MCID: HRT040
MIFTS: 38

Hirata Disease

Categories: Endocrine diseases, Immune diseases, Rare diseases

Aliases & Classifications for Hirata Disease

MalaCards integrated aliases for Hirata Disease:

Name: Hirata Disease 12 20 58 15
Insulin Autoimmune Syndrome 12 20 58
Insulin Autoimmune Hypoglycemia 20

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
SNOMED-CT 67 408539000
ICD10 via Orphanet 33 E16.1
UMLS via Orphanet 72 C0854359
Orphanet 58 ORPHA411593
UMLS 71 C0854359

Summaries for Hirata Disease

GARD : 20 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 CTLA4 Signaling and G-protein signaling N-RAS regulation pathway. Affiliated tissues include t cells, liver and skin, and related phenotypes are hyperinsulinemic hypoglycemia and systemic lupus erythematosus

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

Wikipedia : 74 Insulin autoimmune syndrome (IAS), a rare cause of reversible autoimmune hypoglycemia also known as... more...

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 109)
# Related Disease Score Top Affiliating Genes
1 hypoglycemic coma 30.4 SI INS
2 diabetes mellitus, ketosis-prone 28.6 SLC30A8 SLC30A10 SI MGAM INS
3 hyperinsulinism 10.4
4 hypoglycemia 10.4
5 fasting hypoglycemia 10.3
6 hyperthyroidism 10.2
7 type 1 diabetes mellitus 12 10.2 TNFRSF10A INS
8 type 1 diabetes mellitus 5 10.2 TNFRSF10A INS
9 type 1 diabetes mellitus 2 10.2 TNFRSF10A INS
10 diabetes mellitus 10.1
11 diabetic autonomic neuropathy 10.1 SI INS
12 lactose intolerance 10.1 SI INS
13 pneumatosis cystoides intestinalis 10.1 SI MGAM
14 barre-lieou syndrome 10.1 SI MGAM
15 miliaria 10.1 SI MGAM
16 graves' disease 10.1
17 hyperinsulinemic hypoglycemia 10.1
18 insulinoma 10.1
19 acute laryngopharyngitis 10.1 SI MGAM
20 trigonitis 10.1 SI MGAM
21 chicken egg allergy 10.1 SI MGAM
22 ocular motor apraxia 10.1
23 tremor 10.1
24 sucrase-isomaltase deficiency, congenital 10.1 SI MGAM
25 latent autoimmune diabetes in adults 10.1 INS HLA-DRB1
26 parkinson disease 17 10.1 SI MGAM
27 glycogen storage disease ii 10.1 SI MGAM
28 glycogen storage disease iii 10.1 SI MGAM
29 focal epithelial hyperplasia 10.1 TNFRSF10A HLA-DRB1
30 pemphigoid gestationis 10.0 TNFRSF10A HLA-DRB1
31 mucopolysaccharidosis iv 10.0 SI MGAM
32 mucopolysaccharidosis, type vi 10.0 SI MGAM
33 central nervous system vasculitis 10.0 TNFRSF10A HLA-DRB1
34 protein-energy malnutrition 10.0 SI MGAM
35 type 2 diabetes mellitus 10.0
36 myeloma, multiple 10.0
37 liver cirrhosis 10.0
38 amelogenesis imperfecta, type ie 10.0 TNFRSF10A HLA-DRB1
39 mucopolysaccharidosis, type ii 10.0 SI MGAM
40 scheie syndrome 10.0 SI MGAM
41 panuveitis 10.0 TNFRSF10A HLA-DRB1
42 autoimmune disease 10.0
43 hyperglycemia 10.0
44 cataract 10.0
45 diabetic neuropathy 10.0
46 postgastrectomy syndrome 10.0 SI MGAM INS
47 functional gastric disease 10.0 SI MGAM INS
48 nasopharyngitis 10.0 SI MGAM INS
49 carbohydrate metabolic disorder 10.0 SI MGAM INS
50 krabbe disease 10.0 SI MGAM

