Congenital Sucrase-Isomlatase Deficiency CSID

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Nutritional Adaptations Congenital Sucrase-Isomaltase Deficiency, CSID, is a congenital enzymatic disorder with which the body is unable to properly digest certain carbohydrates. A person with CSID typically produces low or absent amounts of sucrase, low to normal amounts of isomaltase, and low amounts of maltase. Sucrase, isomaltase, and maltase are important enzymes because they break down complex sugars, also called disaccharides and polysaccharides, into simple sugars, also called monosaccharides. Under ideal physiological conditions polysaccharides and disaccharides are broken down during digestion in the mouth, stomach, and small intestine, again, with the aid of enzymes, in order to be absorbed by the body. In a person with CSID this necessary digestion does not take place and therefore causes intestinal complications. As the names imply, sucrase breaks down sucrose (which is composed of one glucose and one fructose monosaccharide), maltase breaks down maltose (which is composed of two glucose monosaccharides), and isomaltase breaks down starches (which is a polysaccharide composed of more than two sugars linked together). People with CSID can break down starches to an extent, but usually cannot fully do so due to the inhibited enzymatic activity of sucrase-isomaltase (which breaks down 60-80% of starches in the small intestine (CSID Cares)). CSID is an autosomal recessive disorder of the sucrase-isomaltase gene (SI). A study of 34 CSID patients sequenced

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