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Important Safety Information
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How is Congenital Sucrase-Isomaltase Deficiency diagnosed?

It is easy to misdiagnose Congenital Sucrase-Isomaltase Deficiency (CSID), especially if the patient presents with nonspecific symptoms. A correct diagnosis of CSID may be delayed while other, more common causes of severe chronic diarrhea are considered.1

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CSID is largely a
clinical diagnosis.

Infants with CSID are often misdiagnosed with food allergies or intolerance to cow’s milk or soy protein. An improvement in symptoms while ingesting a casein-hydrosylate formula may be interpreted as further proof for this mistaken diagnosis when the response actually reflects the switch from dietary carbohydrates to dietary glucose polymers, which are not dependent on sucrase-isomaltase activity for digestion.1

Once the patient outgrows infancy and a larger variety of foods is added to the diet, it becomes increasingly more difficult to correctly identify sucrose as the explicit cause of gastrointestinal (GI) symptoms. In addition, there may be a high incidence of comorbidity between CSID and other enzyme deficiencies involved in carbohydrate digestion.

Such comorbidities and/or misdiagnoses may include allergic gastroenteropathy, cystic fibrosis, celiac disease, severe viral gastroenteritis, lactose intolerance, glucose-galactose intolerance, or other causes of intractable diarrhea. The presence of mild steatorrhea is commonly used to support these alternate diagnoses.1

As the table below indicates, there are several tests that may help in the diagnosis of CSID.

Diagnostic tools to assess 
sucrase enzyme activity

While no test on the market today is 100% accurate, and current tests, including genetic testing, cannot rule out secondary sucrase deficiency, several options are available to help assess the likelihood of a CSID diagnosis.

Disaccharidase (including sucrase) assay via small bowel biopsy

Contact your local Quest Diagnostics® or LabCorp representative

13C-sucrose breath test (13C-SBT)
Metabolic Solutions, 1-603-598-6960

Sucrose hydrogen-methane breath test
Aerodiagnostics™, 1-617-608-3832
Commonwealth Labs, 1-888-258-5966 
Metabolic Solutions, 1-603-598-6960
Trio-Smart®, 1-855-888-1230
Food Marble, [email protected]

Find out more: sucrosechallenge.com

Congenital Sucrase-Isomaltase Deficiency (SI Gene) Test

Contact your local genetic testing lab

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References
  1. Treem WR. Congenital Sucrase-Isomaltase Deficiency. J Pediatr Gastroenterol Nutr. 1995;21:1-14. doi:10.1097/00005176-199507000-00001
Important Safety Information
Sucraid® (sacrosidase) Oral Solution
  • Do not prescribe Sucraid® to patients known to be hypersensitive to yeast, yeast products, papain, or glycerin (glycerol).
  • Sucraid® may cause a serious allergic reaction. Patients should stop taking Sucraid® and get emergency help immediately if any of the following side effects occur: difficulty breathing, wheezing, or swelling of the face. Care should be taken when administering initial doses of Sucraid® to observe any signs of acute hypersensitivity reaction.
  • Although Sucraid® provides replacement therapy for the deficient sucrase, it does not provide specific replacement therapy for the deficient isomaltase.
  • Adverse reactions as a result of taking Sucraid® may include worse abdominal pain, vomiting, nausea, diarrhea, constipation, difficulty sleeping, headache, nervousness, and dehydration.
  • Before prescribing Sucraid® to diabetic patients, the physician should consider that Sucraid® will enable sucrose hydrolysis and the absorption of those hydrolysis products, glucose and fructose.
  • The effects of Sucraid® have not been evaluated in patients with secondary (acquired) disaccharidase deficiency.
  • DO NOT HEAT SOLUTIONS CONTAINING SUCRAID®. Do not put Sucraid® in warm or hot fluids. Do not reconstitute or consume Sucraid® with fruit juice since the acidity of the juice may reduce the enzyme activity of Sucraid®. Half of the reconstituted Sucraid® should be taken at the beginning of the meal or snack and the other half during the meal or snack.
  • Sucraid® should be refrigerated at 36°F-46°F (2°C-8°C) and should be protected from heat and light; single-use containers can be removed from refrigeration and stored at 59°F-77°F (15°C-25°C) for up to 3 days (72 hours). Refer to Instructions for Use for full information on how to take Sucraid®.
Indication
Sucraid® (sacrosidase) Oral Solution is indicated for the treatment of sucrase deficiency, which is part of congenital sucrase-isomaltase deficiency (CSID), in adult and pediatric patients 5 months of age and older.

IMPORTANT SAFETY INFORMATION:

Important Safety Information for Sucraid® (sacrosidase) Oral Solution

  • Do not prescribe Sucraid® to patients known to be hypersensitive to yeast, yeast products, papain, or glycerin (glycerol).
  • Sucraid® may cause a serious allergic reaction. Patients should stop taking Sucraid® and get emergency help immediately if any of the following side effects occur: difficulty breathing, wheezing, or swelling of the face. Care should be taken when administering initial doses of Sucraid® to observe any signs of acute hypersensitivity reaction.
  • Although Sucraid® provides replacement therapy for the deficient sucrase, it does not provide specific replacement therapy for the deficient isomaltase.
  • Adverse reactions as a result of taking Sucraid® may include worse abdominal pain, vomiting, nausea, diarrhea, constipation, difficulty sleeping, headache, nervousness, and dehydration.
  • Before prescribing Sucraid® to diabetic patients, the physician should consider that Sucraid® will enable sucrose hydrolysis and the absorption of those hydrolysis products, glucose and fructose.
  • The effects of Sucraid® have not been evaluated in patients with secondary (acquired) disaccharidase deficiency.
  • DO NOT HEAT SOLUTIONS CONTAINING SUCRAID®. Do not put Sucraid® in warm or hot fluids. Do not reconstitute or consume Sucraid® with fruit juice since the acidity of the juice may reduce the enzyme activity of Sucraid®. Half of the reconstituted Sucraid® should be taken at the beginning of the meal or snack and the other half during the meal or snack.
  • Sucraid® should be refrigerated at 36°F-46°F (2°C-8°C) and should be protected from heat and light; single-use containers can be removed from refrigeration and stored at 59°F-77°F (15°C-25°C) for up to 3 days (72 hours). Refer to Instructions for Use for full information on how to take Sucraid®.

Indication

Sucraid® (sacrosidase) Oral Solution is indicated for the treatment of sucrase deficiency, which is part of congenital sucrase-isomaltase deficiency (CSID), in adult and pediatric patients 5 months of age and older.