If the level of sucrase activity is below the level considered necessary for normal sucrase function, the patient will be diagnosed with CSID.Noninvasive diagnostic methods include two breath tests, which can be useful screening tools but are not specific enough for a confirmed diagnosis of CSID.The first of these is the hydrogen/methane breath test, which measures the amount of hydrogen and methane gases a person exhales after consuming sugary water. The exhaled breath is collected in sealed test tubes at 30-minute intervals over a three-hour period after drinking the sugary water. If a person has CSID and there are little or no working sucrase-isomaltase enzymes in the intestines, greater than normal levels of hydrogen and/or methane are generated and exhaled in the breath. However, there may be other reasons why the person is exhaling excess hydrogen and/or methane gas, such as an overgrowth of bacteria in the small intestine, called small intestinal bacterial overgrowth (SIBO).The second breath test is called the carbon-13 (13C) breath test. Carbon-13 is a stable isotope of carbon that occurs naturally in sucrose, making it possible to track a person’s ability to digest and absorb sucrose by measuring the amount of 13CO2 exhaled after drinking a sugar-water solution. In this breath test, the exhaled breath is collected in sealed test tubes at 30-minute intervals over a 90-minute period after drinking the sugar-water solution.
Diet modification
The level of ability to digest sucrose or starch sugars is unique to each person who is living with CSID and depends on many factors, including the following:
The individual level of function of the digestive enzyme sucrase-isomaltase
How well the other functions of the gastrointestinal tract are working
How much sucrose and starch sugars are consumed
If a person has any other health issues that may affect digestionFor these reasons, a restriction diet needs to be specific for each patient who has CSID. Before making any changes to the diet, it is important for a patient to speak with a healthcare provider, especially if the patient is underweight or not gaining weight as would be expected. It is also advised that the patient work with a registered dietitian or nutritionist who can help identify the foods that can be tolerated and those that cannot be tolerated. A registered dietitian or nutritionist can also help the patient plan a diet that will meet the nutritional needs for normal growth and development. Vitamins, minerals, and additional supplements may be needed to meet all nutritional needs. Epidemiology
The highest prevalence rates are seen in the Inuit populations of Greenland (5–10%), Alaska (3–7%), and Canada (about 3%). European descent prevalence ranges from 0.2% to 0.05%. There is a lower prevalence reported in African Americans and Hispanics compared to Caucasians. See also
Fructose malabsorption
Digestive system diseases
Lactose intolerance

References
External links
"Congenital sucrase-isomaltase deficiency". Genetic Home Reference. US National Library of Medicine.