Sucrose intolerance

Sucrose intolerance
Sucrose
Classification and external resources
Specialty	endocrinology
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]

Overview

Sucrose (also termed 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. Dietary supplements can be taken as a substitute for the enzyme missing or to introduce healthy bacteria into the immune system.

Sucrose intolerance can be caused by genetic mutations in which both parents must contain this gene 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

Abdominal cramps and bloating
Diarrhea and constipation
Vomiting
Hypoglycemia and headaches
Poor weight gain and growth
Upper respiratory tract and viral diseases
Anxiety and heart palpitations
Excess gas production

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.
↑ 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.orphan-europe.com/sucrase_mangel_gb.html (broken link)

Diseases of the digestive system (primarily K20–K93, 530–579)

Upper GI tract

Esophagus	Esophagitis Candidal Eosinophilic Herpetiform Rupture Boerhaave syndrome Mallory-Weiss syndrome UES Zenker's diverticulum LES Barrett's esophagus Esophageal motility disorder Nutcracker esophagus Achalasia Diffuse esophageal spasm Gastroesophageal reflux disease (GERD) Laryngopharyngeal reflux (LPR) Esophageal stricture Megaesophagus

Stomach	Gastritis Atrophic Ménétrier's disease Gastroenteritis Peptic (gastric) ulcer Cushing ulcer Dieulafoy's lesion Dyspepsia Pyloric stenosis Achlorhydria Gastroparesis Gastroptosis Portal hypertensive gastropathy Gastric antral vascular ectasia Gastric dumping syndrome Gastric volvulus

Lower GI tract:
Intestinal/
Enteropathy

Small intestine (Duodenum/Jejunum/Ileum)	Enteritis Duodenitis Jejunitis Ileitis Peptic (duodenal) ulcer Curling's ulcer Malabsorption: Coeliac Tropical sprue Blind loop syndrome Small bowel bacterial overgrowth syndrome Whipple's Short bowel syndrome Steatorrhea Milroy disease bile acid malabsorption

Large intestine (Appendix/Colon)	Appendicitis Colitis Pseudomembranous Ulcerative Ischemic Microscopic Collagenous Lymphocytic Functional colonic disease IBS Intestinal pseudoobstruction / Ogilvie syndrome Megacolon / Toxic megacolon Diverticulitis/Diverticulosis

Large and/or small	Enterocolitis Necrotizing Gastroenterocolitis IBD Crohn's disease Vascular: Abdominal angina Mesenteric ischemia Angiodysplasia Bowel obstruction: Ileus Intussusception Volvulus Fecal impaction Constipation Diarrhea Infectious Intestinal adhesions

Rectum	Proctitis Radiation proctitis Proctalgia fugax Rectal prolapse Anismus

Anal canal	Anal fissure/Anal fistula Anal abscess Anal dysplasia Pruritus ani

GI bleeding/BIS

Accessory

Liver	Hepatitis Viral hepatitis Autoimmune hepatitis Alcoholic hepatitis Cirrhosis PBC Fatty liver NASH Vascular Budd-Chiari syndrome Hepatic veno-occlusive disease Portal hypertension Nutmeg liver Alcoholic liver disease Liver failure Hepatic encephalopathy Acute liver failure Liver abscess Pyogenic Amoebic Hepatorenal syndrome Peliosis hepatis Metabolic disorders Wilson's Disease Hemochromatosis

Gallbladder	Cholecystitis Gallstones/Cholecystolithiasis Cholesterolosis Rokitansky-Aschoff sinuses Postcholecystectomy syndrome Porcelain gallbladder

Bile duct/ Other biliary tree	Cholangitis Primary sclerosing cholangitis Secondary sclerosing cholangitis Ascending Cholestasis/Mirizzi's syndrome Biliary fistula Haemobilia Gallstones/Cholelithiasis Common bile duct Choledocholithiasis Biliary dyskinesia Sphincter of Oddi dysfunction

Pancreatic	Pancreatitis Acute Chronic Hereditary Pancreatic abscess Pancreatic pseudocyst Exocrine pancreatic insufficiency Pancreatic fistula

Abdominopelvic

Hernia	Diaphragmatic Congenital Hiatus Inguinal Indirect Direct Umbilical Femoral Obturator Spigelian Lumbar Petit's Grynfeltt-Lesshaft Undefined location Incisional Internal hernia

Peritoneal	Peritonitis Spontaneous bacterial peritonitis Hemoperitoneum Pneumoperitoneum

Inborn error of carbohydrate metabolism: monosaccharide metabolism disorders (including glycogen storage diseases) (E73–E74, 271)

Sucrose, transport
(extracellular)

Disaccharide catabolism	Congenital alactasia Sucrose intolerance

Monosaccharide transport	Glucose-galactose malabsorption Inborn errors of renal tubular transport (Renal glycosuria) Fructose malabsorption

Hexose → glucose

Monosaccharide catabolism

fructose:	Essential fructosuria Fructose intolerance

galactose/galactosemia:	GALK deficiency GALT deficiency/GALE deficiency

Glucose ⇄ glycogen

Glycogenesis

Glycogenolysis

extralysosomal:	GSD type V, McArdle, muscle glycogen phosphorylase/GSD type VI, Hers', liver glycogen phosphorylase GSD type III, Cori's, debranching

lysosomal/LSD: GSD type II, Pompe's, glucosidase

Glucose ⇄ CAC

Glycolysis	MODY 2/HHF3 GSD type VII, Tarui's, phosphofructokinase Triosephosphate isomerase deficiency Pyruvate kinase deficiency

Gluconeogenesis	PCD Fructose bisphosphatase deficiency GSD type I, von Gierke, glucose 6-phosphatase

Pentose phosphate pathway

Other

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Sucrose intolerance

Overview

Signs and symptoms

See also

References

External links