About The Medical Condition Von Gierke Disease
Find the definition of the medical term Von Gierke Disease. Von Gierke Disease defined and explained for easy understanding at the Medical Conditions from ClusterMed.info.
Von Gierke Disease
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What's The Definition Of The Medical Condition Von Gierke Disease?
An autosomal recessive disease in which gene expression of glucose-6-phosphatase is absent, resulting in hypoglycemia due to lack of glucose production. Accumulation of glycogen in liver and kidney leads to organomegaly, particularly massive hepatomegaly. Increased concentrations of lactic acid and hyperlipidemia appear in the plasma. Clinical gout often appears in early childhood.
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