Glucose 6 Phosphatase & Lactic Acidosis

Overview

Your liver and muscles contain glycogen, which is the storage form of glucose, or blood sugar, that your body uses for energy. Glucose-6-phosphatase is an enzyme that's needed for you to convert glycogen to glucose and release it into the blood. Some people have a glycogen storage disease that causes a build-up of lactic acid to occur as the body tries to raise blood sugar.

Glycogenolysis

Your muscles and liver convert glycogen to glucose by a process called glycogenolysis. The liver sends glucose into the blood to be transported to the brain and other organs during times of starvation, while the muscles convert glycogen to glucose for their own use during exercise and physical activity. The processes are slightly different because the liver requires the help of an enzyme called glucose-6-phosphatase.

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Von Gierke Disease

Von Gierke disease, or Type I glycogen storage disease, is a genetic disorder in which the body can't undergo glycogenolysis in the liver due to a deficiency of glucose-6-phosphatase. Glycogen builds up in the liver, but blood sugar levels drop resulting in hypoglycemia, hunger, fatigue and irritability in infants who have the disease. Glycogen builds up in the liver and causes it to swell. The kidneys may become enlarged as well.

Lactic Acidosis

Lactic acid builds up in the blood as the body attempts to increase blood sugar levels. The liver normally breaks down the lactic acid, but it cannot keep up and a condition called lactic acidosis occurs. Lactic acid levels in the blood can be measured in lab and a doctor may order this test along with blood glucose, triglycerides and uric acid, along with a biopsy of the liver, if Von Gierke disease is suspected.

Treatment

Von Gierke disease is treated by avoiding low blood sugar with frequently carbohydrate feedings throughout the day. A feeding tube that goes through the nose into the stomach may provide additional sugars during the night. Medications may also be given to reduce uric acid levels, blood fat levels and to help bolster the immune system, which can also be damaged. A medical doctor must examine the child and make the diagnosis.

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