Von Gierke Disease (Type I) - A Comprehensive Review in Question and Answer Format

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Question: What is Von Gierke disease?
Answer: Von Gierke disease (type I) is a condition characterized by severe fasting hypoglycemia and the accumulation of glycogen in the liver and kidneys.Question: What are the symptoms of Von Gierke disease?
Answer: The symptoms of Von Gierke disease (type I) include fasting hypoglycemia, elevated blood lactate, triglycerides, uric acid (gout), and hepatomegaly or renomegaly. The liver does not regulate blood glucose.Question: What is the deficient enzyme in Von Gierke disease?
Answer: The deficient enzyme in Von Gierke disease (type I) is glucose-6-phosphatase.Question: What is the treatment for Von Gierke disease?
Answer: The treatment for Von Gierke disease (type I) involves frequent oral glucose/cornstarch, avoidance of fructose and galactose. The condition is also characterized by impaired gluconeogenesis and glycogenolysis. Question: What is the hallmark of Von Gierke disease?
Answer: The hallmark of Von Gierke disease (type I) is severe fasting hypoglycemia.Question: What are the other findings of Von Gierke disease (type I)?
Answer: Other findings of Von Gierke disease (type I) include accumulation of glycogen in the liver and kidneys, elevated blood lactate, triglycerides, uric acid (gout), and hepatomegaly or renomegaly.Question: Why does the liver not regulate blood glucose in Von Gierke disease (type I)?
Answer: The liver does not regulate blood glucose in Von Gierke disease (type I) due to the deficiency of glucose-6-phosphatase.Question: What should be avoided in the treatment of Von Gierke disease (type I)?
Answer: Fructose and galactose should be avoided in the treatment of Von Gierke disease (type I). Question: What are the consequences of impaired gluconeogenesis and glycogenolysis in Von Gierke disease (type I)?
Answer: Impaired gluconeogenesis and glycogenolysis in Von Gierke disease (type I) contribute to the severe fasting hypoglycemia and inability to regulate blood glucose levels.Question: What is the role of oral glucose/cornstarch in the treatment of Von Gierke disease (type I)?
Answer: Oral glucose/cornstarch is a crucial part of the treatment for Von Gierke disease (type I) as it helps to regulate blood glucose levels and prevent hypoglycemia.Question: How does Von Gierke disease (type I) differ from other types of Von Gierke disease?
Answer: Von Gierke disease (type I) is characterized by severe fasting hypoglycemia, glycogen accumulation in liver and kidneys, and deficiency of glucose-6-phosphatase. Other types of Von Gierke disease may have different symptoms and characteristics.Question: Is Von Gierke disease a common condition?
Answer: The frequency of Von Gierke disease is not well documented, but it is considered a rare condition.tab