Sucrose intolerance

Sucrose intolerance, also known as Congenital Sucrase-Isomaltase Deficiency (CSID), is a rare genetic disorder that affects a person's ability to digest certain sugars. People with this condition cannot break down the sugars sucrose and maltose, or other carbohydrates made of these sugar molecules.

Causes

Sucrose intolerance is caused by mutations in the SI gene. This gene provides instructions for producing an enzyme called sucrase-isomaltase, which is found in the small intestine. The enzyme is responsible for breaking down sucrose and maltose into simpler sugars, which can then be absorbed into the bloodstream. When the SI gene is mutated, the body produces little or no sucrase-isomaltase, leading to sucrose intolerance.

Symptoms

The symptoms of sucrose intolerance can vary widely, but they often include abdominal pain, bloating, gas, and diarrhea. These symptoms typically occur after eating foods that contain sucrose or maltose. In severe cases, the condition can lead to malnutrition and weight loss.

Diagnosis

Sucrose intolerance is typically diagnosed through a combination of symptom history, physical examination, and medical tests. These tests may include a hydrogen breath test, which measures the amount of hydrogen in a person's breath after they consume a sugar solution. High levels of hydrogen suggest that the sugar is not being properly digested.

Treatment

There is currently no cure for sucrose intolerance, but the condition can be managed through dietary changes. People with this condition are typically advised to avoid foods that contain sucrose and maltose. In some cases, they may also need to take enzyme replacement therapy to help their bodies break down these sugars.

See also

• Lactose intolerance
• Fructose malabsorption
• Glucose-galactose malabsorption

References

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