Syndromes of Severe Insulin Resistance

The document reviewed Syndromes of Severe Insulin Resistance (SSIRs), detailing both inherited and acquired disorders that lead to profound impairment of insulin action, distinct from common conditions like type 2 diabetes. It described clinical manifestations such as diabetes requiring high insulin doses, acanthosis nigricans, and ovarian hyperandrogenism. The text classified SSIRs into primary disorders of insulin action, lipodystrophies, and other inherited disorders, highlighting specific syndromes like Donohue's syndrome, Rabson-Mendenhall syndrome, and Type A insulin resistance, including their genetic mutations and clinical features. It also discussed HAIR-AN syndrome, Type B insulin resistance, and lipodystrophic syndromes, noting the role of abnormal fatty acid metabolism and ectopic fat accumulation in insulin resistance. The document mentioned treatments like plasmapheresis, immunosuppression, and recombinant leptin for managing symptoms, despite the rarity of these conditions and the lack of evidence-based treatments.