Essential fructosuria

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Essential fructosuria

Biochemistry

Biochemistry

Citric acid cycle

Electron transport chain and oxidative phosphorylation

Gluconeogenesis

Glycogen metabolism

Pentose phosphate pathway

Physiological changes during exercise

Amino acid metabolism

Nitrogen and urea cycle

Fatty acid synthesis

Fatty acid oxidation

Ketone body metabolism

Cholesterol metabolism

Essential fructosuria

Hereditary fructose intolerance

Pyruvate dehydrogenase deficiency

Glucose-6-phosphate dehydrogenase (G6PD) deficiency

Lactose intolerance

Glycogen storage disease type I

Glycogen storage disease type II (NORD)

Glycogen storage disease type III

Glycogen storage disease type IV

Glycogen storage disease type V

Metachromatic leukodystrophy (NORD)

Gaucher disease (NORD)

Niemann-Pick disease types A and B (NORD)

Fabry disease (NORD)

Tay-Sachs disease (NORD)

Mucopolysaccharide storage disease type 1 (Hurler syndrome) (NORD)

Mucopolysaccharide storage disease type 2 (Hunter syndrome) (NORD)

Hartnup disease

Ornithine transcarbamylase deficiency

Phenylketonuria (NORD)

Cystinuria (NORD)

Maple syrup urine disease

Abetalipoproteinemia

Familial hypercholesterolemia

Hypertriglyceridemia

Disorders of carbohydrate metabolism: Pathology review

Disorders of fatty acid metabolism: Pathology review

Dyslipidemias: Pathology review

Glycogen storage disorders: Pathology review

Lysosomal storage disorders: Pathology review

Disorders of amino acid metabolism: Pathology review

Carbohydrates and sugars

Fats and lipids

Folate (Vitamin B9) deficiency

Vitamin B12 deficiency

Wernicke-Korsakoff syndrome

Fat-soluble vitamin deficiency and toxicity: Pathology review

Water-soluble vitamin deficiency and toxicity: B1-B7: Pathology review

Zinc deficiency and protein-energy malnutrition: Pathology review

Key Takeaways

Essential fructosuria is a benign autosomal recessive disorder in which there is a deficiency caused by a deficiency fructokinase enzyme. People with essential fructosuria don't metabolize fructose properly after ingesting a fructose-rich diet, causing it to be in urine, hence the name fructosuria.