Fructosemia Disease Causes Treatment Therapy
Fructosemia disease is an inborn error of metabolism in which eating foods containing fructose or sucrose will result in high blood fructose levels.
What Causes Fructosemia:
Fructosemia may also be called hereditary fructose intolerance, it literally means “fructose in the blood”. Fructosemia occurs as a result of hereditary lack of an enzyme called fructose-1 phosphate aldolase B. This autosomal recessive disease is rare, although some searchers upset that more people have the disorder than have diagnosed. These individuals may naturally avoid fructose after becoming ill following the consumption of fructose-containing foods.
The infant, child or adult with undiagnosed Fructosemia will be normal unless foods containing fructose, sucrose or sorbitol are eaten. If foods containing these carbohydrates are eaten, then fructose levels will increase in the patient’s blood and urine and the person will become ill. Symptoms include vomiting and low blood glucose and may progress to failure to thrive and/or coma.
The severity of the disease appears to be variable, being rather mild in some individuals and causing death in others. In severe cases, livers, kidneys, intestines may be affected, although this damage usually reverses with the elimination of dietary fructose.
Treatment of this disorder is entirely dietary. Foods containing fructose, sucrose or sorbitol must be eliminated from the diet. Fructose is often thought of as ‘fruit sugar’ but far more foods than fruits and juices must be eliminated. Honey contains fructose. Because half of the sucrose becomes fructose when sucrose is metabolised, all foods containing sucrose (sugar) must be eliminated as well. This includes all sugar, whether from cane, beets or sorghum.
Sucrose is also part of maple syrup. Sorbitol metabolism also produces fructose. So this sugar substance must be avoided as well. Some infant formulas and baby foods contain fructose and many sweetened fruit beverages do. Reading food labels and being familiar with the ingredients of the restaurant foods is imperative for those with Fructosemia Disease.
Fructosemia Perspective and Prospects:
Cases od fructosemia were first described in the mid 1950’s. Soon after, the biochemical pathway defect was discovered. Today, genetic counseling may be of benefit to those who have fructosemia and want to have children, although strict avoidance of the three carbohydrates fructose, sucrose and sorbitol will prevent symptoms.
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