ICD-11 classes
13 Diseases of the digestive system
Digestive system disorders of fetus or newborn
KB89 — Neonatal malabsorption syndromes
5C51.50 — Hereditary fructose intolerance
ICD-11 5C51.50 — Hereditary fructose intolerance
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.
The diagnosis includes nothing.
It excludes 1 item.
- Fructose malabsorption (5C61.40)
It has no clarifying diagnoses.
The diagnosis is coded elsewhere: