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Tarui disease (GSD VII) is caused by an inherited deficiency of muscle phosphofructokinase (PFK) and manifests with the combination of myopathy and hemolysis.
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It is also possible that the difference between these calculated intakes is explained by normal day-to-day variation in food intake since the food record periods were short (3 days), just before the exercise test at 8 months the patient returned to ketosis, and in the exercise test, a new increase in ammonia was detected. This suggests that even a short period of more carbohydrates may increase the ammonia level during the exercise test (Figure 2) highlighting the importance of carbohydrates in the high ammonia levels associated with Tarui disease.Tarui Sex Escort
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