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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 flutamid...

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Detalles Bibliográficos
Autores principales: Chen, Yong-Hua, Chen, Qing-Qing, Wang, Chun-Lin
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Baishideng Publishing Group Inc 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8968608/
https://www.ncbi.nlm.nih.gov/pubmed/35434067
http://dx.doi.org/10.12998/wjcc.v10.i8.2522
Descripción
Sumario: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.