Letzte Aktualisierung am 7. December 2023 von Dr. Michael Zechmann-Khreis
Sucrose intolerance is medically correctly called sucrose-isomaltose intolerance or also sucrose-isomaltase deficiency. It is an autosomal recessive inherited metabolic disorder in which normal household sugar (sucrose) is poorly tolerated or not tolerated at all.
This is an enzyme deficiency disease of the small intestine (primary form). The enzyme for the breakdown of sucrose (household sugar), known as saccharase-isomaltase, is not working properly. It is present, but not active. During the ripening phase, it loses contact with the cell membrane, so to speak, and is excreted into the small intestine[1] . The two sugars can therefore not be absorbed, enter the large intestine and are burned there by bacteria to carbon dioxide and water. The consequences are diarrhea, abdominal cramps, vomiting and malaise.
Sucrose-isomaltose malabsorption usually only becomes apparent from around 6 months of age, as this is the first time such sugars are ingested with complementary foods.
Symptoms vary from severe symptoms such as stunted growth, dehydration or explosive diarrhea, to milder symptoms that may not be noticed until adulthood 6).
The incidence in Europe is estimated to be around 0.05% to 0.2% of the population, while the incidence among the Inuit or other circumpolar peoples is thought to be 3-10% 2-5).
Sources:
1) “Journal of Clinical Investigation” (Jacob et al., 106:281-287 (2000))
2) Peterson M, Herber R. Intestinal sucrase deficiency. Trans Assoc Am Physicians 1967; 80:275-283.
3) Welsh J, Poley J, Bhatia M, et al. Intestinal disaccharidase activities in relation to age, race, and mucosal damage. Gastroenterology 1978; 75:847-855.
4) Bell R, Draper H, Bergan JG. Sucrose, lactose, and glucose intolerance in northern Alaskan Eskimos. Am J Clin Nutr 1973; 26:1185-1190.
5) Ellestad-Sayad J, Haworth J, Hildes J. Disaccharide malabsorption and dietary patterns in two Canadian Eskimo communities. Am J Clin Nutr 1978; 31:1473-1478.
6) Uhrich, Stefanie; Wu, Zaining; Huang, Jie-Yu; Scott, C. Ronald; Four Mutations in the SI Gene Are Responsible for the Majority of Clinical Symptoms of CSID; Journal of Pediatric Gastroenterology & Nutrition: November 2012 – Volume 55 – Issue – p S34-S35
