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With early diagnosis, fructose intolerance can be successfully treated by eliminating fructose, sucrose, and sorbitol from the diet (less than 40 mg/kg per day). Sorbitol is an artificial sweetener found in many sugar-free products, such as sugarless gum or diet foods. Patients usually respond favorably within a few weeks and can make a complete recovery if fructose-containing foods are avoided. Early recognition and treatment of the disorder is important to avoid damage to the liver, kidneys, and small intestine.
Early symptoms of hypoglycemia can be treated with oral glucose tablets or gel, available at most pharmacies. The doctor can provide more information about how to manage a hypoglycemic reaction, as well as how to monitor the child's blood glucose levels using a blood glucose meter to prevent a hypoglycemic reaction. Severe hypoglycemia should be treated with a glucagon injection to increase the blood glucose level. In some cases, the child may need an intravenous glucose solution, given in the hospital.
Children with this condition should be managed by a medical specialist in biochemical genetics or metabolism, as well as a registered dietitian who can provide nutrition support and information.
To prevent complications from this disorder, parents should take the following steps:
There is no cure for hereditary fructose intolerance, since the enzyme needed to process fructose is missing at birth. The prognosis depends on how soon the diagnosis is made and how soon fructose and sucrose are eliminated from the child's diet. If the condition is not recognized and the diet is not well controlled, death can occur in infants or young children. With a well-controlled diet, the child will thrive and develop normally. In the absence of liver damage, the child's life expectancy is normal. Most of the damaging effects of the disorder can be prevented by strictly following the fructose-free diet.
Carriers of the gene for hereditary fructose intolerance can be identified through DNA analysis. Anyone who is known to carry the disorder or who has the disorder in his or her family may benefit from genetic counseling. At-risk individuals can be assisted with family planning and reproductive decisions.
Author Info: Altha Roberts Edgren, Angela M. Costello, Thomson Gale, Gale, Detroit, Gale Encyclopedia of Children's Health, 2006This feature is for informational purposes only and should not be used to replace the care and information received from your healthcare provider. Please consult a healthcare professional with any health concerns you may have.
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