Hereditary fructose intolerance

Hereditary fructose intolerance
Definition
Hereditary fructose intolerance is a disorder in which a person lacks the protein needed to break down fructose. Fructose is a fruit sugar that naturally occurs in the body.

Hereditary fructose intolerance
Alternate Names : Fructosemia, Fructose intolerance, Fructose aldolase B-deficiency, Fructose 1, 6 bisphosphate aldolase deficiency
Definition ...

Hereditary fructose intolerance (HFI) . Also known as Fructose-1-Phosphate Aldolase Deficiency , Fructosemia Disorder. An inherited inability to digest fructose (fruit sugar) or its precursors (sugar, sorbitol and brown sugar).

Hereditary Fructose Intolerance (HFI) should not be confused with Dietary Fructose Intolerance. HFI is a deficiency of a hepatic enzyme (fructose-1,6 diphosphatase) causing acute inhibition of hepatic glucose output when fructose is ingested.

Hereditary fructose intolerance may be relatively mild or a very severe disease. Complete avoidance of fructose and sucrose produces good results in most children with this condition. A few children will go on to develop severe liver disease.

Definition Hereditary fructose intolerance is a metabolic disorder in which the small intestine cannot process fructose (fruit sugar) into a source of energy because of an enzyme deficiency that prevents fructose absorption.

Fructose 1-phosphate aldolase (aldolase B) deficiency: This deficiency causes the clinical syndrome of hereditary fructose intolerance. Inheritance is autosomal recessive; incidence is estimated at 1/20,000 births.

Carbohydrate disorders; examples include diabetes insipidus, hereditary fructose intolerance, galactosemia, pyruvate metabolism disorders, von Gierke’s disease, McArdle disease, Pompe’s disease, and Forbes’ disease ...

Causes and incidence - Hereditary fructose intolerance
Causes and incidence - Diabetes mellitus
Causes and incidence - Diabetes insipidus
Diabetes Mellitus - epidemiology - Diabetes Mellitus
more » ...

For instance, cystinosis galactosemia, glycogen storage disease, hereditary fructose intolerance, Lowe syndrome, Wilson disease, tyrosinemia, medullary cystic disease, and vitamin D dependency, have been shown to cause Fanconi syndrome.

Serious illnesses such as kidney failure, liver disease or heart problems
Insulinoma
Hereditary fructose intolerance
Hormonal deficiencies
Early stages of pregnancy
Prolonged fasting
Long periods of strenuous exercise ...

can also result from inherited disorders that disrupt the body's normal breakdown and use of nutrients. Examples include the rare disease cystinosis, in which cystine crystals are deposited in bones and other tissues; hereditary fructose intolerance; ...

See also: Fructose intolerance, Symptom, Deficiency, Diabetes, Cancer