Benke P J, Gold S
Pediatr Res. 1978 Mar;12(3):204-6. doi: 10.1203/00006450-197803000-00008.
Factors which may explain lower serum uric in a new therapy of patients with glycogen storage disease (GSD) type I have been studied. [1-14C]Glycine incorporation into urine uric acid was 0.68% of the injected dose during a 6-day period of frequent high carbohydrate feedings, 0.40% with the same diet and nocturnal nasogastric feeding by Vivonex, and 0.18% in a control patient with GSD type III. Fractional renal uric acid excretion in the patient with GSD type I increased from 11.3% to 26.3% after beginning nocturnal nasogastric feeding of Vivonex. Red cell phosphoribosylpyrophosphate leve,ls were not changed by the therapy. Addition of Vivonex nocturnal feedings to frequent high carbohydrate feedings (1) decreased the accelerated de novo purine synthesis to a level still higher than control and (2) increased fractional renal uric acid excretion.
对可能解释糖原贮积病（GSD）I型患者新疗法中血清尿酸水平较低的因素进行了研究。在频繁高碳水化合物喂养的6天期间，[1-14C]甘氨酸掺入尿尿酸的量为注射剂量的0.68%；采用相同饮食并通过Vivonex进行夜间鼻饲时，该比例为0.40%；在一名GSD III型对照患者中为0.18%。GSD I型患者开始夜间鼻饲Vivonex后，肾尿酸排泄分数从11.3%增加到26.3%。该疗法未改变红细胞磷酸核糖焦磷酸水平。在频繁高碳水化合物喂养基础上增加Vivonex夜间喂养，（1）将加速的嘌呤从头合成降低至仍高于对照的水平，（2）增加了肾尿酸排泄分数。
