ICD-11 code : 5C61.3;
Preferred Label : Alpha, alpha trehalase deficiency;
ICD-11 definition : 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.;
Origin ID : 2025219157;
ICD-10 Mapping
Validated automatic mappings to NTBT
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.