hereditary fructose intolerance…

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fructosemia, fructose intolerance, fructose aldolase b-deficiency, hereditary fructose intolerance, metabolic disease, absence of an enzyme, aldolase b, fructose, fruit sugar that naturally occurs, man-made fructose is used, a sweetener in many foods, baby food, drinks, hereditary fructose intolerance, autosomal recessive disease, common, 1 in 20,000, european countries, in fructose-intolerant people, ingestion of fructose, fruit sugar, sucrose, cane, beet sugar, table sugar, produces complicated chemical changes that cannot be corrected, the absence, the enzyme aldolase b, ingestion of fructose causes, profound hypoglycemia, low blood sugar, progressive liver damage, body is unable to convert its energy storage material, glycogen, into glucose, subsequently, blood sugar falls, hypoglycemia, blocks, in the metabolic pathway of fructose processing, cause a build-up of substances that damage the liver, hereditary fructose intolerance relatively mild, a very severe disease, severe form, eliminating fructose, sucrose, diet, prevent progressive liver disease, onset of symptoms after starting an infant on food, formula, poor feeding, a baby, irritability, prolonged neonatal jaundice, vomiting, convulsions, excessive sleepiness, intolerance, fruits, avoidance of fruits, fructose/sucrose-containing foods, doing well after eating foods, fructose/sucrose, early symptoms of fructose intolerance, resemble those of galactosemia, irritability, jaundice, vomiting, convulsions, an enlarged liver, spleen, later problems relate more to liver disease, physical examination, jaundice, yellow skin, icterus, yellow eyes, hepatosplenomegaly, enlarged liver, spleen, tests that confirm the diagnosis, positive urine test, reducing substances, hypoglycemia, low blood sugar, after receiving fructose/sucrose, excess uric acid in blood, hyperuricemia, the kidney to work properly, abnormally high amounts of sugars, amino acids, salts appearing in urine, liver failure shown, blood test, bleeding tendency revealed by coagulation tests of blood, liver biopsy, enzyme studies, genetic testing, fructose intolerance available, complete elimination of fructose, sucrose, diet, effective treatment, most patients, treatment of individual complications follows mainstream medical guidelines, patients, take medication to lower the level of uric acid in blood, thereby decrease risk, gout, absolute elimination of fructose, glucose produces good results in most children, fructose intolerance, a few children, go on to develop progressive liver disease, prognosis depends on how soon the diagnosis is made, how soon fructose, sucrose, eliminated, baby's diet, hypoglycemia, illness, due to eating fructose-, sucrose-containing foods, strong avoidance of fructose-containing foods, due to noxious effects, seizures, bleeding, gout, liver failure, death, child, above symptoms after began feeding the child formula, solid food, care of a medical specialist in biochemical genetics, metabolism is strongly recommended, child has this disorder, preliminary evidence suggests that parents of a child, carriers, the mutant gene, at increased risk, gout, arthritis, excess uric acid, uric acid crystals, in the joints, important to notify physician, family history of hereditary fructose intolerance, genetic counseling of value to prospective parents, a family history of fructose intolerance, the damaging effects, prevented by strict adherence to a fructose-free diet.