5C61.3 Alpha, alpha trehalase deficiency

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


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.

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