014
4
Drug-induced insulin autoimmune syndrome. 61 52
19070385 2009
5
Autoimmune insulin syndrome. 52
2698754 1989
6
Insulin autoimmune syndrome - time to remember. 61
31909453 2020
7
Insulin autoimmune syndrome after exposure to clopidogrel: a case report. 61
31858916 2019
8
Insulin autoimmune syndrome induced by exogenous insulin injection: a four-case series. 61
31883520 2019
9
Comparison of Two Autoimmune Dysglycemia Syndromes: Insulin Autoimmune Syndrome (IAS) and Type B Insulin Resistance Syndrome (B-IRS). 61
31683342 2019
10
Nationwide survey of endogenous hyperinsulinemic hypoglycemia in Japan (2017-2018): Congenital hyperinsulinism, insulinoma, non-insulinoma pancreatogenous hypoglycemia syndrome and insulin autoimmune syndrome (Hirata's disease). 61
31742894 2019
11
HLA DRB1*0415: a new possible genetic susceptibility factor for Hirata's disease. 61
31734778 2019
12
Insulin autoimmune syndrome in patients with type 2 diabetes: A report of two cases. 61
31445080 2019
13
Rituximab for treatment of refractory insulin autoimmune syndrome associated with non-Hodgkin B-cell lymphoma. 61
31009087 2019
14
Insulin Autoimmune Syndrome Diagnosis and Therapy in a Single Chinese Center. 61
30992145 2019
15
Recurrent hypoglycemia, a rare case of insulin autoimmune syndrome in a young African American male. 61
31235174 2019
16
Insulin autoimmune syndrome possibly caused by coenzyme Q10. 61
31191778 2019
17
Pitfalls in the diagnosis of insulin autoimmune syndrome (Hirata's disease) in a hypoglycemic child: a case report and review of the literature. 61
30862762 2019
18
A novel treatment of hyperinsulinemic hypoglycemia induced by insulin antibodies with alkali administration: a case report. 61
30853027 2019
19
Insulin autoimmune syndrome as a cause of recurrent hypoglycemia in a carbimazole user: a case report from Nepal. 61
30799961 2019
20
Enhancement of postprandial endogenous insulin secretion rather than exogenous insulin injection ameliorated insulin antibody-induced unstable diabetes: a case report. 61
30621663 2019
21
GLUCOSE CHANGES IN A PATIENT WITH INSULIN AUTOIMMUNE SYNDROME DEMONSTRATED BY CONTINUOUS GLUCOSE MONITORING. 61
31966997 2019
22
Potential cause-effect relationship between insulin autoimmune syndrome and alpha lipoic acid: Two case reports. 61
30086435 2019
23
Insulin Autoimmune Syndrome in a 25-Year-Old, Previously Healthy Kuwaiti Man. 61
31915554 2019
24
Insulin autoimmune syndrome: Three case reports. 61
30572448 2018
25
Autoimmune Hypoglycemia Relapse on Glucocorticoids, Effectively Treated with Azathioprine. 61
31313558 2018
26
Insulin autoimmune syndrome in an occidental woman: a case report and literature review. 61
30462811 2018
27
Assessment and Management of Anti-Insulin Autoantibodies in Varying Presentations of Insulin Autoimmune Syndrome. 61
30085133 2018
28
Insulin autoimmune syndrome: from diagnosis to clinical management. 61
30306074 2018
29
Insulin autoimmune syndrome (Hirata's disease) in an Italian patient: a case report and review of the literature. 61
28917079 2018
30
A case report of insulin autoimmune syndrome in a Central European individual. 61
29683798 2018
31
Immunoadsorption Followed by Rituximab as a Definitive Treatment for Insulin Autoimmune Syndrome (Hirata Syndrome): A Case Report. 61
29282202 2018
32
Therapeutic plasma exchange normalizes insulin-mediated response in a child with type 1 diabetes and insulin autoimmune syndrome. 61
28370959 2018
33
Insulin autoimmune syndrome in an Argentine woman taking α-lipoic acid: A case report and review of the literature. 61
30627435 2018
34
Insulin autoimmune syndrome in a pregnant female: A rare case report: Erratum. 61
29369220 2018
35
Insulin Autoimmune Syndrome: a rare case of hypoglycaemia resolving with immunosuppression. 61
29588555 2018
36
Spontaneously remitting insulin autoimmune syndrome in a patient taking alpha-lipoic acid. 61
30532997 2018
37
[Current Status of Health Foods Including Their Interactions with Drugs and Adverse Events]. 61
30504666 2018
38
Autoimmune hypoglycaemia caused by alpha-lipoic acid: a rare condition in Caucasian patients. 61
30532998 2018
39
Insulin autoimmune syndrome in a pregnant female: A rare case report. 61
29390469 2017
40
Clopidogrel-Induced Insulin Autoimmune Syndrome: A Newly Recognized Cause of Hypoglycemia in a Patient Without Diabetes. 61
29264578 2017
41
Resolution of Hypoglycemia and Cardiovascular Dysfunction After Rituximab Treatment of Insulin Autoimmune Syndrome. 61
28461453 2017
42
High glycated albumin (GA) levels and the GA/HbA1c ratio in patients with insulin autoimmune syndrome. 61
30603322 2017
43
Rare Cause of Recurrent Hypoglycemia: Insulin Autoimmune Syndrome. 61
29318060 2017
44
Human serum albumin: Possible cause of insulin autoimmune syndrome. 61
27804283 2016
45
The insulin autoimmune syndrome (IAS) as a cause of hypoglycaemia: an update on the pathophysiology, biochemical investigations and diagnosis. 61
27071154 2016
46
Insulin-induced autoimmune syndrome: A case report. 61
27882163 2016
47
High fasting serum insulin level due to autoantibody interference in insulin immunoassay discloses autoimmune insulin syndrome: a case report. 61
27492703 2016
48
Methimazole-induced insulin autoimmune syndrome. 61
27540463 2016
49
Insulin autoimmune syndrome during the administration of clopidogrel. 61
26847582 2016
50
Spontaneous hypoglycemia: diagnostic evaluation and management. 61
26951054 2016

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
11.06 SLC30A8 INS GCG
2
Show member pathways
10.91 HLA-DRB1 HLA-DOA
3
Show member pathways
10.82 INS GCG

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 transport vesicle membrane GO:0030658 8.96 SLC30A8 HLA-DRB1
2 MHC class II protein complex GO:0042613 8.62 HLA-DRB1 HLA-DOA

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

# Name GO ID Score Top Affiliating Genes
1 adaptive immune response GO:0002250 9.54 IFNE HLA-DRB1 HLA-DOA
2 antigen processing and presentation GO:0019882 9.32 HLA-DRB1 HLA-DOA
3 positive regulation of insulin secretion involved in cellular response to glucose stimulus GO:0035774 9.26 HLA-DRB1 GCG
4 zinc ion transport GO:0006829 9.16 SLC30A8 SLC30A10
5 negative regulation of reactive oxygen species biosynthetic process GO:1903427 8.96 SLC30A10 INS
6 antigen processing and presentation of peptide or polysaccharide antigen via MHC class II GO:0002504 8.62 HLA-DRB1 HLA-DOA

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

# Name GO ID Score Top Affiliating Genes
1 cation transmembrane transporter activity GO:0008324 9.16 SLC30A8 SLC30A10
2 MHC class II protein complex binding GO:0023026 8.96 HLA-DRB1 HLA-DOA
3 MHC class II receptor activity GO:0032395 8.62 HLA-DRB1 HLA-DOA

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 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
53 NINDS
54 Novoseek
56 OMIM
57 OMIM via Orphanet
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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