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 14)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 hyperinsulinemic hypoglycemia 58 31 obligate (100%) Obligate (100%) HP:0000825
2 systemic lupus erythematosus 58 31 hallmark (90%) Very frequent (99-80%) HP:0002725
3 insulin-resistant diabetes mellitus 58 31 hallmark (90%) Very frequent (99-80%) HP:0000831
4 autoimmune antibody positivity 58 31 hallmark (90%) Very frequent (99-80%) HP:0030057
5 increased circulating antibody level 31 hallmark (90%) HP:0010702
6 arthralgia/arthritis 58 31 frequent (33%) Frequent (79-30%) HP:0005059
7 weight loss 58 31 frequent (33%) Frequent (79-30%) HP:0001824
8 acanthosis nigricans 58 31 frequent (33%) Frequent (79-30%) HP:0000956
9 nonketotic hypoglycemia 58 31 frequent (33%) Frequent (79-30%) HP:0001958
10 fasting hypoglycemia 58 31 frequent (33%) Frequent (79-30%) HP:0003162
11 reactive hypoglycemia 58 31 frequent (33%) Frequent (79-30%) HP:0012051
12 autoimmunity 58 Frequent (79-30%)
13 increased antibody level in blood 58 Very frequent (99-80%)
14 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, Skin, B Cells

Publications for Hirata Disease

Articles related to Hirata Disease:

(show top 50) (show all 220)
# Title Authors PMID Year
1
Diagnosis of insulin autoimmune syndrome using polyethylene glycol precipitation and gel filtration chromatography with ex vivo insulin exchange. 61 20
27588366 2017
2
Use of the continuous glucose monitoring system to treat insulin autoimmune syndrome: quantification of glucose excursions and evaluation of treatment efficacy. 20 61
25077287 2014
3
An uncommon cause of hypoglycemia: insulin autoimmune syndrome. 61 20
25060673 2014
4
Drug-induced insulin autoimmune syndrome. 20 61
19070385 2009
5
Autoimmune insulin syndrome. 20
2698754 1989
6
Insulin autoimmune syndrome in patients with type 2 diabetes: A report of two cases. 61
31445080 2021
7
[One case report of insulin autoimmune syndrome induced by clopidogrel]. 61
33397023 2021
8
[Insulin autoimmune syndrome caused by proton pump inhibitors: a case report]. 61
33397024 2021
9
Uncooked cornstarch for the prevention of hypoglycemic events. 61
33455416 2021
10
Long-term follow-up of insulin autoimmune syndrome in an elderly patient. 61
33363855 2020
11
Clinical and Laboratory Aspects of Insulin Autoantibody-Mediated Glycaemic Dysregulation and Hyperinsulinaemic Hypoglycaemia: Insulin Autoimmune Syndrome and Exogenous Insulin Antibody Syndrome. 61
33343044 2020
12
Insulin autoimmune syndrome: a rare cause of spontaneous hypoglycaemia in non-diabetic individuals. 61
33298474 2020
13
A novel type of extreme insulin resistance: nonhypoglycaemic insulin autoimmune syndrome. 61
33382420 2020
14
Combinations of plasma exchange with steroids treats α-lipoic acid induced insulin autoimmune syndrome in a Chinese woman. 61
33295634 2020
15
Methimazole-induced insulin autoimmune syndrome in Graves' disease with hypokalemia: A case report and literature review. 61
32973942 2020
16
Insulin autoimmune syndrome misdiagnosed as an insulinoma in a woman presenting with a pancreatic cystic lesion and taking alpha lipoic acid: a lesson to be learned. 61
33175361 2020
17
Insulin Autoimmune Syndrome - A Case Series. 61
33117451 2020
18
Analysis of the clinical characteristics of insulin autoimmune syndrome induced by methimazole. 61
33119911 2020
19
[Clinical Characteristics of Lipoic Acid-mediated Insulin Autoimmune Syndrome in Two Patients with Type 2 Diabetes Mellitus]. 61
33131528 2020
20
Insulin autoimmune syndrome: A rare cause of hypoglycemia. 61
33209842 2020
21
Assessment of adverse reactions to α-lipoic acid containing dietary supplements through spontaneous reporting systems. 61
32778460 2020
22
[Hypoglycemic syndrome in patients with monoclonal gammopathy]. 61
33351331 2020
23
Insulin autoimmune syndrome. 61
32554828 2020
24
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 2020
25
Insulin Autoimmune Syndrome: A Case of Clopidogrel-induced Autoimmune Hypoglycemia. 61
32182368 2020
26
HLA DRB1*0415: a new possible genetic susceptibility factor for Hirata's disease. 61
31734778 2020
27
Insulin autoimmune syndrome - time to remember. 61
31909453 2020
28
Insulin Autoimmune Syndrome After Exposure to Clopidogrel: A Case Report. 61
31858916 2020
29
Insulin Autoimmune Syndrome (Hirata Disease): A Comprehensive Review Fifty Years After Its First Description. 61
32308449 2020
30
Insulin autoimmune syndrome induced by exogenous insulin injection: a four-case series. 61
31883520 2019
31
Comparison of Two Autoimmune Dysglycemia Syndromes: Insulin Autoimmune Syndrome (IAS) and Type B Insulin Resistance Syndrome (B-IRS). 61
31683342 2019
32
Rituximab for treatment of refractory insulin autoimmune syndrome associated with non-Hodgkin B-cell lymphoma. 61
31009087 2019
33
Insulin autoimmune syndrome possibly caused by coenzyme Q10. 61
31191778 2019
34
Insulin Autoimmune Syndrome Diagnosis and Therapy in a Single Chinese Center. 61
30992145 2019
35
Recurrent hypoglycemia, a rare case of insulin autoimmune syndrome in a young African American male. 61
31235174 2019
36
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
37
A novel treatment of hyperinsulinemic hypoglycemia induced by insulin antibodies with alkali administration: a case report. 61
30853027 2019
38
Enhancement of postprandial endogenous insulin secretion rather than exogenous insulin injection ameliorated insulin antibody-induced unstable diabetes: a case report. 61
30621663 2019
39
Insulin Autoimmune Syndrome in a 25-Year-Old, Previously Healthy Kuwaiti Man. 61
31915554 2019
40
Potential cause-effect relationship between insulin autoimmune syndrome and alpha lipoic acid: Two case reports. 61
30086435 2019
41
Insulin autoimmune syndrome as a cause of recurrent hypoglycemia in a carbimazole user: a case report from Nepal. 61
30799961 2019
42
GLUCOSE CHANGES IN A PATIENT WITH INSULIN AUTOIMMUNE SYNDROME DEMONSTRATED BY CONTINUOUS GLUCOSE MONITORING. 61
31966997 2019
43
Insulin autoimmune syndrome: Three case reports. 61
30572448 2018
44
Autoimmune Hypoglycemia Relapse on Glucocorticoids, Effectively Treated with Azathioprine. 61
31313558 2018
45
Insulin autoimmune syndrome in an occidental woman: a case report and literature review. 61
30462811 2018
46
Assessment and Management of Anti-Insulin Autoantibodies in Varying Presentations of Insulin Autoimmune Syndrome. 61
30085133 2018
47
Insulin autoimmune syndrome: from diagnosis to clinical management. 61
30306074 2018
48
A case report of insulin autoimmune syndrome in a Central European individual. 61
29683798 2018
49
Insulin autoimmune syndrome (Hirata's disease) in an Italian patient: a case report and review of the literature. 61
28917079 2018
50
Immunoadsorption Followed by Rituximab as a Definitive Treatment for Insulin Autoimmune Syndrome (Hirata Syndrome): A Case Report. 61
29282202 2018

