Hereditary fructose intolerance

Injury	Disease	Nutrition	Poison

Symptoms	Surgery	Test	Special Topic

Hereditary fructose intolerance

Alternative Names:

Fructosemia; Fructose intolerance; Fructose aldolase B-deficiency
Treatment:

Complete elimination of fructose and sucrose from the diet is an effective treatment for most patients. Treatment of individual complications follows mainstream medical guidelines. For example, some patients can take medication to lower the level of uric acid in their blood and thereby decrease their risk for gout.

Expectations (prognosis):

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Absolute elimination of fructose and glucose produces good results in most children with fructose intolerance. A few children will go on to develop progressive liver disease. The prognosis depends on how soon the diagnosis is made and how soon fructose and sucrose can be eliminated from the baby's diet.

Complications:

Hypoglycemia
Illness due to eating fructose- or sucrose-containing foods
Strong avoidance of fructose-containing foods due to noxious effects
Seizures
Bleeding
Gout
Liver failure
Death

Calling your health care provider:

Call your health care provider if your child developed the above symptoms after you began feeding the child formula or solid food. The care of a medical specialist in biochemical genetics or metabolism is strongly recommended if your child has this disorder.

Preliminary evidence suggests that parents of a child with this disorder (as well as other carriers of the mutant gene) may be at increased risk for gout (arthritis caused by excess uric acid in the body and uric acid crystals in the joints). Therefore, it is important to notify your physician if you have a family history of hereditary fructose intolerance.