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The following summary is from Orphanet, a European reference portal for information on rare diseases and orphan drugs.
Orpha Number: 2298
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.
It is a rare disorder that affects middle-aged adults, predominantly females.
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.
The syndrome is associated with the presence of serum auto-antibodies against the insulin receptor.
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 depends on the underlying autoimmune disease, but it is unfavorable in cases with hypoglycemia (leading to death in 50% of cases).
Visit the Orphanet disease page for more resources.
Source: GARD Last updated on 05-01-20
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