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 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. Affiliated tissues include pancreas, skin and t 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 68)
# 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 28.6 ABCC8 GCG INS KCNJ11
4 diabetes mellitus 28.3 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 maturity-onset diabetes of the young, type 10 10.0 INS KCNJ11
25 rheumatic heart disease 10.0 HLA-DRB1 MYOM2
26 ocular motor apraxia 10.0
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 alcoholic liver cirrhosis 9.7
43 arthritis 9.7
44 glomerulonephritis 9.7
45 hepatitis 9.7
46 lymphoma 9.7
47 hepatitis c 9.7
48 viral hepatitis 9.7
49 spondylitis 9.7
50 hyperthyroidism 9.7

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 reactive hypoglycemia 60 33 frequent (33%) Frequent (79-30%) HP:0012051
10 fasting hypoglycemia 60 33 frequent (33%) Frequent (79-30%) HP:0003162
11 nonketotic hypoglycemia 60 33 frequent (33%) Frequent (79-30%) HP:0001958
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

MalaCards organs/tissues related to Hirata Disease:

42
Pancreas, Skin, T Cells, B Cells, Liver, Heart

Publications for Hirata Disease

Articles related to Hirata Disease:

(show top 50) (show all 117)
# 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
Rituximab for treatment of refractory insulin autoimmune syndrome associated with non-Hodgkin B-cell lymphoma. ( 31009087 )
2019
3
Potential cause-effect relationship between insulin autoimmune syndrome and alpha lipoic acid: Two case reports. ( 30086435 )
2019
4
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
5
Insulin Autoimmune Syndrome Diagnosis and Therapy in a Single Chinese Center. ( 30992145 )
2019
6
Therapeutic plasma exchange normalizes insulin-mediated response in a child with type 1 diabetes and insulin autoimmune syndrome. ( 28370959 )
2018
7
Insulin autoimmune syndrome (Hirata's disease) in an Italian patient: a case report and review of the literature. ( 28917079 )
2018
8
Immunoadsorption Followed by Rituximab as a Definitive Treatment for Insulin Autoimmune Syndrome (Hirata Syndrome): A Case Report. ( 29282202 )
2018
9
Insulin autoimmune syndrome in a pregnant female: A rare case report: Erratum. ( 29369220 )
2018
10
Insulin Autoimmune Syndrome: a rare case of hypoglycaemia resolving with immunosuppression. ( 29588555 )
2018
11
A case report of insulin autoimmune syndrome in a Central European individual. ( 29683798 )
2018
12
Assessment and Management of Anti-Insulin Autoantibodies in Varying Presentations of Insulin Autoimmune Syndrome. ( 30085133 )
2018
13
Insulin autoimmune syndrome: from diagnosis to clinical management. ( 30306074 )
2018
14
Insulin autoimmune syndrome in an occidental woman: a case report and literature review. ( 30462811 )
2018
15
Spontaneously remitting insulin autoimmune syndrome in a patient taking alpha-lipoic acid. ( 30532997 )
2018
16
Insulin autoimmune syndrome: Three case reports. ( 30572448 )
2018
17
Insulin autoimmune syndrome in an Argentine woman taking α-lipoic acid: A case report and review of the literature. ( 30627435 )
2018
18
Clopidogrel-Induced Insulin Autoimmune Syndrome: A Newly Recognized Cause of Hypoglycemia in a Patient Without Diabetes. ( 29264578 )
2017
19
Rare Cause of Recurrent Hypoglycemia: Insulin Autoimmune Syndrome. ( 29318060 )
2017
20
Resolution of Hypoglycemia and Cardiovascular Dysfunction After Rituximab Treatment of Insulin Autoimmune Syndrome. ( 28461453 )
2017
21
