Disease Information for Sucrose intolerance (disaccharidase df)

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Clinical Manifestations
Signs & Symptoms
Buttock area rash
Abdominal bloating
Abdominal fullness feeling
Abdominal Pain
Abdominal Pain Crampy
Abdominal Pain in Children
Abdominal pain/Improves fasting
Acute Diarrhea
Acute Diarrhea in Children
Bloating and Gas
Bloating and 'gas'/Flatulence
Chronic Diarrhea in a Child
Colicky Abdominal Pain
Diarrhea
Diarrhea in Children
Diarrhea, chronic
Diarrhea, explosive
Diarrhea, recurrent
Diarrhea/painless
Flatulence
Infant Apparent abdominal pain
Infant with Formula/Feeding problem
Postprandial bloating/fullness
Apparently well Silent disorder possible
Sucrose intolerance/complaint
Weight Loss
Disease Progression
Course/Chronic disorder
Course/Chronic only
Demographics & Risk Factors
Population Group
Child
Infant
Population/Pediatrics population
Sex & Age Groups
Population/Child
Population/Children/all
Population/Infant
Population/Toddler
Laboratory Tests
Abnormal Lab Findings (Non Measured)
Sucrose tolerance test abnormal (Lab)
STOOL acid/PH low
Abnormal Lab Findings - Increased
STOOL Reducing substances
Diagnostic Test Results
Pathology
BX/Jejunum/Disaccharidase assay abnormal
Associated Diseases & Rule outs
Rule Outs
Lactose intolerance syndrome
Associated Disease & Complications
Diaper rash
Disaccharidase deficiency
Infant colic syndrome
Sucrose intolerance/malabsorption
Disease Mechanism & Classification
Class
CLASS/Pediatric disorders (ex)
Pathophysiology
Pathophysiology/Genomic indentifiers (polymorphism/snip/mutations)
Process
PROCESS/Autosomal recessive disorder (ex)
PROCESS/Enzyme defect/Metabolic disorder (ex)
PROCESS/Hereditofamilial (category)
PROCESS/Metabolic/storage disorder (category)
Synonyms
Synonym
intolerance sucrose, sucrose intolerance, Sucrose intolerance (disorder), Synonym/Congenital Disaccharide intolerance I, Synonym/Congenital Sucrose Intolerance, Synonym/Congenital Sucrose Isomaltose Malabsorption, Synonym/CSID, Synonym/Disaccharide intolerance I, Synonym/SI deficiency, Synonym/SI deficiency (CSID), Synonym/Sucrase-isomaltase deficiency, congenital, Synonym/Sucrose isomaltose malabsorption, Synonym/Sucrose-Isomaltose Disaccharide Intolerance I, Synonym/Sucrose-Isomaltose malabsorption-congenital
Treatment
Other Treatments
TX/Specific diet/avoidance
Definition

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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External Links Related to Sucrose intolerance (disaccharidase df)
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Wikipedia
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PubMed (National Library of Medicine)
NGC (National Guideline Clearinghouse)
Medscape (eMedicine)
Harrison's Online (accessmedicine)
NEJM (The New England Journal of Medicine)
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