Fructose intolerance is caused by deficiency of the enzyme fructose-1-phosphate aldolase. Inherited as an autosomal recessive condition, it manifests with symptoms of hypoglycaemia after the ingestion of fructose, the result of accumulation of fructose-1-phosphate.
Diagnosis is confirmed by the measurement of the enzyme in the liver.
Treatment is with a fructose-free diet, which if adhered to, is concordant with a good prognosis.
Last reviewed 01/2018