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Sucrose intolerance

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Sucrose intolerance

Sucrose intolerance
Classification and external resources
ICD-10 E74.3
ICD-9-CM 271.3
OMIM 222900
DiseasesDB 29844

Sucrose intolerance, also called congenital sucrase-isomaltase deficiency (CSID)[1] or sucrase-isomaltase deficiency,[2] is the condition in which sucrase, an enzyme needed for proper metabolism of sucrose (sugar), is not produced in the small intestine.

It is common among the Inupiat people.[3]

Contents

  • Overview 1
  • Signs and Symptoms of Sucrose Intolerance 2
  • See also 3
  • References 4
  • External links 5

Overview

Sucrose (also known as saccharose) is a disaccharide and is a two-sugar chain composed of glucose and fructose which are bonded together. A more familiar name is table, beet, or cane sugar. In most cases, sucrose intolerance is an autosomal recessive, genetic, metabolic disease, and involves deficiency in the enzyme sucrase, which breaks apart the glucose and fructose molecules. Monosaccharides, or single sugar units, are absorbed directly into the blood. When disaccharides are consumed, they must be broken down into monosaccharides by enzymes in the intestines before they can be absorbed. Sucrose can sometimes be found in the stool as an indication of an inability to digest and absorb sucrose in the body.

A deficiency of sucrase may result in malabsorption of sugar, which can lead to potentially serious symptoms. It is important for those with sucrose intolerance to minimize sucrose consumption as much as possible. Foods such as fruit and starch can help heal the body and reduce some symptoms. Supplements can be taken as a substitution of the enzyme missing or to introduce healthy bacteria into the immune system.

Sucrose intolerance (congenital sucrase-isomaltase enzyme deficiency) can be caused by genetic mutations in which both parents must contain this gene in order for the child to carry the disease. Sucrose intolerance can also be caused by irritable bowel syndrome, aging, or small intestine disease. There are specific tests used to determine sucrose intolerance. The most common tests are the sucrose breath test, genetic analysis or enzyme activity determination, which takes place in the small intestine.

Signs and Symptoms of Sucrose Intolerance

See also

References

  1. ^ Sander P, Alfalah M, Keiser M, et al. (January 2006). "Novel mutations in the human sucrase-isomaltase (SI) gene that cause congenital carbohydrate malabsorption". Hum. Mutat. 27 (1): 119.  
  2. ^ Baudon JJ, Veinberg F, Thioulouse E, Morgant G, Aymard P, Charritat JL (April 1996). "Sucrase-isomaltase deficiency: changing pattern over two decades". J. Pediatr. Gastroenterol. Nutr. 22 (3): 284–8.  
  3. ^ Meier RJ, Draper H, Milan F (January 1991). "Pedigree analysis of sucrose intolerance among Native Alaskans". Arctic Med Res 50 (1): 8–12.  


External links

  • http://www.orphan-europe.com/sucrase_mangel_gb.html (broken link)
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