ICD-11 classes
13 Diseases of the digestive system
Diseases of small intestine
DA90 — Nonstructural developmental anomalies of small intestine
5C61.3 — Alpha, alpha trehalase deficiency

ICD-11 5C61.3 — Alpha, alpha trehalase deficiency

Alpha, alpha trehalase deficiency is characterised by diarrhoea and vomiting after ingestion of trehalose, a disaccharide found mainly in mushrooms. The disease is very rare in most populations but the incidence has been estimated at least 1 in 13 in Greenland. Isolated trehalose intolerance is due to a deficiency of trehalase (TREH; 11q23.3), a brush-border membrane glycoprotein.

The diagnosis includes nothing.

The diagnosis excludes nothing.

It has no clarifying diagnoses.

The diagnosis is coded elsewhere:

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