What is Fructosemia?

Hereditary fructose intolerance (fructosemia) is one of the more common (about 1:20,000 in Caucasians) inborn errors of metabolism (OMIM 229600), usually due to a mutation in the aldolase B gene (ALDOB; OMIM 612724), which encodes fructose-1-phosphate aldolase (fructoaldolase; EC 4.1.

What does Fruc mean?

1 : a crystalline sugar C6H12O6 sweeter and more soluble than glucose. 2 : the very sweet levorotatory d-form of fructose that occurs especially in fruit juices and honey. — called also fruit sugar, levulose.

What is HFI biochemistry?

Hereditary fructose intolerance (HFI) is a metabolic disease caused by the absence of an enzyme called aldolase B. In people with HFI, ingestion of fructose (fruit sugar) and sucrose (cane or beet sugar, table sugar) causes severe hypoglycemia (low blood sugar) and the build up of dangerous substances in the liver.

What is the medical term for essential fructosuria?

Essential fructosuria is a rare autosomal recessive disorder of fructose metabolism (see this term) caused by a deficiency of fructokinaseenzyme activity. It is characterized by elevated fructosemia and presence of fructosuria following ingestion of fructose and related sugars (sucrose, sorbitol).

What does it mean to have fructose in your blood?

Presence of fructose in the circulating blood. See also: hereditary fructose intolerance. Presence of fructose in the circulating blood. Synonym (s): fructosaemia. Want to thank TFD for its existence? Tell a friend about us, add a link to this page, or visit the webmaster’s page for free fun content . Mentioned in ?

What do you need to know about hereditary fructose intolerance?

Hereditary fructose intolerance is a condition that affects a person’s ability to digest the sugar fructose. Fructose is a simple sugar found primarily in fruits. Affected individuals develop signs and symptoms of the disorder in infancy when fruits, juices, or other foods containing fructose are introduced into the diet.

What are the causes of galactosemia and fructosemia?

The plasma samples from the patients with fructosemia and galactosemia were obtained before dietary treatment. Several disorders and/or conditions are known to cause N-glycan abnormalities, such as galactosemia (16), fructosemia (17), chronic alcohol abuse (18), and HUS due to a Streptococcus pneumoniae infection (19).