Diabetes in a Patient with Glycogen Storage Disease Type 1a

• Published in: Internal Medicine 2024/08/01, Vol.63(15), pp.2153-2156
• Main Authors: Kanemaru, Yoshinori, Harada, Norio, Wada, Naoki, Yasuda, Takuma, Okamura, Emi, Fujii, Toshihito, Ogura, Masahito, Inagaki, Nobuya
• Format: Article
• Language: English
• Subjects: Adult; Blood Glucose - metabolism; Case Report; diabetes; Diabetes mellitus; dipeptidyl peptidase-4 inhibitor; Dipeptidyl-Peptidase IV Inhibitors - therapeutic use; Female; Glucose monitoring; Glycogen; glycogen storage disease type 1a; Glycogen Storage Disease Type I - complications; Glycogen Storage Disease Type I - diagnosis; Glycoside Hydrolase Inhibitors - therapeutic use; Humans; Hyperglycemia; Hyperglycemia - complications; Hyperglycemia - diagnosis; Hypoglycemia; Hypoglycemic Agents - therapeutic use; intermittently scanned continuous glucose monitoring; Life span; Male; Rare diseases; Storage diseases; α-Glucosidase; α-glucosidase inhibitor
• ISSN: 0918-2918; 1349-7235; 1349-7235
• DOI: 10.2169/internalmedicine.2766-23

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).