DEFINITION
Hereditary fructose intolerance is an inherited disorder in
which there is a lack of enzymes required for the metabolism of fructose.
CAUSE
In this disorder, the body loses enzymes (aldolase B) is
required to use fructose, the sugar contained in cane sugar (sucrose) and lots
of fruits. As a result, a byproduct of fructose accumulate in the body,
inhibiting the formation of glycogen and the conversion of glucose to use as
energy.
SYMPTOMS
Consumption of more than a small amount of fructose or sucrose
cause low blood sugar levels (hypoglycemia), with symptoms such as sweating,
confusion, and sometimes seizures, and even coma.
Children who continue to consume foods containing fructose
can damage the kidneys and liver, causing jaundice (yellow), vomiting, mental
retardation, seizures, or even death.
Chronic symptoms that can be found among other appetite
disorders, failure to thrive, digestive disorders, heart failure, and kidney
damage.
DIAGNOSIS
Early diagnosis and restrictions eaten since early infancy
can help prevent more serious disorder. Diagnosis is made when it was
discovered the enzyme missing in chemical examination of liver tissue samples.
TREATMENT
Patients should avoid foods containing fructose (mainly
found in sweet fruits), sucrose and sorbitol. Severe hypoglycemia needs to be
overcome by giving glucose through the blood vessels. Mild hypoglycemia can be
overcome by providing glucose tablets, which should always be taken by people
with hereditary fructose intolerance.
REFERENCE
- E, Chad H. Hereditary Fructose Intolerance. Medline Plus.
2011.
- Lee M Sanders. Disorders of Carbohydrate Metabolism. Merck
Manual. 2009.
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