Hereditary fructose intolerance

Understanding the condition

Hereditary fructose intolerance (HFI) is a rare genetic disorder caused by a deficiency of the enzyme aldolase B, essential for metabolizing fructose in the liver. This enzyme deficiency leads to the accumulation of toxic byproducts when fructose, sucrose, or sorbitol is consumed. Unlike fructose malabsorption, which primarily causes digestive discomfort, HFI can result in severe complications like hypoglycemia, liver damage, and kidney issues.

The symptoms of HFI often manifest in infancy when fruits, sweetened beverages, or formulas containing fructose or sucrose are introduced. Common signs include nausea, vomiting, abdominal pain, and failure to thrive. In severe cases, prolonged exposure to fructose can lead to lethargy, jaundice, and even seizures.

Is hereditary fructose intolerance treatment possible?

The simple answer is no, you can not cure HFI. However hereditary fructose intolerance treatment involves strict dietary management to avoid foods containing fructose, sucrose, and sorbitol. Careful label reading is critical, as fructose is hidden in various processed foods and sweeteners. Substituting high-fructose foods with safe alternatives like glucose-based sweeteners ensures adequate caloric intake without triggering symptoms.

For those managing hereditary fructose intolerance, collaborating with a registered dietitian is invaluable. They can help create a balanced diet plan that meets nutritional needs while avoiding harmful ingredients. Regular monitoring and education are essential for preventing accidental exposure and ensuring long-term health.

With the right hereditary fructose intolerance treatment approach, individuals can effectively manage this condition, maintaining a safe and healthy lifestyle.

Professional Guidance:

You can read more about the topic in our knowledge bank or you can contact one of our dieticians for personalized advice. If you’re facing symptoms and don’t know where they come from, you can take the intolerance test for more information:

*These statements have not been evaluated by the Food and Drug Administration. This product is not intended to diagnose, treat, cure, or prevent any disease.