Alpha, alpha trehalase deficiency

Description

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.

Index terms

Alpha, alpha trehalase deficiency

Exclusions from above levels

3A10.Z – Hereditary haemolytic anaemia due to enzyme deficiency
5A71.01 – Congenital adrenal hyperplasia
5A81.1 – 5-alpha-reductase deficiency

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LD28.01 – Marfan syndrome
LD28.1 – Ehlers-Danlos syndrome
LD2A.4 – androgen resistance syndrome
18 – Pregnancy, childbirth or the puerperium
http://id.who.int/icd/entity/1004987305 – Transitory endocrine or metabolic disorders specific to fetus or newborn

Sibling codes

5C61.0 – Glucose-galactose malabsorption
5C61.1 – Maltase-glucoamylase deficiency
5C61.2 – Congenital sucrase-isomaltase deficiency
5C61.4 – Acquired monosaccharide malabsorption
5C61.5 – Disorders of facilitated glucose transport
5C61.6 – Lactose intolerance
5C61.Y – Other specified disorders of carbohydrate absorption or transport
5C61.Z – Disorders of carbohydrate absorption or transport, unspecified

Foundation entity

Alpha, alpha trehalase deficiency – http://id.who.int/icd/entity/2025219157

Part of: 5C61: Disorders of carbohydrate absorption or transport › 05: Endocrine, nutritional or metabolic diseases