Abstract
Excess purine degradation in exercising muscle due to impaired glycolysis causes hyperuricemia in muscle glycogenoses (myogenic hyperuricemia). However, hyperuricemia has been seen more frequently in Type VII than in Type V. This study was designed to clarify a metabolic basis for the different frequency of hyperuricemia between the diseases. 5 patients (2 males, 3 females) with Type V and 4 (3 males, 1 female) with Type VII participated in the study. 1) In every patient, semi ischemic forearm exercise caused no increase in lactate but exaggerated increases in ammonia, inosine and hypoxanthine in cubital venous blood. The ammonia increase was not different between Type V and Type VII, but it was greater in male than female patients. 2) Semiischemic forearm exercise performed after glucagon injection showed that lactate production was induced by exercise in Type V but not in Type VII. 3) Saline or glucose solution was infused in a patient with Type V during exercise on a bicycle ergometer. With saline infusion, ammonia and hypoxanthine in systemic circulation increased greatly after exercise. Conversely, with glucose infusion there were no increases in these metabolites. These findings indicated that blood glucose can be available as metabolic fuel through muscle glycolysis in Type V but not in Type VII. Thus, muscle purine degradation may be accelerated more in Type VII than in Type V, leading to a higher frequency of hyperuricemia.
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Mineo, I., Hara, N., Kono, N. et al. 88 MYOGENIC HYPERURICEMIA: A COMPARATIVE STUDY BETWEEN TYPE V AND TYPE VII GLYOGENOSIS. Pediatr Res 24, 125 (1988). https://doi.org/10.1203/00006450-198807000-00112
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DOI: https://doi.org/10.1203/00006450-198807000-00112