Hereditary Fructose Intolerance

What is hereditary fructose intolerance?

Hereditary fructose intolerance is a serious condition that prevents the body from breaking down the sugars that are found in fruits, called fructose, as well as some other forms of sugar. The condition is caused by the body not having enough of a specific enzyme called aldolase B. Hereditary fructose intolerance typically affects infants when they start eating foods containing fruit. Early symptoms of this condition can include vomiting and slower than normal growth. If not treated, babies can develop seizures and liver and kidney problems from repeatedly eating sugar-containing foods.

How common is hereditary fructose intolerance?

About 1 in 20,000 babies is born with hereditary fructose intolerance worldwide each year.

What can be done for hereditary fructose intolerance?

Hereditary fructose intolerance can be treated with diet. People with this condition are instructed by their doctors to avoid eating or being treated with medications that contain fructose or sucrose, or products made from them. These include high-fructose corn syrup, sorbitol, sucralose, polysorbate, honey, agave syrup, inverted sugar, maple-flavored syrup, molasses, palm or coconut sugar, and sorghum.

How is hereditary fructose intolerance inherited?

Hereditary fructose intolerance is caused by changes in the ALDOB gene. It is a recessive inherited condition, which means that a person must inherit two altered copies of the ALDOB gene, one from each parent, in order to have the disease. People who have only one copy of the altered ALDOB gene are called carriers, and are not expected to have symptoms of the condition. If a baby is identified as a carrier for hereditary fructose intolerance through Eli screening, genetic counseling and additional testing in the parents is recommended.


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