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Disaccharidases are glycoside hydrolases, enzymes that break down certain types of sugars called disaccharides into simpler sugars called monosaccharides.In the human body, disaccharidases are made mostly in an area of the small intestine's wall called the brush border, making them members of the group of "brush border enzymes".
Excessive flatus and abdominal bloating may reflect excessive gas production due to fermentation of unabsorbed carbohydrate, especially among patients with a primary or secondary disaccharidase deficiency, such as lactose intolerance or sucrose intolerance. Malabsorption of dietary nutrients and excessive fluid secretion by inflamed small ...
Sucrose intolerance or genetic sucrase-isomaltase deficiency (GSID) is the condition in which sucrase-isomaltase, an enzyme needed for proper metabolism of sucrose (sugar) and starch (e.g., grains), is not produced or the enzyme produced is either partially functional or non-functional in the small intestine.
Acid maltase deficiency (AMD) also known as Pompe disease was first described by Dutch pathologist JC Pompe in 1932. [ 10 ] [ 11 ] AMD is a non sex linked autosomal recessive condition in which excessive accumulation of glycogen build up within lysosome vacuoles in nearly all types of cells all over the body.
This is a shortened version of the third chapter of the ICD-9: Endocrine, Nutritional and Metabolic Diseases, and Immunity Disorders.It covers ICD codes 240 to 279.The full chapter can be found on pages 145 to 165 of Volume 1, which contains all (sub)categories of the ICD-9.
HFI is caused by a deficiency of fructose 1,6-biphosphate aldolase in the liver, kidney cortex and small intestine. Infants and adults are asymptomatic unless they ingest fructose or sucrose. [citation needed] Deficiency of hepatic fructose 1,6-biphosphate (FBPase) causes impaired gluconeogenesis, hypoglycemia and severe metabolic acidemia.
Glucose-galactose malabsorption generally becomes apparent in the first few weeks of a baby's life. Affected infants experience severe diarrhea resulting in life-threatening dehydration, increased acidity of the blood and tissues (), and weight loss when fed breast milk or regular infant formulas.
Breaking apart a double sugar into its two monosaccharides is accomplished by hydrolysis with the help of a type of enzyme called a disaccharidase. As building the larger sugar ejects a water molecule, breaking it down consumes a water molecule. These reactions are vital in metabolism.