Hereditary fructose intolerance
Alternate Names : Fructosemia, Fructose intolerance, Fructose aldolase B-deficiency, Fructose 1, 6 bisphosphate aldolase deficiency
Treatment
Removing fructose and sucrose from the diet is an effective treatment for most patients. Complications are treated. For example, some patients can take medication to lower the level of uric acid in their blood and decrease their risk for gout.
Prognosis (Expectations)
Hereditary fructose intolerance may be mild or severe.
Avoiding fructose and sucrose helps most children with this condition. A few children with a severe form of the disease will develop severe liver disease. Even removing fructose and sucrose from the diet may not prevent severe liver disease in these children. How well a person does depends on: - How soon the diagnosis is made
- How soon fructose and sucrose can be removed from the diet
- How well the enzyme works in the body
Complications
- Avoidance of fructose-containing foods due to their effects
- Bleeding
- Death
- Gout
- Illness from eating foods containing fructose or sucrose
- Liver failure
- Low blood sugar (hypoglycemia)
- Seizures
Calling Your Health Care Provider
Call your health care provider if your child develops symptoms of this condition after feeding starts. If your child has this condition, experts recommend seeing a doctor who specializes in biochemical genetics or metabolism.
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