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 11.11 HLA-DRB1 HLA-DOA
2
Show member pathways
10.91 HLA-DRB1 HLA-DOA
3 10.83 SI MGAM
4 9.58 SI MGAM

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 Golgi apparatus GO:0005794 9.35 TNFRSF10A SLC30A8 SLC30A10 SI HLA-DRB1
2 transport vesicle membrane GO:0030658 9.16 SLC30A8 HLA-DRB1
3 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 carbohydrate metabolic process GO:0005975 9.58 SI MGAM INS
2 antigen processing and presentation GO:0019882 9.43 HLA-DRB1 HLA-DOA
3 cellular zinc ion homeostasis GO:0006882 9.37 SLC30A8 SLC30A10
4 zinc ion transport GO:0006829 9.32 SLC30A8 SLC30A10
5 zinc ion transmembrane transport GO:0071577 9.26 SLC30A8 SLC30A10
6 negative regulation of reactive oxygen species biosynthetic process GO:1903427 9.16 SLC30A10 INS
7 antigen processing and presentation of peptide or polysaccharide antigen via MHC class II GO:0002504 8.96 HLA-DRB1 HLA-DOA
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 9.37 SI MGAM
2 zinc ion transmembrane transporter activity GO:0005385 9.32 SLC30A8 SLC30A10
3 cation transmembrane transporter activity GO:0008324 9.26 SLC30A8 SLC30A10
4 MHC class II protein complex binding GO:0023026 9.16 HLA-DRB1 HLA-DOA
5 MHC class II receptor activity GO:0032395 8.96 HLA-DRB1 HLA-DOA
6 alpha-1,4-glucosidase activity GO:0004558 8.62 SI MGAM

Sources for Hirata Disease

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
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
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 05-Mar-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 TGDB
70 Tocris
71 UMLS
72 UMLS via Orphanet
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