A person who has a sucrose intolerance cannot digest sucrose, a sugar found in many foods, including fruits. The malabsorption syndrome is termed congenital sucrase-isomaltase ceficiency, or CSID, and may result from a genetic mutation. Symptoms can appear as early as 6 months of age and can be soothed by ingesting sucrase, an enzyme that breaks down sucrose into its components for energy use.
Onset
CSID appears in infancy after the child is weaned from breast milk or formula containing only lactose and is introduced to fruits and fruit juices and other foods containing starch and sucrose. The prevalence of this disease is estimated to be 1 in 5,000 in the European population, but is actually higher in the native people of Canada, Greenland and Alaska, according to 2007 information from Orphanet. Estimates of incidence in these populations is between 3 and 10 percent, as noted in 2007 information provided by Madisons Foundation.
Symptoms
The sucrase-somaltase deficiency is indicated by chronic watery diarrhea, diaper rash from the diarrhea, stomach bloating, gas and sometimes vomiting. The child exhibits signs of stomach pain, muscle wasting, irritability and a general failure to grow and thrive due to poorly absorbed nutrition. Meal times and toilet training may become difficult for both the parent and child. However, the sucrose intolerance may resolve as the child grows older.
Diagnosis
A diagnosis of CSID is often difficult to make because the symptoms mimic other diseases including irritable bowel syndrome, celiac disease and cystic fibrosis. They are also similar to infection, or heart and liver disease symptoms. An intestinal biopsy of small intestinal tissue determines if there is a sucrase deficiency and impaired isomaltase functioning. A breath hydrogen test is given to look for elevated hydrogen levels in the child. Stool is examined for a pH of 5 to 6.
Treatment
A diet that limits sucrose and starch containing foods may alleviate symptoms of CSID and provide enough nutrition for the child to grow properly. However, such a restrictive diet is difficult to follow. Enzyme replacement therapy taken orally adds sucrase derived from a yeast solution to the body so that it can break down sucrose and starches. This enzyme must be taken each time food with sucrose or starch is ingested to prevent symptoms.
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