Fructose is in many healthy foods, but for someone with a hereditary fructose intolerance, eating these foods could be dangerous. Most people with hereditary fructose intolerance are diagnosed in childhood. With genetic testing, a pregnant woman might even be able to find out if her baby has this problem before birth. Fortunately, this rare disorder can typically be treated by changes in diet, although a fructose-free lifestyle can be challenging to stick with.
Fructose
Fructose is a type of simple sugar found in foods. Fruits are the most well known source of fructose, but this sugar is also a component of sucrose, also known as table sugar. High fructose corn syrup, which is commonly added to commercially produced foods and drinks, also contains fructose. In the body, fructose is broken down into the simpler sugar glucose, which the body uses to fuel all cellular activity.
Hereditary Intolerance
People with the genetic disorder hereditary fructose intolerance cannot properly metabolize fructose. These people lack the enzyme aldolase B, which is used to digest fructose. This disorder is autosomal recessive, which means that both parents must pass on one copy of the gene for the child to have it. People can be carriers if they have only one copy of the gene, which they then have a 50-percent chance of passing on to their children. If someone with a hereditary fructose intolerance eats fructose or sucrose, blood sugar levels can drop to dangerous levels and toxic byproducts can build up in the liver.
Symptoms
Symptoms of hereditary fructose intolerance typically appear shortly after a baby begins eating formula or solid food. Breast milk does not contain fructose or sucrose, so exclusively breastfed babies will not show symptoms until they begin eating other foods that do contain these sugars. Symptoms include jaundice, irritability, excessive sleepiness, vomiting, convulsions and problems eating. Over the long-term, severe liver disease can develop if fructose is not eliminated from the diet.
Treatment
The basic treatment for this disorder is to remove all sources of fructose and sucrose from the diet. This includes avoiding all processed foods made with high fructose corn syrup, table sugar and honey. People with hereditary fructose intolerance can consume foods made with artificial sweeteners such as aspartame, saccharine or stevia. Someone on a fructose-free diet might need to take vitamin supplements to get some of the nutrients normally found in fruit.
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