关键词: I型糖原累积症；饮食管理；现状调查

Abstract: Objective: To investigate the status quo of dietary management of children with GSDI. Methods: A convenient sample of 76 children with GSDI aged 3-18 years and their parents from pediatric outpatients of a Beijing tertiary grade A hospital were investigated with a self-designed dietary management questionnaire. Results: Totally 10 children (13.2%) had consulted a dietitian, 7 (9.2%) had strictly followed the nutrition prescription, and 65 (85.5%) could follow the basic principles of dietary control. The total intake of three meals, the intake of staple food, and the intake of other kinds of food which could be reasonably provided according to the nutrition prescription and the condition of blood glucose, accounted for 9.2%, 19.7% and 7.9% respectively. Only 11 (14.5%) children could always or often choose coarse grains, and 4 (5.3%) could always or often regard high-starch food as the staple food. Conclusion: There are many malnutrition problems in children with GSD I, which are related to their poor dietary management. Most of the children do not control their diet or only follow the dietary principles, and the collocation of three meals can not meet their energy and nutrient requirements. In the future, medical staff should provide more targeted and specific dietary guidance.

Key words: Glycogen Storage Disease Type I; dietary management; status quo