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Glucose-galactose malabsorption is a rare disorder; only a few hundred cases have been identified worldwide. However, as many as 10 percent of the population may have a somewhat reduced capacity for glucose absorption without associated health problems. This condition may be a milder variation of glucose-galactose malabsorption. [2]
Lactose is a disaccharide sugar composed of galactose and glucose that is found in milk. Lactose can not be absorbed by the intestine and needs to be split in the small intestine into galactose and glucose by the enzyme called lactase; unabsorbed lactose can cause abdominal pain, bloating, diarrhea, gas, and nausea. [citation needed]
Worldwide, around 65% of adults are affected by lactose malabsorption. [ 5 ] [ 8 ] Other mammals usually lose the ability to digest lactose after weaning . Lactose intolerance is the ancestral state of all humans before the recent evolution of lactase persistence in some cultures, which extends lactose tolerance into adulthood. [ 9 ]
The only treatment for classic galactosemia is eliminating lactose and galactose from the diet (e.g. exclusion of dairy products containing lactose). [9] [20] [21] Lactose-restricted diet is efficient in resolving acute complications; however, it is not sufficient to prevent long-term complications affecting the brain and female gonads. [20]
E.g., Nitisinone prevents the formation of toxic metabolites for patients with Tyrosinemia Type I and enables normal growth and development in combination with a low-protein diet; Vitamins. E.g., thiamine supplementation benefits several types of disorders that cause lactic acidosis.
Some FODMAPs, such as fructose, are readily absorbed in the small intestine of humans via GLUT receptors. [19] Absorption thus depends on the appropriate expression and delivery of these receptors in the intestinal enterocyte to both the apical surface, contacting the lumen of the intestine (e.g., GLUT5), and to the basal membrane, contacting the blood (e.g., GLUT2). [19]
After resorption in the gut, the monosaccharides are transported, through the portal vein, to the liver, where all non-glucose monosacharids (fructose, galactose) are transformed into glucose as well. [4] Glucose (blood sugar) is distributed to cells in the tissues, where it is broken down via cellular respiration, or stored as glycogen.
[13] [24] Typically, the severe diarrhea improves with a diet low in glucose/galactose and the tolerability to glucose/galactose improves with age. [13] [25] Hirschsprung's disease (HSCR), a gut motility disorder characterized by a lack of nerve cells in the large intestine which are needed to move the stool through the digestive tract.