Post by tummyache on Sept 10, 2012 13:32:26 GMT -5
I found the following on the web and thought I would see what others thought - are there really different levels of sensitivity? I iltalicized parts I am wondering about:
[1] www.bu.edu/aldolase/HFI/hfiinfo/detail.html
The treatment for HFI is the exclusion of fructose, sucrose, and sorbitol ([less than 40 mg/kg per day) from the diet (3) and results in complete alleviation of most symptoms and a normal life span (1). Older HFI subjects who adhere to a self-imposed fructose-restricted diet may continue to live undiagnosed and lead relatively normal lives. Complete exclusion of fructose is often difficult, however, and many HFI patients develop a syndrome of chronic fructose intoxication characterized by retarded growth, chronic liver disease, and hepatomegaly (3, 9, 15, 16). Furthermore, if not treated properly, these patients suffer episodes of hypoglycemia, general ill health, and strained relationships with family members due to their peculiar eating habits (17). Constant daily risk remains for HFI individuals due to the increasingly widespread use of these sugars as nutrients and sweeteners (18).
[2] omim.org/entry/229600
Clinical Variability - An adult form of fructose intolerance was reported by Lameire et al. (1978) in a Belgian patient. When a fructose solution was used for intravenous alimentation during management of viral meningitis, a 21-year-old man developed severe illness characterized by acute jaundice, gastrointestinal bleeding, hypoglycemia, proximal tubular acidosis, and disseminated intravascular coagulation. The renal disorder was characterized by glycosuria, amino aciduria, phosphaturia, and bicarbonaturia with high urinary pH despite metabolic acidosis. Liver fructose-1-phosphate aldolase activity was 30% of normal, and fructose-1,6-diphosphate aldolase activity was normal. In classic fructose intolerance, these values are 0 to 6% and 10 to 50% of normal, respectively. Thus, the patient appeared to have a mild form of the disorder, may have been heterozygous, and likely showed manifestations only because of the massive fructose infusion.
[3] www.counsyl.com/diseases/hereditary-fructose-intolerance/
Symptoms of the disease can vary from mild to severe. People with HFI often show an aversion to sweets and fruit, and thus those with a mild case may be protected from some of the symptoms they would otherwise experience.
[4] www.merckmanuals.com/home/childrens_health_issues/hereditary_metabolic_disorders/disorders_of_carbohydrate_metabolism.html
The diagnosis is made when a chemical examination of a sample of liver tissue determines that the enzyme is missing. Treatment involves excluding fructose (generally present in sweet fruits), sucrose, and sorbitol (a sugar substitute) from the diet. Severe attacks of hypoglycemia respond to glucose given by vein. Milder attacks are treated with glucose tablets, which should be carried by anyone who has hereditary fructose intolerance.
[1] www.bu.edu/aldolase/HFI/hfiinfo/detail.html
The treatment for HFI is the exclusion of fructose, sucrose, and sorbitol ([less than 40 mg/kg per day) from the diet (3) and results in complete alleviation of most symptoms and a normal life span (1). Older HFI subjects who adhere to a self-imposed fructose-restricted diet may continue to live undiagnosed and lead relatively normal lives. Complete exclusion of fructose is often difficult, however, and many HFI patients develop a syndrome of chronic fructose intoxication characterized by retarded growth, chronic liver disease, and hepatomegaly (3, 9, 15, 16). Furthermore, if not treated properly, these patients suffer episodes of hypoglycemia, general ill health, and strained relationships with family members due to their peculiar eating habits (17). Constant daily risk remains for HFI individuals due to the increasingly widespread use of these sugars as nutrients and sweeteners (18).
[2] omim.org/entry/229600
Clinical Variability - An adult form of fructose intolerance was reported by Lameire et al. (1978) in a Belgian patient. When a fructose solution was used for intravenous alimentation during management of viral meningitis, a 21-year-old man developed severe illness characterized by acute jaundice, gastrointestinal bleeding, hypoglycemia, proximal tubular acidosis, and disseminated intravascular coagulation. The renal disorder was characterized by glycosuria, amino aciduria, phosphaturia, and bicarbonaturia with high urinary pH despite metabolic acidosis. Liver fructose-1-phosphate aldolase activity was 30% of normal, and fructose-1,6-diphosphate aldolase activity was normal. In classic fructose intolerance, these values are 0 to 6% and 10 to 50% of normal, respectively. Thus, the patient appeared to have a mild form of the disorder, may have been heterozygous, and likely showed manifestations only because of the massive fructose infusion.
[3] www.counsyl.com/diseases/hereditary-fructose-intolerance/
Symptoms of the disease can vary from mild to severe. People with HFI often show an aversion to sweets and fruit, and thus those with a mild case may be protected from some of the symptoms they would otherwise experience.
[4] www.merckmanuals.com/home/childrens_health_issues/hereditary_metabolic_disorders/disorders_of_carbohydrate_metabolism.html
The diagnosis is made when a chemical examination of a sample of liver tissue determines that the enzyme is missing. Treatment involves excluding fructose (generally present in sweet fruits), sucrose, and sorbitol (a sugar substitute) from the diet. Severe attacks of hypoglycemia respond to glucose given by vein. Milder attacks are treated with glucose tablets, which should be carried by anyone who has hereditary fructose intolerance.
