HFI - Hereditary Fructose Intolerance
Nov 11, 2013 17:38:07 GMT
Post by Charlie Arnold on Nov 11, 2013 17:38:07 GMT
When reactions occur to fructose (or sugars generally) in children, especially if symptoms appear as soon as you start weaning then HFI must be eliminated first as this can become very serious.
If symptoms occur later in life then HFI is unlikely to be the cause of symptoms but not unheard of as the patient may have naturally avoided fructose containing foods and just not known why they feel unwell after eating certain foods.
Hereditary fructose intolerance is an inherited condition where the body does not produce the enzyme Aldolase B(also called fructose 1-phosphate) needed to break down and absorb fructose (fruit sugar).
The undigested fructose then collects in the liver and kidneys, eventually causing liver and kidney failure. One person in about 20,000 is born with this disorder. It is reported more frequently in the United States and Northern European countries than in other parts of the world. It occurs with equal frequency in males and females.
Causes and symptoms:
Fructose intolerance is an inherited disorder passed on to children through their parents' genes. Both the mother and father have the gene that causes the condition, but may not have symptoms of fructose intolerance themselves. (This is called an autosomal recessive pattern of inheritance.) The disorder will not be apparent until the infant is fed formula, juice, fruits, or baby foods that contain fructose.
Initial symptoms include vomiting, dehydration, and unexplained fever. Other symptoms include extreme thirst and excessive urination and sweating. There will also be a loss of appetite and a failure to grow. Tremors and seizures caused by low blood sugar can occur. The liver becomes swollen and the patient becomes jaundiced with yellowing of the eyes and skin. Left untreated, this condition can lead to coma and death.
Diagnosis:
Urine tests can be used to detect fructose sugar in the urine. Blood tests can also be used to detect hyperbilirubinemia and high levels of liver enzymes in the blood. A liver biopsy may be performed to test for levels of enzymes present and to evaluate the extent of damage to the liver. A fructose-loading test where a dose of fructose is given to the patient in a well-controlled hospital or clinical setting may also be used to confirm fructose intolerance. Both the biopsy and the loading test can be very risky, particularly in infants that are already sick. Safest test is a DNA analysis but as not all mutations have yet been detected then this can lead to a false negative, but it is the least invasive and stressful to the body. Alternatively a diet exclusion may give the answer that is needed.
Treatment:
Once diagnosed, fructose intolerance can be successfully treated by eliminating fructose from the diet. Patients usually respond within three to four weeks and can make a complete recovery if fructose-containing foods are avoided. Early recognition and treatment of the disease is important to avoid damage to the liver, kidneys, and small intestine.
Prognosis:
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 can develop normally.
For more information on this head over to www.hfiinfo.proboards.com
If symptoms occur later in life then HFI is unlikely to be the cause of symptoms but not unheard of as the patient may have naturally avoided fructose containing foods and just not known why they feel unwell after eating certain foods.
Hereditary fructose intolerance is an inherited condition where the body does not produce the enzyme Aldolase B(also called fructose 1-phosphate) needed to break down and absorb fructose (fruit sugar).
The undigested fructose then collects in the liver and kidneys, eventually causing liver and kidney failure. One person in about 20,000 is born with this disorder. It is reported more frequently in the United States and Northern European countries than in other parts of the world. It occurs with equal frequency in males and females.
Causes and symptoms:
Fructose intolerance is an inherited disorder passed on to children through their parents' genes. Both the mother and father have the gene that causes the condition, but may not have symptoms of fructose intolerance themselves. (This is called an autosomal recessive pattern of inheritance.) The disorder will not be apparent until the infant is fed formula, juice, fruits, or baby foods that contain fructose.
Initial symptoms include vomiting, dehydration, and unexplained fever. Other symptoms include extreme thirst and excessive urination and sweating. There will also be a loss of appetite and a failure to grow. Tremors and seizures caused by low blood sugar can occur. The liver becomes swollen and the patient becomes jaundiced with yellowing of the eyes and skin. Left untreated, this condition can lead to coma and death.
Diagnosis:
Urine tests can be used to detect fructose sugar in the urine. Blood tests can also be used to detect hyperbilirubinemia and high levels of liver enzymes in the blood. A liver biopsy may be performed to test for levels of enzymes present and to evaluate the extent of damage to the liver. A fructose-loading test where a dose of fructose is given to the patient in a well-controlled hospital or clinical setting may also be used to confirm fructose intolerance. Both the biopsy and the loading test can be very risky, particularly in infants that are already sick. Safest test is a DNA analysis but as not all mutations have yet been detected then this can lead to a false negative, but it is the least invasive and stressful to the body. Alternatively a diet exclusion may give the answer that is needed.
Treatment:
Once diagnosed, fructose intolerance can be successfully treated by eliminating fructose from the diet. Patients usually respond within three to four weeks and can make a complete recovery if fructose-containing foods are avoided. Early recognition and treatment of the disease is important to avoid damage to the liver, kidneys, and small intestine.
Prognosis:
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 can develop normally.
For more information on this head over to www.hfiinfo.proboards.com