Fructose metabolism disorders

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6 Terms

1
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What are the 2 main disorders of fructose?

  • essential fructosuria

  • hereditary fructose intolerance

2
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What is essential fructosuria caused by?

  • benign autosomal recessive condition

  • caused by deficiency of fructokinase

3
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What are the signs and symptoms of essential fructosuria?

  • asymptomatic

  • causes fructose in urine

4
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What are the causes of hereditary fructose intolerance?

  • autosomal recessive condition

  • caused by deficiency of aldolase B

  • causing an accumulation of fructose-1-phosphate

5
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What are the symptoms of hereditary fructose intolerance?

  • vomiting

  • hypoglycemia

  • jaundice

  • hepatomegaly

  • hyperuricaemia

6
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What is the management of hereditary fructose intolerance?

  • reduce intake of fructose

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