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Inability to metabolize the carbohydrate fructose due to a hereditary absence or deficiency of the enzyme 1,6-biphosphate aldolase B. Clinical signs develop early in life. They include hypoglycemia, jaundice, hepatomegaly, vomiting, lethargy, irritability, and convulsions. Fructose can be identified in the urine. The fructose tolerance test should not be used because it can induce irreversible coma.
Acute attacks are treated by glucose administration. For long-term therapy, all foods containing fructose (present in sweet fruits and sugar cane) and sucrose and sorbitol (the latter used as a sweetening agent in foods and drugs) must be eliminated from the diet.
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