- Sucrose intolerance
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Sucrose intolerance Classification and external resources
SucroseICD-10 E74.3 ICD-9 271.3 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 metabolization of sucrose, is not produced in the small intestine.
It is more common among the Inupiat.[3]
Contents
Overview
Sucrose (also known as saccharose) is a disaccharide (simple sugars) 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, which is a digestive deficiency of the enzyme sucrase. Foods containing single sugars, allows the body to absorb them directly into your blood. However, when you eat disaccharides you must digest them first. Intestinal cells contain enzymes that are responsible for splitting disaccharides into monosaccharides (single sugar unit) in order for them to enter the bloodstream. Products in which are digested are delivered from the blood to the liver. This allows enzymes to modify nutrients. 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 sucrose may result in malabsorption of sugar, which can lead to more serious symptoms. It is important to discard as many sugar containing foods from the diet when sucrose intolerant. 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 welcome healthy bacteria into the immune system.
Sucrose Intolerance (Congenital sucrase-isomaltase enzyme deficiency) can be caused by genetic mutations (the common ones being SI and SII), 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.
Symptoms of Sucrose Intolerance
- Abdominal cramps and bloating
- Diarrhea and constipation
- Vomiting
- Hypoglycemia and headaches
- Poor weight gain and growth
- Upper respiratory tract and viral infections
- Anxiety and heart palpitations
- Excess gas production
See also
References
- ^ 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. doi:10.1002/humu.9392. PMID 16329100.
- ^ 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. doi:10.1097/00005176-199604000-00010. PMID 8708882. http://meta.wkhealth.com/pt/pt-core/template-journal/lwwgateway/media/landingpage.htm?issn=0277-2116&volume=22&issue=3&spage=284.
- ^ Meier RJ, Draper H, Milan F (January 1991). "Pedigree analysis of sucrose intolerance among Native Alaskans". Arctic Med Res 50 (1): 8–12. PMID 2021397.
External links
- http://www.csidinfo.com/
- http://www.orphan-europe.com/sucrase_mangel_gb.html
- http://www.sucraid.net/
Inborn error of carbohydrate metabolism: monosaccharide metabolism disorders (including glycogen storage diseases) (E73–E74, 271) Sucrose, transport
(extracellular)Disaccharide catabolismLactose intolerance · Sucrose intoleranceMonosaccharide transportHexose → glucose Monosaccharide catabolismGlucose ⇄ glycogen Glucose ⇄ CAC Pentose phosphate pathway Other This medical symptom article is a stub. You can help Wikipedia by expanding it.