A型胰岛素抵抗综合征的治疗及五年随访：一例报告
Treatment and five-year follow-up of type A insulin resistance syndrome: A case report.
作者信息
Chen Yong-Hua, Chen Qing-Qing, Wang Chun-Lin
机构信息
Department of Pediatrics, the First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou 310003, Zhejiang Province, China.
出版信息
World J Clin Cases. 2022 Mar 16;10(8):2522-2528. doi: 10.12998/wjcc.v10.i8.2522.
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.
背景
A型胰岛素抵抗综合征（TAIRS）是一种罕见的疾病，其特征是由于胰岛素受体信号缺陷导致严重胰岛素抵抗。目前尚无治疗TAIRS的特效药物。我们报告1例TAIRS患者接受吡格列酮和氟他胺联合治疗5年并取得成功的病例。
病例摘要
我们报道1例罕见的11岁9个月女性患者，患有A型胰岛素抵抗和胰岛素受体（INSR）杂合突变（c.3614C>T），接受了吡格列酮和氟他胺联合治疗。该治疗方案降低了糖化血红蛋白、空腹胰岛素和雄激素水平。
结论
吡格列酮减轻了该TAIRS患者的胰岛素抵抗，氟他胺改善了男性化症状。
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