Disease Information for Sucrose intolerance (disaccharidase df)

Congenital Disaccharide intolerance I; Disaccharide Intolerance I; CSID ; Congenital Sucrose Isomaltose Malabsorption ; SI Deficiency; Sucrase-Isomaltase Deficiency, Congenital ; Sucrose Intolerance; Congenital Disaccharide intolerance I is a rare inherited metabolic disorder characterized by the deficiency or absence of the enzymes sucrase and isomaltase; This enzyme complex (sucrase-isomaltase) assists in the breakdown of a certain sugars (ie, sucrose) and certain products of starch digestion (dextrins); The sucrase-isomaltase enzyme complex is normally found within the tiny, finger-like projections (microvilli or brush border) lining the small intestine; When this enzyme complex is deficient, nutrients based on ingested sucrose and starch cannot be absorbed properly from the gut; Symptoms of this disorder become evident soon after sucrose or starches, as found in modified milk formulas with sucrose or polycose, are ingested by an affected infant; Breast-fed infants or those on lactose-only formula manifest no symptoms until such time as sucrose (found in fruit juices, solid foods, and/or some medications) is introduced into the diet; Symptoms are variable among affected individuals but usually include watery diarrhea, abdominal swelling (distension) and/or discomfort, among others; Intolerance to starch often disappears within the first few years of life and the symptoms of sucrose intolerance usually improve as the affected child ages; Disaccharide intolerance I is inherited as an autosomal recessive genetic trait;----------------[NORD WEBSITE 2006]----------------------------------

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