Treatment and five-year follow-up of type A insulin resistance syndrome: A case report

BACKGROUND Type A insulin resistance syndrome (TAIRS) is a rare disorder characterized by severe insulin resistance due to defects in insulin receptor signaling. No specific drugs are available for the treatment of TAIRS. We report a case of TAIRS successfully treated with pioglitazone and flutamide for 5 years. CASE SUMMARY We present the rare case of a female patient aged 11 years and 9 mo with type A insulin resistance and an INSR heterozygous mutation (c.3614C>T), who was treated with a combination of pioglitazone and flutamide. This treatment regimen reduced hemoglobin A1c, fasting insulin and androgen levels. CONCLUSION Pioglitazone attenuated insulin resistance in this patient with TAIRS, and flutamide ameliorated masculinization.