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5C51.50 Hereditary fructose intolerance

International Classification of Diseases for Mortality and Morbidity Statistics, 11th Revision, v2024-01

Hereditary fructose intolerance is an autosomal recessive disorder due to a deficiency of fructose-1-phosphate aldolase activity, which results in an accumulation of fructose-1-phosphate in the liver, kidney, and small intestine, and is characterised by severe abdominal pain, vomiting, and hypoglycaemia following ingestion of fructose or other sugars metabolised through fructose-1-phosphate.

exclusions

• Fructose malabsorption  (5C61.40)

synonyms

• Hereditary fructose intolerance
• Hereditary fructose-1-phosphate aldolase deficiency
• Hereditary fructosaemia
• Fructose intolerance
• Fructosaemia
• Aldolase B deficiency
• fructose-1,6-bisphosphate aldolase b deficiency (disorder)
• fructose-biphosphate aldolase b deficiency
• fructose-1-phosphate aldolase deficiency
• fructose intolerance of newborn
• ALDB - [aldolase B] deficiency
• deficiency of fructose-bisphosphate aldolase

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