Von Gierke disease

Von Gierke disease is actually a group of inherited metabolic disorders which all involve glycogen storage. Glycogen is converted to glucose to fuel muscles, and so problems with its metabolism affect the whole body. The diseases are inherited, through recessive characteristics. Therefore both parents must be carriers for their offspring to have a chance of having these problems.

Symptoms:

• hypoglycemia
• A thin chest and limbs and a protruberant belly Need for frequent feeding
• Stunted growth
• Delayed puberty
• Enlarged liver
• Gout
• Easy bruising
• nose bleeds

Much like diabetes, another disease in which blood sugar levels are an issue the sufferer must follow a severely restricted diet. Feeding is necessary during the night. Allopurinol can be used to control gout, as it lowers the levels of uric acid in the blood.

Any of a group of closely-related congenital diseases caused by a metabolic error in which the body deposits an abnormally high amount of glycogen in the kidneys and liver.

The disease produces symptoms such as hypoglycemia (low blood sugar) and hyperlipemia (an excess of lipid molecules in the blood, which can lead to problems such as clogged arteries and heart attacks). These disease are also known as "glycogen storage disease" and "glycogenosis".

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From the BioTech Dictionary at http://biotech.icmb.utexas.edu/. For further information see the BioTech homenode.