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Hereditary fructose intolerance - Symptom

Alternative Names

Fructosemia; Fructose intolerance; Fructose aldolase B-deficiency; Fructose 1, 6 bisphosphate aldolase deficiency

Symptoms:

Symptoms can be seen after a baby starts eating food or formula.

The early symptoms of fructose intolerance are similar to those of galactosemia. Later symptoms relate more to liver disease.

Symptoms may include:

Convulsions
Excessive sleepiness
Irritability
Jaundice
Poor feeding as a baby
Problems after eating fruits and fructose/sucrose-containing foods
Vomiting

Signs and tests:

Physical examination may show:

Enlarged liver and spleen (hepatosplenomegaly)
Yellow skin or eyes

Tests that confirm the diagnosis include:

Blood sugar will be low, especially after receiving fructose or sucrose. Uric acid levels will be high.

Reviewed last on: 4/15/2009
Chad Haldeman-Englert, MD, Division of Human Genetics, Children's Hospital of Philadelphia, Philadelphia, PA. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

References

Steinmann B, Santer R, van den Berghe G. Disorders of Fructose Metabolism. In: Fernandes J, Saudubray JM, van den Berghe G, Walter JH, eds. Inborn Metabolic Diseases. 4th ed. New York, NY:Springer;2006:chap 9.

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