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本文观察了61例患有Ⅰ型和Ⅲ型糖原贮积病患儿,男44例,女17例。年龄在1～19岁,平均年龄7.8±5.4岁。均有肝脏肿大,其中大部分生长发育迟缓、智力低下。实验室检查空腹血糖低于正常,血甘油三酯、胆固醇升高并有酸中毒症状。因系先天代谢性疾病,目前饮食治疗是主要措施,我们采用合理控制能量(?g/kg)、适量蛋白(?g/kg)、低脂肪,(?g/kg),低嘌呤(<? mg/d)、免果糖及半乳糖以及少用单双糖类的膳食。在上述基础上采用玉米淀粉1.75～2.2克/(千克·次)作为加餐,每隔6h 服用1次,以维持机体葡萄糖内环境的稳定。治疗结果提示此种饮食疗法对Ⅰ型及Ⅲ型息儿有较好疗效。
This article observed 61 patients with type Ⅰ and Ⅲ glycogen storage disease, 44 males and 17 females. The age of 1 to 19 years old, the average age of 7.8 ± 5.4 years. Have liver enlargement, most of them growth retardation, mental retardation. Laboratory tests lower than normal fasting blood glucose, triglycerides, elevated cholesterol and acidosis symptoms. Due to the congenital metabolic diseases, the current diet is the main measure, we use reasonable control of energy (? G / kg), the amount of protein (? G / kg), low fat, (? G / kg), low purine mg / d), free fructose and galactose, and the use of single and double carbohydrate diet. On the basis of the above using corn starch 1.75 ~ 2.2 g / (kg · times) as a meal, taken once every 6h, in order to maintain the stability of the body’s glucose environment. Treatment results suggest that this diet therapy for type I and type III interest in children have a good effect.