Congenital or hereditary fructose intolerance is an autosomal recessive disorder in which fructose metabolism is impaired due to a deficiency of the enzyme fructose-1-phosphate adolase. Because of this deficiency, fructose-1-phosphate accumulates in the kidneys, liver and small intestines, making it harder for the body to use and make glucose. This results in hypoglycemia every time fructose is ingested.
Symptoms
Symptoms of congenital fructose intolerance include severe abdominal pain, vomiting and hypoglycemia after eating foods with fructose or with sugars that get converted into fructose during digestion. Prolonged intake of fructose can cause liver and kidney failure. Also, patients can develop a strong aversion to sweet foods. However, symptoms can be relieved by following a fructose-free diet, which will also help prevent any recurring symptoms.
Foods Containing Fructose
Fructose is naturally found in fruits, fruit juices, honey and some syrups. It is also found as a component of table sugar or in commercially processed foods that contain granulated fructose or high-fructose corn syrup. Other foods that have fructose and should be limited or avoided are foods containing confectioner's or powdered sugar, regular sodas, flavored water, sports drinks, sweetened milk or foods with sorbitol, a sugar alcohol that gets converted to fructose during the process of normal digestion.
What to Eat
Because you will be avoiding certain foods, it is very important that you maintain adequate nutrient and calorie intake. Foods that you can eat include milk, cheese, and other milk products without added fruit or flavoring; meat, poultry, fish or eggs prepared without added breading; gluten-free breads and crackers, which contain little to no fructose; and vegetables such as artichokes, beets, squash, carrots and celery. As for fruits, those that also contain little fructose and have been shown to be well tolerated are berries, citrus fruits, stone fruits, or bananas, kiwis and pineapples.
Other Tips/Guidelines
Try to limit your portions, as smaller portions will logically contain less fructose. Also, you might want to keep a food/symptoms journal so that you can monitor which foods are well tolerated and which ones worsen your symptoms. This will help you avoid them in the future. Finally, try to eat five to six smaller meals throughout the day instead of two or three larger ones. This will help your body with digestion and prevent it from having to deal with too much fructose at one time.
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