Advances in sodium-dependent glucose transporters 2 inhibitor therapy in glycogen storage disease type Ⅰ b
Glycogen storage disease type Ⅰ b(GSD Ⅰ b)is an autosomal recessive genetic disease characterized by abnormal glycogen metabolism.The clinical manifestations include signs of metabolic abnormality of the typical GSD I,and the patients with GSD Ⅰ b also have neutropenia and symptoms including recurrent infection,oral and intestinal mucosal ulcers,inflammatory bowel diseases and autoimmune thyroiditis.However,the detailed mechanism of GSD Ⅰ b has not been clarified.Treatment with sodium-dependent glucose transporters 2(SGLT2)inhibitors improves both neutropenia and neutrophil function through reducing 1,5-anhydroglucitol(1,5-AG)in plasma,as well as alleviates the related clinical symptoms,so SGLT2 inhibitors are increasingly used in the treatment of GSD Ⅰ b.Here it reviews the neutropenia mechanism and recent advances in animal-based research and clinical application of SGLT2 inhibitor therapy forGSD Ⅰb.
Glycogen storage disease type ⅠSodium-dependent glucose transporter 2Neutropenia
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