Enzyme, Gastrointestinal

Oral replacement therapy in sucrase deficiency, as seen in congenital sucrase-isomaltase deficiency (CSID)

C

Animal studies have not been conducted. Should be administered to a pregnant woman only when indicated; compatible with breast-feeding

Hypersensitivity to yeast, yeast products, or glycerin

Hypersensitivity reactions to sacrosidase, including bronchospasm, have been reported. Administer initial doses in a setting where acute hypersensitivity reactions may be treated within a few minutes. Skin testing for hypersensitivity may be performed prior to administration to identify patients at risk.

1% to 10%: Gastrointestinal: Abdominal pain, vomiting, nausea, diarrhea, constipation

<1%:

Central nervous system: Insomnia, headache, nervousness

Endocrine & metabolic: Dehydration

Respiratory: Bronchospasm

Miscellaneous: Hypersensitivity reaction

Symptoms may include epigastric pain, drowsiness, lethargy, nausea, and vomiting; gastrointestinal bleeding may occur. Rare manifestations include hypertension, respiratory depression, coma, and acute renal failure. Treatment is symptomatic and supportive. Forced diuresis, hemodialysis, and/or urinary alkalinization are not likely to be useful.

Drug-drug interactions have not been evaluated

Store under refrigeration at 4°C to 8°C (36°F to 46°F); protect from heat or light

Sacrosidase is a naturally occurring gastrointestinal enzyme which breaks down the disaccharide sucrose to its monosaccharide components. Hydrolysis is necessary to allow absorption of these nutrients.

Absorption: Sacrosidase is metabolized in the gastrointestinal tract to individual amino acids, which may be absorbed

Oral:

Children >15 kg and Adults: 17,000 int. units (2 mL) per meal or snack

Doses should be diluted with 2-4 oz of water, milk, or formula with each meal or snack. Approximately one-half of the dose may be taken before, and the remainder of a dose taken at the completion of each meal or snack.

May be inactivated or denatured if administered with fruit juice, warm or hot food or liquids. Since isomaltase deficiency is not addressed by supplementation of sacrosidase, adherence to a low-starch diet may be required.

No information available to require special precautions

No effects or complications reported

Use exactly as directed. Dilute dose in 2-4 ounces of water, milk, or formula; do not dilute with fruit juice or warm or cold liquids. Take half the dose at beginning of meal and half the dose at end of meal. Maintain adequate fluid intake (2-3 L/day of fluids unless instructed to restrict fluid intake). Follow prescribers recommended diet exactly. You may experience headache or nervousness (use caution when driving or engaging in tasks requiring alertness until response to drug is known); nausea, vomiting, or GI disturbance (frequent small meals, frequent mouth care, chewing gum, or sucking hard candy may help). Report immediately skin rash or difficulty breathing; persistent vomiting, abdominal pain, or blood in stools; change in CNS status (depression, agitation, lethargy); or other adverse response. Pregnancy precautions: Inform prescriber if you are or intend to be pregnant.

Solution, oral: 8500 int. units per mL

Treem WR, McAdams L, Stanford L, et al, "Sacrosidase Therapy for Congenital Sucrose-Isomaltase Deficiency," J Pediatr Gastroenterol Nutr, 1999, 28(2):137-42.