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Description

Hereditary fructose intolerance (HFI) is an inherited disorder caused by a deficiency of the enzyme aldolase B, which is necessary for the metabolism of fructose. People with HFI are unable to break down fructose, a sugar found in many fruits, vegetables, and sweeteners. When fructose is consumed, it accumulates in the liver and other organs, leading to a variety of symptoms, including abdominal pain, vomiting, and diarrhea. In severe cases, HFI can cause liver failure and even death. Treatment for HFI involves avoiding foods and beverages that contain fructose, as well as taking medications to help the body break down fructose.

Synonyms

• aldolase B deficiency
• fructose-1-phosphate aldolase deficiency
• fructose aldolase B deficiency
• fructose biphosphate aldolase B deficiency
• fructose intolerance
• fructosemia
• hereditary fructose intolerance
• hereditary fructosuria

Basic Lab Tests (measurements)

• plasma
  •      methionine
  •      tyrosine
• serum
  •      alanine aminotransferase
  •      aldolase
  •      bilirubin
  •      uric acid
  •      aldolase B
  •      potassium
  •      bicarbonate
  •      direct bilirubin
  •      chloride
  •      indirect bilirubin
  •      aspartate aminotransferase
  •      phosphate
• urine
  •      phosphate
  •      urine color
  •      fructose
  •      osmolal gap
  •      albumin
  •      bilirubin
  •      uric acid
  •      albumin:creatinine ratio
  •      anion gap
  •      red urine
  •      protein
  •      ammonia
  •      cloudy urine
  •      glucose
  •      pH

Detailed Disease and Condition Information (use the search buttons below to find details on these topics)
All of the following must be considered when interpreting clinical findings and are too extensive to be covered on this site: