Hereditary fructose intolerance
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Hereditary fructose intolerance
Biochemistry
Biochemistry and metabolism
Amino acid metabolism
Nitrogen and urea cycle
Citric acid cycle
Electron transport chain and oxidative phosphorylation
Gluconeogenesis
Glycogen metabolism
Glycolysis
Pentose phosphate pathway
Physiological changes during exercise
Cholesterol metabolism
Fatty acid oxidation
Fatty acid synthesis
Ketone body metabolism
Metabolic disorders
Alkaptonuria
Cystinuria (NORD)
Hartnup disease
Homocystinuria
Maple syrup urine disease
Ornithine transcarbamylase deficiency
Phenylketonuria (NORD)
Essential fructosuria
Galactosemia
Glucose-6-phosphate dehydrogenase (G6PD) deficiency
Hereditary fructose intolerance
Lactose intolerance
Pyruvate dehydrogenase deficiency
Abetalipoproteinemia
Familial hypercholesterolemia
Hyperlipidemia
Hypertriglyceridemia
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
Mucopolysaccharide storage disease type 1 (Hurler syndrome) (NORD)
Mucopolysaccharide storage disease type 2 (Hunter syndrome) (NORD)
Fabry disease (NORD)
Gaucher disease (NORD)
Krabbe disease
Leukodystrophy
Metachromatic leukodystrophy (NORD)
Niemann-Pick disease type C
Niemann-Pick disease types A and B (NORD)
Tay-Sachs disease (NORD)
Cystinosis
Disorders of amino acid metabolism: Pathology review
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
Assessments
Hereditary fructose intolerance
Flashcards
0 / 11 complete
USMLE® Step 1 questions
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High Yield Notes
3 pages
Flashcards
Hereditary fructose intolerance
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Questions
USMLE® Step 1 style questions USMLE
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External References
First Aid
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Cirrhosis p. 398
fructose intolerance p. 78
Fructose intolerance p. 78
Hypoglycemia
fructose intolerance p. 78
Jaundice p. 402
fructose intolerance p. 78
Vomiting
fructose intolerance p. 78
Summary
Hereditary fructose intolerance is an autosomal recessive genetic disorder in which there is a deficiency of the aldolase B enzyme, which leads to the accumulation of fructose 1-phosphate. People with hereditary fructose intolerance present with severe hypoglycemia, vomiting, hepatomegaly, and renal failure.
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