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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. All GSID patients lack fully ...
A deficiency is responsible for sucrose intolerance.Congenital sucrase-isomaltase deficiency (CSID), also called genetic sucrase-isomaltase deficiency (GSID), and sucrose intolerance, is a genetic, intestinal disorder that is caused by a reduction or absence of sucrase and isomaltase [13] Explanations for GSID include:
Sacrosidase (trade name Sucraid) is a medication used to replace sucrase in people lacking this enzyme. [1] It is available as an oral solution. Sucraid is approved by the U.S. Food and Drug Administration (FDA) for the therapy of the genetically determined sucrase deficiency that is part of the Congenital Sucrase-Isomaltase Deficiency (CSID).
One form, sucrase-isomaltase, is secreted in the small intestine on the brush border. [1] The enzyme invertase , which occurs more commonly in plants, fungi and bacteria, also hydrolyzes sucrose (and other fructosides) but by a different mechanism: it is a fructosidase, whereas sucrase is a glucosidase.
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.
Inborn errors of metabolism are often referred to as congenital metabolic diseases or inherited metabolic disorders. [2] Another term used to describe these disorders is "enzymopathies". This term was created following the study of biodynamic enzymology , a science based on the study of the enzymes and their products.
Isomaltase (EC 3.2.1.10) is an enzyme that breaks the bonds linking saccharides, which cannot be broken by amylase or maltase. It digests polysaccharides at the alpha 1-6 linkages. It digests polysaccharides at the alpha 1-6 linkages.
Succinate coenzyme Q reductase deficiency of; Succinic acidemia lactic acidosis congenital; Succinic acidemia; Succinic semialdehyde dehydrogenase deficiency; Succinyl-CoA acetoacetate transferase deficiency; Sucrase-isomaltase deficiency; Sucrose intolerance; Sudden cardiac death; Sudden infant death syndrome; Sudden sniffing death syndrome ...