Uric acid metabolism in therapy of glycogen storage disease type I.

Abstract

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.

• Authors: P J Benke, S Gold
• Journal: Pediatric research (Pediatr Res) Vol. 12 Issue 3 Pg. 204-6 (Mar 1978) ISSN: 0031-3998 [Print] United States
• PMID: 273863 (Publication Type: Journal Article, Research Support, U.S. Gov't, P.H.S.)

Chemical References

• Dietary Carbohydrates
• Uric Acid
• Glycine

Topics

• Child
• Dietary Carbohydrates (administration & dosage, pharmacology, therapeutic use)
• Glycine (metabolism)
• Glycogen Storage Disease Type I (diet therapy, metabolism)
• Humans
• Intubation, Gastrointestinal
• Male
• Uric Acid (metabolism)