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Hereditary Fructose intolerance (def of aldolaseB) Q

Severe disease of Infants that appear with the ingestion of fructose containing Food


[Table sugar, fruit, sweetened cereals)


Deficiency of aldolase B causes a rapid accumulation of fructose-1- PO4 and initiates Sever toxic symptoms when exposed to fructose.


Clinical Manifestation

  1. Patients with fructose intolerance are perfectly healthy and asymptomic until fructose is ingested.
  2. Early clinical Manifestations resemble galactosenia include Jaundice, hepatomegoly vomiting, lethargy irritability and convulsions.
  3. Acute fructose ingestion produces symptomatic hypoglycemia chronic ingestion results in hepatic disease. 


  1. Reducing substance in urine.
  2. Definitive diagnosis is made by assay of aldolase B activity in the liver.
  3. Fructose tolerance tests are contraindicated because they may be followed by hypoglycemia, shock, death. 



Complete elimination of cell sources of sucrose and fructose from diet.

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