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

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

Physical examination may show:

• Enlarged liver and spleen (hepatosplenomegaly)
• Yellow skin or eyes

Tests that confirm the diagnosis include:

• Blood clotting tests
• Blood sugar test
• Enzyme studies
• Genetic testing
• Kidney function tests
• Liver function tests
• Liver biopsy
• Uric acid blood test
• Urinalysis

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

Previous Section

Hereditary fructose intolerance Overview

Next Section

Hereditary fructose intolerance Treatment

Review Date: 04/15/2009
Reviewed By: 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.

A.D.A.M., Inc. is accredited by URAC, also known as the American Accreditation HealthCare Commission (www.urac.org)