A Case Report of Diabetes in a Patient with Glycogen Storage Disease Type 1a

Intern Med. 2024 Jan 2. doi: 10.2169/internalmedicine.2766-23. Online ahead of print.

Abstract

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).

Keywords: diabetes; dipeptidyl peptidase-4 inhibitor; glycogen storage disease type 1a; hypoglycemia; intermittently scanned continuous glucose monitoring; α-glucosidase inhibitor.