Hereditary Fructose Intolerance

Aka: Hereditary Fructose Intolerance

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II. Pathophysiology

  1. Intolerance to fructose or sucrose

III. Symptoms (follows sucrose or fructose exposure)

  1. Vomiting
  2. Hypoglycemia
  3. Seizures
  4. Coma
  5. Hepatomegaly (see Hepatomegaly in Newborns)
  6. Jaundice (Direct Hyperbilirubinemia)

IV. Labs

  1. Hepatic fructose-1-phosphatase aldolase activity

V. Management

  1. Fructose and sucrose eliminated from diet

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