Sucrose intolerance
Encyclopedia
Sucrose intolerance, also called congenital sucrase-isomaltase deficiency (CSID) or Sucrase-isomaltase deficiency, 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.
(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
.
Sucrase
Sucrase is the name given to a number of enzymes that catalyze the hydrolysis of sucrose to fructose and glucose. The enzyme invertase, which occurs more commonly in plants, also hydrolyzes sucrose but by a different mechanism.-Physiology:...
, an enzyme
Enzyme
Enzymes are proteins that catalyze chemical reactions. In enzymatic reactions, the molecules at the beginning of the process, called substrates, are converted into different molecules, called products. Almost all chemical reactions in a biological cell need enzymes in order to occur at rates...
needed for proper metabolization
Metabolism
Metabolism is the set of chemical reactions that happen in the cells of living organisms to sustain life. These processes allow organisms to grow and reproduce, maintain their structures, and respond to their environments. Metabolism is usually divided into two categories...
of sucrose
Sucrose
Sucrose is the organic compound commonly known as table sugar and sometimes called saccharose. A white, odorless, crystalline powder with a sweet taste, it is best known for its role in human nutrition. The molecule is a disaccharide composed of glucose and fructose with the molecular formula...
, is not produced in the small intestine
Small intestine
The small intestine is the part of the gastrointestinal tract following the stomach and followed by the large intestine, and is where much of the digestion and absorption of food takes place. In invertebrates such as worms, the terms "gastrointestinal tract" and "large intestine" are often used to...
.
It is more common among the Inupiat.
Overview
SucroseSucrose
Sucrose is the organic compound commonly known as table sugar and sometimes called saccharose. A white, odorless, crystalline powder with a sweet taste, it is best known for its role in human nutrition. The molecule is a disaccharide composed of glucose and fructose with the molecular formula...
(also known as saccharose) is a disaccharide
Disaccharide
A disaccharide or biose is the carbohydrate formed when two monosaccharides undergo a condensation reaction which involves the elimination of a small molecule, such as water, from the functional groups only. Like monosaccharides, disaccharides form an aqueous solution when dissolved in water...
(simple sugars) composed of glucose
Glucose
Glucose is a simple sugar and an important carbohydrate in biology. Cells use it as the primary source of energy and a metabolic intermediate...
and fructose
Fructose
Fructose, or fruit sugar, is a simple monosaccharide found in many plants. It is one of the three dietary monosaccharides, along with glucose and galactose, that are absorbed directly into the bloodstream during digestion. Fructose was discovered by French chemist Augustin-Pierre Dubrunfaut in 1847...
, which are bonded together. A more familiar name is table, beet, or cane sugar. In most cases, sucrose intolerance is an autosomal recessive genetic
Genetic
Genetic may refer to:*Genetics, in biology, the science of genes, heredity, and the variation of organisms**Genetic, used as an adjective, refers to heredity of traits**Gene, a unit of heredity in the genome of an organism...
metabolic disease, which is a digestive deficiency
Deficiency
A deficiency is generally a lack of something. It may also refer to:*A deficient number, in mathematics, a number n for which σ A deficiency is generally a lack of something. It may also refer to:...
of the enzyme sucrase
Sucrase
Sucrase is the name given to a number of enzymes that catalyze the hydrolysis of sucrose to fructose and glucose. The enzyme invertase, which occurs more commonly in plants, also hydrolyzes sucrose but by a different mechanism.-Physiology:...
. 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
Malabsorption
Malabsorption is a state arising from abnormality in absorption of food nutrients across the gastrointestinal tract.Impairment can be of single or multiple nutrients depending on the abnormality...
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
Irritable bowel syndrome
Irritable bowel syndrome is a diagnosis of exclusion. It is a functional bowel disorder characterized by chronic abdominal pain, discomfort, bloating, and alteration of bowel habits in the absence of any detectable organic cause. In some cases, the symptoms are relieved by bowel movements...
, 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
Small intestine
The small intestine is the part of the gastrointestinal tract following the stomach and followed by the large intestine, and is where much of the digestion and absorption of food takes place. In invertebrates such as worms, the terms "gastrointestinal tract" and "large intestine" are often used to...
.
Symptoms of Sucrose Intolerance
- Abdominal cramps and bloating
- DiarrheaDiarrheaDiarrhea , also spelled diarrhoea, is the condition of having three or more loose or liquid bowel movements per day. It is a common cause of death in developing countries and the second most common cause of infant deaths worldwide. The loss of fluids through diarrhea can cause dehydration and...
and constipation - Vomiting
- HypoglycemiaHypoglycemiaHypoglycemia or hypoglycæmia is the medical term for a state produced by a lower than normal level of blood glucose. The term literally means "under-sweet blood"...
and headaches - Poor weight gain and growth
- Upper respiratory tractUpper respiratory tractThe upper respiratory tract or upper airway primarily refers to the parts of the respiratory system lying outside of the thorax or above the sternal angle. Another definition commomly used in medicine is the airway above the glottis or vocal cords...
and viral infections - AnxietyAnxietyAnxiety is a psychological and physiological state characterized by somatic, emotional, cognitive, and behavioral components. The root meaning of the word anxiety is 'to vex or trouble'; in either presence or absence of psychological stress, anxiety can create feelings of fear, worry, uneasiness,...
and heart palpitations - Excess gas production
See also
- Fructose malabsorptionFructose malabsorptionFructose malabsorption, formerly named "dietary fructose intolerance," is a digestive disorder in which absorption of fructose is impaired by deficient fructose carriers in the small intestine's enterocytes. This results in an increased concentration of fructose in the entire intestine.Fructose...
- GastroenterologyGastroenterologyGastroenterology is the branch of medicine whereby the digestive system and its disorders are studied. The name is a combination of three Ancient Greek words gaster , enteron , and logos...
- Lactose intoleranceLactose intoleranceLactose intolerance, also called lactase deficiency or hypolactasia, is the inability to digest and metabolize lactose, a sugar found in milk...
- Sucrase-isomaltaseSucrase-isomaltaseSucrase-isomaltase is a glucosidase enzyme.A deficiency is responsible for sucrose intolerance.-External links:*...
External links
- http://www.csidinfo.com/
- http://www.orphan-europe.com/sucrase_mangel_gb.html
- http://www.sucraid.net/