Published online Mar 16, 2022. doi: 10.12998/wjcc.v10.i8.2522
Peer-review started: July 27, 2021
First decision: October 22, 2021
Revised: November 2, 2021
Accepted: February 10, 2022
Article in press: February 10, 2022
Published online: March 16, 2022
Processing time: 226 Days and 13 Hours
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.
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.
Pioglitazone attenuated insulin resistance in this patient with TAIRS, and flutamide ameliorated masculinization.
Core Tip: Type A insulin resistance syndrome (TAIRS) is a rare disorder characterized by severe insulin resistance due to defects in signaling through the insulin receptor. We present the rare case of a female patient aged 11 years and 9 mo who had type A insulin resistance with an INSR heterozygous mutation (c.3614C>T). This is the first case report describing the use of pioglitazone and flutamide used in combination in a child with TAIRS. This protocol for TAIRS is inexpensive, effective, and free of side effects.