Definition:
Fructose intolerance is also known as fructose poisoning which is a condition that can be inherited. This disorder is marked by a liver enzymes deficiencies specifically those responsible to metabolize fructose. Another name for the disorder is hereditary fructosemia.
Diagnosis:
Fructose can be diagnosed through a number of evaluative tools. An enzymatic assay can be done in order to check for the aldolase activity; a fructose tolerance test can be performed where the patient is injected intravenously with fructose and the levels of his or her fructose, glucose and phosphate are monitored. A non-invasive DNA testing can be done but negative results are not conclusive as well as a hydrogen breath test.
Treatment:
Treatment includes a change of diet. Fructose and sucrose has to be eliminated from the diet so that symptoms and the underlying disorder will not occur. If a fructose and sucrose restricted diet is adhered by the individual, he or she will have a good prognosis.
Symptoms and Signs:
Symptoms for the disorder appear when fructose is ingested by the individual. Symptoms include hypoglycemia, vomiting, hemorrhage, jaundice, hyperuricemia, hepatomegaly, and later on kidney failure will soon develop.
Causes:
The liver enzyme deficient in fructose intolerance is aldolase-B which is responsible for converting fructose-1-phospate to glyceraldehydes and DHAP. When there is a deficiency in aldolase-B fructose cannot be metabolized any further and therefore traps phosphates. If phosphates are not released, thus stunting the process of forming glucose and causing hypoglycemia in the individual.
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