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About CSID

How Common Is CSID?

How Common Is CSID?

Congenital Sucrase-Isomaltase Deficiency (CSID) is considered a rare disease. Because its symptoms overlap with more common gastrointestinal disorders, it is often difficult to diagnose. It is likely that the true prevalence of CSID is underestimated and that numerous individuals suffering from chronic diarrhea and abdominal pain may have CSID but remain undiagnosed. Many of those with CSID may wait many years for a diagnosis because the incidence is so rare and the currently accepted diagnostic tools are invasive procedures. Recent clinical and genetic studies suggest that CSID is a more common disease than previously thought.1-11

In the past, clinical studies of small populations have found a high prevalence of CSID: 5% in indigenous Greenlanders, 7% of Canadian Inuit populations, and 3% in indigenous Alaskans.12-14 At the same time, estimates of the prevalence of CSID in non-Hispanic White North Americans was lower, with a prevalence of approximately 2 in 1000 or 0.2%.15 In other studies, the prevalence was even lower in African-Americans.16 CSID occurs with equal frequency in males and females.

While CSID is a rare disease in the general population, data suggests sucrase deficiency may be more common in patients with GI symptoms. An early study of sucrase enzyme activity in biopsied tissue from 217 Americans who underwent an endoscopic biopsy estimated an 8.9% prevalence of individuals with a level of sucrase activity below the lower limit for the normal population.15 The most recent and largest study to estimate CSID prevalence was a review of clinical findings for 27,875 patients who were referred by a gastroenterologist for endoscopic biopsy and enzyme analysis to diagnose the cause of their chronic gastrointestinal symptoms. In this study, 9.3% were found to have deficient sucrase activity.17

Additional studies are underway and could help to more accurately determine the prevalence of CSID. It is possible that a significant proportion of affected children and adults are not being tested and therefore are not being diagnosed with CSID.

