MCID: INS009
MIFTS: 20

Insulin-Resistance Type B

Categories: Endocrine diseases, Immune diseases, Rare diseases

Aliases & Classifications for Insulin-Resistance Type B

MalaCards integrated aliases for Insulin-Resistance Type B:

Name: Insulin-Resistance Type B 20
Insulin-Resistance Syndrome Type B 58 29
Insulin Resistance - Type B 70

Characteristics:

Orphanet epidemiological data:

58
insulin-resistance syndrome type b
Inheritance: Not applicable; Age of onset: Adult;

Classifications:

Orphanet: 58  
Rare endocrine diseases


Summaries for Insulin-Resistance Type B

GARD : 20 The following summary is from Orphanet, a European reference portal for information on rare diseases and orphan drugs. Orpha Number: 2298 Definition Type B insulin-resistance syndrome belongs to the group of extreme insulin-resistance syndromes (which includes leprechaunism, the lipodystrophies, Rabson-Mendenhall syndrome, and type A insulin resistance syndrome; see these terms) and occurs in the context of immune dysfunction. Epidemiology It is a rare disorder that affects middle-aged adults, predominantly females. Clinical description It may occur in the context of a well-characterized autoimmune disease (systemic lupus erythematosus; see this term), or suggest an immune disease (such as an elevated sedimentation rate, proteinuria, high levels of antinuclear antibodies or decreased levels of certain complement factors). The onset of the disease is usually marked with a rapidly progressive nonketotic and severely insulin-resistant diabetes, along with acanthosis nigricans (the typical skin lesion associated with insulin resistance) and hirsutism. Paradoxal hypoglycemia is sometimes observed and may be extremely severe. Etiology The syndrome is associated with the presence of serum auto-antibodies against the insulin receptor. Diagnostic methods The diagnosis is based on the clinical picture, results of laboratory tests, and on detection of anti-insulin receptor auto-antibodies in the serum. Management and treatment Treatment of the underlying autoimmune disease consists of non-specific immunosuppressors associated with very high doses of insulin to try to control the hyperglycemia. Prognosis Prognosis depends on the underlying autoimmune disease, but it is unfavorable in cases with hypoglycemia (leading to death in 50% of cases).

MalaCards based summary : Insulin-Resistance Type B, is also known as insulin-resistance syndrome type b. Affiliated tissues include salivary gland, and related phenotypes are weight loss and acanthosis nigricans

Related Diseases for Insulin-Resistance Type B

Diseases in the Rare Insulin-Resistance Syndrome family:

Insulin-Resistance Type B

Symptoms & Phenotypes for Insulin-Resistance Type B

Human phenotypes related to Insulin-Resistance Type B:

