Department of Diabetes, Endocrinology and Nutrition, Graduate School of Medicine, Kyoto University, Japan.
P.I.I.F. Tazuke-Kofukai Medical Research Institute, Kitano Hospital, Japan.
Intern Med. 2024 Aug 1;63(15):2153-2156. doi: 10.2169/internalmedicine.2766-23. Epub 2024 Jan 2.
Glycogen storage disease type 1a (GSD-1a) is a rare congenital disease. Recently, life expectancy with GSD-1a has been improved by its early diagnosis and management. Complications of diabetes with GSD-1a are extremely rare. The optimal treatment for glucose control using this disease combination remains unclear. The existence of GSD-1a and diabetes can cause both hypoglycemia and hyperglycemia, making glucose control especially problematic. In the present report, α-glucosidase inhibitor (α-GI) and dipeptidyl peptidase-4 (DPP-4) inhibitors improved hyperglycemia without symptoms of hypoglycemia in a patient with diabetes and GSD-1a using intermittent continuous glucose monitoring (isCGM).
糖原贮积病 1a 型(GSD-1a)是一种罕见的先天性疾病。最近,通过早期诊断和管理,GSD-1a 的预期寿命得到了提高。糖尿病伴 GSD-1a 的并发症极为罕见。这种疾病组合使用何种方法来控制血糖的最佳治疗方法仍不清楚。GSD-1a 和糖尿病的存在会导致低血糖和高血糖,使血糖控制特别成问题。在本报告中,使用间歇性连续血糖监测(isCGM),α-葡萄糖苷酶抑制剂(α-GI)和二肽基肽酶-4(DPP-4)抑制剂改善了高血糖而没有低血糖症状的糖尿病伴 GSD-1a 患者的病情。