References
  1. Treem WR. Congenital sucrase-isomaltase deficiency. J Pediatr Gastroenterol Nutr. 1995;21(1):1-14. doi:10.1097/00005176-199507000-00001
  2. Treem WR. Clinical aspects and treatment congenital sucrase-isomaltase deficiency. J Pediatr Gastroenterol Nutr. 2012;55(suppl 2):S7-13. doi:10.1097/01.mpg.0000421401.57633.90
  3. Alfalah M, Keiser M, Leeb T, et al. Compound heterozygous mutations affect protein folding and function in patients with congenital sucrase-isomaltase deficiency. Gastroenterology. 2009;136(3):883-92. doi:10.1053/j.gastro.2008.11.038
  4. Gericke B, Amiri M, Naim HY. The multiple roles of sucrase-isomaltase in the intestinal physiology. Mol Cell Pediatr. 2016;3(1):2. doi:10.1186/s40348-016-0033-y
  5. Jacob R, Zimmer KP, Schmitz J, et al. Congenital sucrase-isomaltase deficiency arising from cleavage and secretion of a mutant form of the enzyme. J Clin Invest. 2000;106(2):281-7. doi: 10.1172/JCI9677
  6. Keiser M, Alfalah M, Pröpsting MJ, et al. Altered folding, turnover, and polarized sorting act in concert to define a novel pathomechanism of congenital sucrase-isomaltase deficiency. J Biol Chem. 2006;281(20):14393-9. doi: 10.1074/jbc.M513631200
  7. Naim HY, Heine M, Zimmer KP. Congenital sucrase-isomaltase deficiency: heterogeneity of inheritance, trafficking, and function of an intestinal enzyme complex. J Pediatr Gastroenterol Nutr. 2012;55(suppl 2):S13-20. doi: 10.1097/01.mpg.0000421402.57633.4b
  8. Ritz V, Alfalah M, Zimmer KP, et al. Congenital sucrase-isomaltase deficiency because of an accumulation of the mutant enzyme in the endoplasmic reticulum. Gastroenterol. 2003;125(6):1678-85. doi: 10.1053/j.gastro.2003.09.022
  9. Sander P, Alfalah M, Keiser M, et al. Novel mutations in the human sucrase-isomaltase gene (SI) that cause congenital carbohydrate malabsorption. Human Mutat. 2006;27(1):119. doi:10.1002/humu.9392
  10. Spodsberg N, Jacob R, Alfalah M, et al. Molecular basis of aberrant apical protein transport in an intestinal enzyme disorder. J Biol Chem. 2001;276(26):23506-10. doi:10.1074/jbc.C100219200
  11. Uhrich S, Wu Z, Huang J, Scott CR. Four mutations in the SI gene are responsible for the majority of clinical symptoms of CSID. J Pediatr Gastroenterol Nutr. 2012; 55(2):S34-5. doi: 10.1097/01.mpg.0000421408.65257.b5
  12. Gudman-Høyer E, Fenger HJ, Kern-Hansen P, Madsen PR. Sucrase deficiency in Greenland. Incidence and genetic aspects. Scand J Gastroenterol. 1987;22(1):24-8. doi: 10.3109/00365528708991851
  13. Ellestad-Sayed JJ, Haworth JC, Hildes JA. Disaccharide malabsorption and dietary patterns in two Canadian Eskimo communities. Am J Clin Nutr. 1978;31(8):1473-8. doi: 10.1093/ajcn/31.8.1473
  14. Bell RR, Draper HH, Bergan JG. Sucrose, lactose, and glucose tolerance in northern Alaskan Eskimos. Am J Clin Nutr. 1978;26(11):1185-90. doi: 10.1093/ajcn/26.11.1185
  15. Peterson ML, Herber R. Intestinal sucrase deficiency. Trans Assoc Am Physicians. 1967:80:275-83.
  16. Welsh JD, Poley JR, Bhatia M, Stevenson DE. Intestinal disaccharidase activities in relation to age, race, and mucosal damage. Gastroenterology. 1978;75(5):847-55.
  17. Nichols BL Jr, Adams B, Roach CM, Ma CX, Baker SS. Frequency of sucrase deficiency in mucosal biopsies. J Pediatr Gastroenterol Nutr. 2012;55(suppl 2):S28-30. doi: 10.1097/01.mpg.0000421405.42386.64

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IMPORTANT SAFETY INFORMATION:

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Important Safety Information for Sucraid® (sacrosidase) Oral Solution

  • Tell your doctor if you are allergic to, have ever had a reaction to, or have ever had difficulty taking yeast, yeast products, papain, or glycerin (glycerol).
  • Sucraid® may cause a serious allergic reaction. If you notice any swelling or have difficulty breathing, get emergency help right away.
  • Sucraid® does not break down some sugars that come from the digestion of starch. You may need to restrict the amount of starch in your diet. Your doctor will tell you if you should restrict starch in your diet.
  • Tell your doctor if you have diabetes, as your blood glucose levels may change if you begin taking Sucraid®. Your doctor will tell you if your diet or diabetes medicines need to be changed.
  • Some patients treated with Sucraid® may have worse abdominal pain, vomiting, nausea, or diarrhea. Constipation, difficulty sleeping, headache, nervousness, and dehydration have also occurred in patients treated with Sucraid®. Check with your doctor if you notice these or other side effects.
  • Sucraid® has not been tested to see if it works in patients with secondary (acquired) sucrase deficiency.
  • NEVER HEAT SUCRAID® OR PUT IT IN WARM OR HOT BEVERAGES OR INFANT FORMULA. Do not mix Sucraid® with fruit juice or take it with fruit juice. Take Sucraid® as prescribed by your doctor. Normally, half of the dose of Sucraid® is taken just before a meal or snack and the other half is taken 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®.

You are encouraged to report negative side effects of prescription drugs to the FDA. Visit www.FDA.gov/medwatch or call 1-800-FDA-1088.

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.