58 31 (show top 50) (show all 51)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 weight loss 58 31 hallmark (90%) Very frequent (99-80%) HP:0001824
2 acanthosis nigricans 58 31 hallmark (90%) Very frequent (99-80%) HP:0000956
3 hypotriglyceridemia 58 31 hallmark (90%) Very frequent (99-80%) HP:0012153
4 elevated erythrocyte sedimentation rate 58 31 hallmark (90%) Very frequent (99-80%) HP:0003565
5 systemic lupus erythematosus 58 31 hallmark (90%) Very frequent (99-80%) HP:0002725
6 postprandial hyperglycemia 58 31 hallmark (90%) Very frequent (99-80%) HP:0011998
7 fasting hyperinsulinemia 58 31 hallmark (90%) Very frequent (99-80%) HP:0008283
8 abnormal oral glucose tolerance 58 31 hallmark (90%) Very frequent (99-80%) HP:0004924
9 abnormal circulating fatty-acid concentration 58 31 hallmark (90%) Very frequent (99-80%) HP:0004359
10 type ii diabetes mellitus 58 31 frequent (33%) Frequent (79-30%) HP:0005978
11 proteinuria 58 31 frequent (33%) Frequent (79-30%) HP:0000093
12 fatigue 58 31 frequent (33%) Frequent (79-30%) HP:0012378
13 enlarged polycystic ovaries 58 31 frequent (33%) Frequent (79-30%) HP:0008675
14 hypoalbuminemia 58 31 frequent (33%) Frequent (79-30%) HP:0003073
15 hirsutism 58 31 frequent (33%) Frequent (79-30%) HP:0001007
16 leukopenia 58 31 frequent (33%) Frequent (79-30%) HP:0001882
17 glycosuria 58 31 frequent (33%) Frequent (79-30%) HP:0003076
18 antinuclear antibody positivity 58 31 frequent (33%) Frequent (79-30%) HP:0003493
19 increased serum testosterone level 58 31 frequent (33%) Frequent (79-30%) HP:0030088
20 insulin-resistant diabetes mellitus 58 31 frequent (33%) Frequent (79-30%) HP:0000831
21 abnormality of circulating leptin level 58 31 frequent (33%) Frequent (79-30%) HP:0004361
22 nephritis 58 31 frequent (33%) Frequent (79-30%) HP:0000123
23 decreased serum complement factor b 58 31 frequent (33%) Frequent (79-30%) HP:0005416
24 alopecia 58 31 occasional (7.5%) Occasional (29-5%) HP:0001596
25 biliary cirrhosis 58 31 occasional (7.5%) Occasional (29-5%) HP:0002613
26 thrombocytopenia 58 31 occasional (7.5%) Occasional (29-5%) HP:0001873
27 skin rash 58 31 occasional (7.5%) Occasional (29-5%) HP:0000988
28 osteoarthritis 58 31 occasional (7.5%) Occasional (29-5%) HP:0002758
29 pneumonia 58 31 occasional (7.5%) Occasional (29-5%) HP:0002090
30 abnormal salivary gland morphology 58 31 occasional (7.5%) Occasional (29-5%) HP:0010286
31 increased circulating iga level 58 31 occasional (7.5%) Occasional (29-5%) HP:0003261
32 multiple myeloma 58 31 occasional (7.5%) Occasional (29-5%) HP:0006775
33 hodgkin lymphoma 58 31 occasional (7.5%) Occasional (29-5%) HP:0012189
34 increased body weight 58 31 occasional (7.5%) Occasional (29-5%) HP:0004324
35 increased circulating igg level 58 31 occasional (7.5%) Occasional (29-5%) HP:0003237
36 hyperinsulinemic hypoglycemia 58 31 occasional (7.5%) Occasional (29-5%) HP:0000825
37 fasting hypoglycemia 58 31 occasional (7.5%) Occasional (29-5%) HP:0003162
38 diabetic ketoacidosis 58 31 occasional (7.5%) Occasional (29-5%) HP:0001953
39 glucose intolerance 58 Very frequent (99-80%)
40 hyperinsulinemia 58 Very frequent (99-80%)
41 autoimmunity 58 Very frequent (99-80%)
42 abnormality of lipid metabolism 58 Frequent (79-30%)
43 polycystic ovaries 58 Frequent (79-30%)
44 lymphoma 58 Occasional (29-5%)
45 decreased body weight 58 Frequent (79-30%)
46 insulin resistance 58 Very frequent (99-80%)
47 hyperglycemia 58 Very frequent (99-80%)
48 enlarged ovaries 58 Frequent (79-30%)
49 abnormal glucose tolerance 58 Very frequent (99-80%)
50 ketosis 58 Occasional (29-5%)

Drugs & Therapeutics for Insulin-Resistance Type B

Search Clinical Trials , NIH Clinical Center for Insulin-Resistance Type B

Genetic Tests for Insulin-Resistance Type B

Genetic tests related to Insulin-Resistance Type B:

# Genetic test Affiliating Genes
1 Insulin-Resistance Syndrome Type B 29

Anatomical Context for Insulin-Resistance Type B

MalaCards organs/tissues related to Insulin-Resistance Type B:

40
Salivary Gland

Publications for Insulin-Resistance Type B

Articles related to Insulin-Resistance Type B:

# Title Authors PMID Year
1
Type B insulin resistance syndrome. 61
27254267 2016
2
Regression of acanthosis nigricans correlates with disappearance of anti-insulin receptor autoantibodies and achievement of euglycemia in type B insulin resistance syndrome. 61
17445543 2007
3
Insulin resistance in obese boys with acanthosis nigricans. 61
1304190 1992
4
Immunoprecipitation of 125I-insulin crosslinked receptor. 61
6873516 1983
5
Receptor binding studies and clinical effects of human insulin (recombinant DNA): studies in patients with newly diagnosed type i diabetes, type II diabetes, insulin resistance (type A and type B), insulin antibodies, insulin allergy, and "brittle" diabetes. 61
6765528 1982
6
Remission of insulin resistant diabetes in two patients with anti-insulin receptor antibodies. 61
6758201 1982

Variations for Insulin-Resistance Type B

Expression for Insulin-Resistance Type B

Search GEO for disease gene expression data for Insulin-Resistance Type B.

Pathways for Insulin-Resistance Type B

GO Terms for Insulin-Resistance Type B

Sources for Insulin-Resistance Type B

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 20-May-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
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