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Galactosemia
Overview Food Sources Recommendations Side Effects
Inborn errors of metabolism
Alternative Names: Galactosemia - nutritional considerations; Fructose intolerance - nutritional considerations; Maple sugar urine disease (MSUD) - nutritional considerations; Phenylketonuria (PKU) - nutritional considerations; Branched chain ketoaciduria - nutritional considerations Recommendations: Inborn errors of metabolism often demand diet changes. The type and extent of the changes depends on the specific metabolic error. Registered dietitians and physicians can help with the diet modifications needed for each disease. Text Continues Below The following are some examples of inborn errors of metabolism. FRUCTOSE INTOLERANCE Fructose intolerance is a genetic disorder involving the breakdown of the carbohydrate fructose. It is potentially life-threatening but may be treated by diet changes. Food sources of fructose include fruits, fruit juices, sucrose (all sugars -- cane, beet, white, brown), corn syrups, honey, sorbitol, levulose, invert sugar, and some vegetable and starches. All fructose should be avoided in the diet. The severity of the restriction depends on individual tolerance. Sugar and fructose are found in many foods, making the diet difficult to follow. GALACTOSEMIA Galactosemia is a genetic disorder involving the breakdown of the carbohydrate galactose to glucose. It can result in cataracts, enlarged liver, enlarged spleen and mental retardation. Typically, the disease is found in milk-fed infants shortly after birth. This is because milk contains large amounts of galactose. Page:  1 | 2 | Next >>