Trehalase deficiency

In the gut, trehalose is rapidly metabolized to glucose by the specific enzyme trehalase. A small minority of the population exhibits a primary (hereditary) or secondary (acquired) trehalase deficiency and thus may experience intestinal discomfort after ingestion of excessive amounts of trehalose owing to the osmotic activity of undigested trehalose in the gut. However, smaller amounts of trehalose are tolerated by such individuals without any symptoms. ... 

Sucrase-Isomaltase and Trehalase Deficiencies and Monosaccharide Malabsorption... 

Sucrase-isomaltase deficiency usually presents clinically in infancy when sucrose and fruit are introduced in the diet, " but sometimes first presents in adulthood. The deficiency is rare in Caucasians, but more common in Eskimo groups. Deficiencies of both lactase and sucrase-isomaltase may occur secondary to other small bowel diseases (e.g., cehac disease, Crohn s disease,or acute gastroenteritis). Trehalase deficiency is a rare disorder,except in Greenland, where it occurs in 8% of the population. It is manifested by diarrhea following the ingestion of mushrooms. 

Sucrase-isomaltase deficiency can be investigated by using 50 g sucrose instead of lactose. An increase in breath hydrogen of >20 ppm (20 pL/L) within 2 hours is diagnostic. It is rarely necessary to test for trehalase deficiency, although a breath test using 25 g trehalose has been described. ... 

Gudmund-Hoyer E, Fenger HJ, Skovbjerg H, Kern-Hansen P, Madsen PR. Trehalase deficiency in Greenland. Scand J Gastroenterol 1988 23 775-8. 

The levels of aa-trehalase activity in jejunal biopsy specimens from normals and from patients with a deficiency of sucrose a-D-glucohydrolase-oligo-l,6-D-glucosidase ( sucrase-isomaltase ) have been compared." ... 

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