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

摘要

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.3614 C>T),who was treated with a combination of pioglitazone and flutamide.This treatment regimen reduced hemoglobin A1 c,fasting insulin and androgen levels.CONCLUSION Pioglitazone attenuated insulin resistance in this patient with TAIRS,and flutamide ameliorated masculinization.