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

Yong-Hua Chen; Qing-Qing Chen; Chun-Lin Wang

doi:10.12998/wjcc.v10.i8.2522

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

World J Clin Cases. 2022 Mar 16;10(8):2522-2528. doi: 10.12998/wjcc.v10.i8.2522.

Authors

Yong-Hua Chen¹, Qing-Qing Chen¹, Chun-Lin Wang²

Affiliations

¹ Department of Pediatrics, the First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou 310003, Zhejiang Province, China.
² Department of Pediatrics, the First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou 310003, Zhejiang Province, China. hzwangcl@zju.edu.cn.

Abstract

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.

Keywords: Case report; Flutamide; Pioglitazone; Treatment; Type A insulin resistance syndrome.

Publication types

Case Reports