Type B insulin resistance syndrome
| Classification according to ICD-10 | |
|---|---|
| E13.- | Other specified diabetes mellitus |
| ICD-10 online (WHO version 2019) | |
The insulin resistance syndrome type B is a rare disease with the main features of hyperinsulinemia , acanthosis nigricans and signs of hyperandrogenism as hirsutism .
The name was suggested by the American doctor C. Ronald Kahn and colleagues in 1976.
distribution
The frequency is unknown; the condition affects middle-aged adults, mostly women. It is associated with disorders of the immune system such as systemic lupus erythematosus .
Clinical manifestations
Clinical criteria are:
- Occur along with dysfunction of the immune system
- Initially mostly rapidly increasing, non- ketotic and markedly insulin-resistant diabetes mellitus
- Acanthosis nigricans
- Hirsutism
diagnosis
The diagnosis is based on the clinical findings, in particular the detection of anti-insulin receptor autoantibodies in the blood serum .
Differential diagnosis
Other diseases from the group of extremely insulin-resistant syndromes must be distinguished:
- Leprechaunism
- Lipodystrophies
- Rabson-Mendenhall Syndrome
- Insulin resistance syndrome type A (in young women without detectable antibodies against insulin receptors )
therapy
The treatment is directed against the underlying autoimmune disease and against the hyperglycaemia .
literature
- Seon Mee Kang, Heung Yong Jin and others a .: Type B insulin-resistance syndrome presenting as autoimmune hypoglycemia, associated with systemic lupus erythematosus and interstitial lung disease. In: The Korean Journal of Internal Medicine. Vol. 28, 2013, p. 98, doi: 10.3904 / kjim.2013.28.1.98 .
Individual evidence
- ↑ a b c d e Insulin resistance syndrome type B. In: Orphanet (database for rare diseases).
- ↑ CR Kahn, JS Flier, RS Bar, JA Archer, P. Gorden, MM Martin, J. Roth: The syndromes of insulin resistance and acanthosis nigricans. Insulin-receptor disorders in man. In: The New England Journal of Medicine . Vol. 294, No. 14, April 1976, pp. 739-745, doi: 10.1056 / NEJM197604012941401 , PMID 176581 .
- ↑ R. Malek, AY Chong et al. a .: Treatment of Type B Insulin Resistance: A Novel Approach to Reduce Insulin Receptor Autoantibodies. In: The Journal of Clinical Endocrinology & Metabolism. Vol. 95, 2010, p. 3641, doi: 10.1210 / jc.2010-0167 .
