Living With Hereditary Fructose Intolerance
October 7, 2009
Fructose is a form of sugar found naturally in fruits, vegetables and honey. Hereditary Fructose Intolerance is a condition that inhibits proper metabolism of fructose. This is a rare genetic condition. It is estimated that 1 in 20,000 might suffer from this condition.
Deficiency of the aldolase B enzyme is responsible for this condition. Hereditary fructose intolerance should not be confused with fructose malabsorption, or normal fructose intolerance. In fructose malabsorption, the small intestine could not absorb fructose from the foods. However, both hereditary fructose intolerance and fructose malabsorption share the same symptoms.
Hereditary fructose intolerance causes
After the small intestine absorbs fructose from the foods, the enzymes secreted by the liver breaks the fructose to form glucose. Two enzymes known as fructose-1-phosphate aldolase and aldolase B are required to accomplish the entire fructose metabolism process.
At first, the fructose-1-phosphate aldolase enzyme converts fructose into fructose-1-phosphate. Finally, the partially metabolized fructose is converted into glucose with the help of the aldolase B enzyme. Deficiency or absence of aldolase B causes incomplete metabolism of fructose.
Consequently, fructose-1-phosphate collects in the liver, intestine and kidneys. This obstructs production of glucose, leading to hypoglycemia or low blood sugar level after fructose intake.
Symptoms of hereditary fructose intolerance
Common symptoms of hereditary fructose intolerance include abdominal pain, vomiting, irritability, excessive sleeping, convulsions and decline in blood sugar level following consumption of fructose. If people suffering from hereditary fructose intolerance continues taking fructose for a prolonged period, kidney and liver damage might occur.
Hereditary fructose intolerance remedies
Hereditary fructose intolerance is a genetic condition. It could not be treated with medication. A diet free from fructose could only help to prevent damages caused by hereditary fructose intolerance. Besides fructose, people suffering from this rare genetic condition should also avoid consumption of sorbitol.
People with hereditary fructose intolerance should also stay away from sucrose, found in sugar, cane sugar and beet sugar. Honey, fruit syrup, maple syrup, high fructose corn syrup and invert syrup are harmful for people with hereditary fructose intolerance.
Fruits such as apples, apricots, grapes, peaches, plumps, raisins, strawberries, raspberries, gooseberries and bananas are not safe for people suffering from this condition. They should also avoid beans.
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