Understanding Von Gierke disease: Causes, Symptoms, and Management

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Von Gierke disease

by Alike Medical Team ∙ Updated on June 13, 2023

Additional names

This group contains additional names: - Type I glycogen storage disease - GSD I

General

Von Gierke disease is a condition in which the body cannot break down glycogen. Glycogen is a form of sugar (glucose) that is stored in the liver and muscles. It is normally broken down into glucose to give you more energy when you need it.

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Signs & symptoms

These are symptoms of von Gierke disease: * Constant hunger and need to eat often * Easy bruising and nosebleeds * Fatigue * Irritability * Puffy cheeks, thin chest and limbs, and swollen belly

Diagnosis

The doctor will perform a physical exam. The exam may show signs of: * Delayed puberty * Enlarged liver * Gout * Inflammatory bowel disease * Liver tumors * Severe low blood sugar * Stunted growth or failure to grow * Children with this condition are usually diagnosed before age 1. Tests that may be done include: * Biopsy of liver or kidney * Blood sugar test * Genetic testing * Lactic acid blood test * Triglyceride level * Uric acid blood test If a person has this disease, test results will show low blood sugar and high levels of lactate (produced from lactic acid), blood fats (lipids), and uric acid.

Treatment

The goal of treatment is to avoid low blood sugar. Eat frequently during the day, especially foods that contain carbohydrates (starches). Older children and adults may take cornstarch by mouth to increase their carbohydrate intake. In some children, a feeding tube is placed through their nose into the stomach throughout the night to provide sugars or uncooked cornstarch. The tube can be taken out each morning. Alternatively, a gastrostomy tube (G-tube) can be placed to deliver food directly to the stomach overnight. A medicine to lower uric acid in the blood and decrease the risk for gout may be prescribed. Your provider may also prescribe medicines to treat kidney disease, high lipids, and to increase the cells that fight infection. People with von Gierke disease cannot properly break down fruit or milk sugar. It is best to avoid these products.

Note

☝ We provide information on prescription and over-the-counter medicines, diagnosis, procedures and lab tests. This material is provided for educational purposes only and is not medical advice, diagnosis or treatment.

National Institutes of Health ∙ World Health Organization ∙ MedlinePluse ∙ Centers for Disease Control and Prevention

☝ All information has been reviewed by certified physicians from Alike

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