Fanconi Bickel syndrome

Management of Fanconi Bickel syndrome (FBS) generally focuses on treating the signs and symptoms of the condition. For treatment of the symptoms of kidney disease, a doctor may focus on replacing the water and electrolytes that are lost from the kidneys. Additionally, vitamin D and phosphate supplements can help prevent bone weakening (rickets).^[1] 

It is recommended that people with Fanconi Bickel syndrome follow a galactose-restricted diet. Galactose is a substance that is broken down into glucose. Because people with Fanconi Bickel syndrome have trouble moving glucose throughout the body, limiting galactose prevents the buildup of glucose and glycogen in the liver and kidneys. Galactose is found in food such as milk, cheese, yogurt, and legumes. People with Fanconi Bickel syndrome should primarily eat fructose as their main carbohydrate. In addition, people with Fanconi Bickel syndrome should eat small, frequent meals in order to avoid developing low blood sugar between meals. Finally, cornstarch may be used as a substitute to provide necessary sugars to the body.^[1]