Diagnosis of insulin autoimmune syndrome using polyethylene glycol precipitation and gel filtration chromatography with ex vivo insulin exchange. ( 27588366 )
2017
22
Insulin autoimmune syndrome in a pregnant female: A rare case report. ( 29390469 )
2017
23
High glycated albumin (GA) levels and the GA/HbA1c ratio in patients with insulin autoimmune syndrome. ( 30603322 )
2017
24
Insulin Autoimmune Syndrome: a rare cause of postprandial hypoglycemia. ( 27855237 )
2016
25
Insulin autoimmune syndrome during the administration of clopidogrel. ( 26847582 )
2016
26
Novel Management of Insulin Autoimmune Syndrome with Rituximab and Continuous Glucose Monitoring. ( 26982011 )
2016
27
The insulin autoimmune syndrome (IAS) as a cause of hypoglycaemia: an update on the pathophysiology, biochemical investigations and diagnosis. ( 27071154 )
2016
28
Insulin Autoimmune Syndrome Accompanied by Multiple Myeloma. ( 27522998 )
2016
29
Methimazole-induced insulin autoimmune syndrome. ( 27540463 )
2016
30
Human serum albumin: Possible cause of insulin autoimmune syndrome. ( 27804283 )
2016
31
Gliclazide-Induced Insulin Autoimmune Syndrome: A Rare Case Report and Review on Literature. ( 28017142 )
2016
32
Recurrent Hypoglycaemia due to Insulin Autoimmune Disease (Hirata Disease) Associated with Monoclonal Gammopathy of Unknown Significance. ( 27608696 )
2015
33
Rare association of insulin autoimmune syndrome with ankylosing spondylitis. ( 26527431 )
2015
34
Unusual Manifestations of Essential Monoclonal Gammopathy. II. Simulation of the Insulin Autoimmune Syndrome. ( 26241232 )
2015
35
Insulin Autoimmune Syndrome: 73 Cases of Clinical Analysis. ( 26315093 )
2015
36
Insulin autoimmune syndrome (Hirata Disease) in European Caucasians taking α-lipoic acid. ( 24111525 )
2014
37
An uncommon cause of hypoglycemia: insulin autoimmune syndrome. ( 25060673 )
2014
38
Recurrent hypoglycemia from insulin autoimmune syndrome. ( 23979685 )
2014
39
Hypoglycemic coma due to insulin autoimmune syndrome induced by methimazole: A rare case report. ( 25289063 )
2014
40
Analytical and clinical challenges in a patient with concurrent type 1 diabetes, subcutaneous insulin resistance and insulin autoimmune syndrome. ( 24711924 )
2014
41
Use of the continuous glucose monitoring system to treat insulin autoimmune syndrome: quantification of glucose excursions and evaluation of treatment efficacy. ( 25077287 )
2014
42
Characterization of insulin antibodies by Surface Plasmon Resonance in two clinical cases: brittle diabetes and insulin autoimmune syndrome. ( 24386337 )
2013
43
Two cases of methimazole-induced insulin autoimmune syndrome in graves' disease. ( 24396652 )
2013
44
Insulin autoimmune syndrome induced by methimazole in a patient with Graves' disease. ( 23817229 )
2013
45
Insulin autoimmune syndrome induced by methimazole in a Korean girl with Graves' disease. ( 24904848 )
2013
46
Insulin autoimmune syndrome in a health supplement user: the effectiveness of cornstarch therapy for treating hypoglycemia. ( 23370747 )
2013
47
A novel atypical presentation of insulin autoimmune syndrome (Hirata's disease) in a child. ( 23843578 )
2013
48
Insulin autoimmune syndrome caused by an adhesive skin patch containing loxoprofen-sodium. ( 24190150 )
2013
49
Insulin glulisine may cause a disease resembling insulin autoimmune syndrome: case report. ( 24265369 )
2013
50
Recurrent Insulin Autoimmune Syndrome Caused by α-Lipoic Acid in Type 2 Diabetes. ( 24396698 )
2013

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 8.32 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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