The nutritional management of hepatic glycogen storage diseases / 中华实用儿科临床杂志

ABSTRACT

Hepatic glycogen storage diseases (GSDs) are a group of rare inherited disorders of glycogen metabolism.Currently, since there is no specific treatment, nutritional therapy becomes the most effective way to reduce and relieve clinical symptoms.Although all types of hepatic GSDs are mainly treated with raw cornstarch, for different subtypes of hepatic GSDs, specific treatment strategies and management are different, so as to the key issues concerned during the follow-up.Therefore, attention should be paid to the long-term nutritional management of patients with different types of hepatic GSDs.