Division of Endocrinology and Metabolism, Department of Medicine, Jichi Medical University, Shimotsuke, Japan.
J Diabetes Investig. 2020 Sep;11(5):1363-1365. doi: 10.1111/jdi.13241. Epub 2020 Mar 20.
Type A insulin resistance (IR) syndrome is a severe IR form caused by insulin receptor (INSR) gene defects. Antidiabetic drugs, including high-dose insulin and insulin-sensitizing agents, often fail to control associated hyperglycemia. Therapy with recombinant human insulin-like growth factor 1 can be more effective, but it is expensive. We report a case of type A IR syndrome with an in-frame INSR heterozygous deletion (ΔLeu999) that was treated with a combination of conventional therapy and ipragliflozin, a sodium-glucose cotransporter 2 inhibitor. Treatment reduced hemoglobin A1c levels (10.0-7.5%) and induced weight loss (54.4-52.0 kg) within 2 months, and the effects were sustained for >3 years. Sodium-glucose cotransporter 2 inhibitors might be useful to normalize blood glucose in type A IR syndrome by reducing bodyweight and ameliorating glucotoxicity.
A型胰岛素抵抗（IR）综合征是一种由胰岛素受体（INSR）基因缺陷引起的严重 IR 形式。抗糖尿病药物，包括大剂量胰岛素和胰岛素增敏剂，往往无法控制相关的高血糖。用重组人生长因子 1 治疗可能更有效，但价格昂贵。我们报告了一例 A 型 IR 综合征，其 INSR 杂合缺失（ΔLeu999）为框内缺失，用常规治疗联合伊帕格列净（一种钠-葡萄糖共转运蛋白 2 抑制剂）治疗。治疗在 2 个月内降低了血红蛋白 A1c 水平（10.0-7.5%）并减轻了体重（54.4-52.0kg），并且效果持续了>3 年。钠-葡萄糖共转运蛋白 2 抑制剂可能通过减轻体重和改善糖毒性来使 A 型 IR 综合征的血糖正常化。
