Hereditary Fructose Intolerance

Hereditary Fructose Intolerance (HFI) is an inherited metabolic disorder in which the body cannot properly metabolize fructose (fruit sugar), sucrose (table sugar), or sorbitol. It is caused by deficiency of the enzyme aldolase B in the liver, kidneys, and small intestine. Unlike the more common and benign fructose malabsorption, HFI is a serious condition requiring strict dietary management.

When individuals with HFI consume fructose or related sugars, a toxic intermediate (fructose-1-phosphate) accumulates in the body. This blocks the liver's ability to release glucose into the blood and to make new glucose, leading to severe hypoglycemia (low blood sugar). Continued exposure damages the liver and kidneys.

Symptoms typically appear when infants are first introduced to fruits, juices, or formula containing fructose. Signs include nausea, vomiting, abdominal pain, excessive sweating, tremors, confusion, and sometimes seizures after eating fructose-containing foods. If the condition is not recognized and fructose intake continues, it can lead to liver and kidney damage, failure to thrive, and potentially death.

HFI is caused by mutations in the ALDOB gene and is inherited in an autosomal recessive pattern. The estimated prevalence is 1 in 20,000-30,000 people. Treatment is a strict fructose-free, sucrose-free, and sorbitol-free diet. With proper dietary management, individuals with HFI can lead healthy lives and may even naturally develop an aversion to sweet foods.

NutraHacker examines the following gene related to Hereditary Fructose Intolerance:

• ALDOB

For more information about your own genetic profile as related to Hereditary Fructose Intolerance, please check out our NutraHacker Carrier Status and Drug Response Report.

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