Sacrosidase (Systemic)
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VA CLASSIFICATION
Primary: HS451
Commonly used brand name(s): Sucraid.
Note: For a listing of dosage forms and brand names by country availability, see Dosage Forms section(s).
Category:
Enzyme replenisher—
Indications
Accepted
Sucrase deficiency (treatment)—Sacrosidase is indicated as oral replacement therapy of genetically determined sucrase deficiency, which is part of congenital sucrase-isomaltase deficiency (CSID) {01}.
Note: CSID is a chronic, autosomal recessive, inherited, phenotypically heterogenous disease with very variable enzyme activity. It is usually characterized by a complete or almost complete lack of endogenous sucrase activity, a very marked reduction in isomaltase activity, a moderate decrease in maltase activity, and normal lactase concentrations. {01}
Sacrosidase provides specific replacement therapy for deficient sucrase, but not for deficient isomaltase. As a result, the need for restriction of starch in the diet to maximize reduction in symptoms should be evaluated. {01}
Definitive diagnosis of CSID is accomplished with a small bowel biopsy (measurement of intestinal disaccharidases). A breath hydrogen test (measurement of expired breath hydrogen under controlled conditions following a sucrose challenge, to measure excess hydrogen excreted in exhalation) will help confirm the diagnosis but is prone to false negatives when used alone. An oral sucrose tolerance test has a high incidence of false positives. Differential urinary disaccharide testing appears to have good agreement with the small intestinal biopsy. {01}
If a small bowel biopsy or breath hydrogen test is not clinically appropriate, or is difficult or inconvenient to perform, a short-term trial (e.g., 1 week) of sacrosidase may be warranted to assess response in suspected cases of sucrase deficiency {01}.
The effects of sacrosidase in patients with secondary (acquired) disaccharidase deficiencies have not been evaluated {01}.
Pharmacology/Pharmacokinetics
Physicochemical characteristics:
Source—
Derived from baker's yeast (Saccharomyces cerevisiae ) {01}. Consists of 513 amino acids {01}.
Molecular weight—
Glycosylated monomer: Apparent—100,000 grams per mole (range 66,000 to 116,000) {01}
Note: There is evidence that sacrosidase exists in solution as a monomer, dimer, tetramer, and octomer ranging from 100,000 grams per mole to 800,000 grams per mole {01}.
Isoelectric point
4 {01}.
Mechanism of action/Effect:
Sacrosidase replaces deficient sucrase {01}. Sucrase is produced naturally in the brush border of the small intestine, primarily the distal duodenum and jejunum, and hydrolyzes the disaccharide sucrose into its component monosaccharides (glucose and fructose). Isomaltase, which sacrosidase does not replace, breaks down disaccharides from starch into simple sugars. {01}
When endogenous human sucrase is absent, sucrose is not metabolized. Unhydrolyzed sucrose and starch are not absorbed from the intestine, leading to osmotic retention of water, which may result in loose stools. In addition, unabsorbed sucrose in the colon is fermented by bacterial flora to produce increased amounts of hydrogen, methane, and water, possibly leading to excessive gas, bloating, abdominal cramps, nausea, and vomiting. Chronic malabsorption of disaccharides may result in malnutrition, failure to thrive, and falling behind in expected growth and development curves. Previously, the treatment of CSID has required the continual use of a strict sucrose-free diet. {01}
Precautions to Consider
Cross-sensitivity and/or related problems
Patients sensitive to yeast, yeast products, or glycerin (glycerol) also may be sensitive to sacrosidase {01}.
Carcinogenicity
Studies to determine the carcinogenic potential of sacrosidase have not been done in humans or animals {01}.
Mutagenicity
Studies to determine the mutagenic potential of sacrosidase have not been done in humans or animals {01}.
Pregnancy/Reproduction
Fertility—
Studies have not been done in humans or animals {01}.
Pregnancy—
Studies have not been done in humans or animals {01}.
Although sacrosidase is not expected to cause fetal harm, risk-benefit should be considered before it is given to pregnant women {01}.
FDA Pregnancy Category C {01}.
Breast-feeding
Sacrosidase is broken down in the gastrointestinal tract, and its component amino acids and peptides are then absorbed as nutrients {01}.
Pediatrics
Studies performed in children as young as 5 months of age have not demonstrated pediatrics-specific problems that would limit the usefulness of sacrosidase in children {01}.
Medical considerations/Contraindications
The medical considerations/contraindications included have been selected on the basis of their potential clinical significance (reasons given in parentheses where appropriate)— not necessarily inclusive (» = major clinical significance).
Risk-benefit should be considered when the following medical problems exist
» Diabetes mellitus{01} (because sacrosidase enables the products of sucrose hydrolysis, glucose and fructose, to be absorbed, careful planning of the diet is recommended {01})
» Sensitivity to sacrosidase, yeast, yeast products, or glycerin (glycerol){01}
Side/Adverse Effects
Note: In clinical trials, most side/adverse effects of sacrosidase were minor and associated with the underlying disease {01}.
One case of a serious hypersensitivity reaction (wheezing) has been reported in a child with a history of asthma {01}.
The following side/adverse effects have been selected on the basis of their potential clinical significance (possible signs and symptoms in parentheses where appropriate)—not necessarily inclusive:
Those indicating need for medical attention only if they continue or are bothersome
Incidence less frequent
Abdominal or stomach pain{01}
constipation{01}
dehydration (increased thirst)
diarrhea{01}
headache{01}
insomnia{01} (trouble in sleeping)
nausea and vomiting
nervousness{01}
Note: Abdominal pain and diarrhea can be manifestations of the disease being treated (CSID).
Overdose
For more information on the management of overdose or unintentional ingestion, contact a Poison Control Center (see Poison Control Center Listing ).
Clinical effects of overdose
No cases of overdose have been reported {01}.
Patient Consultation
As an aid to patient consultation, refer to Advice for the Patient, Sacrosidase (Systemic) .
In providing consultation, consider emphasizing the following selected information (» = major clinical significance):
Before using this medication
» Conditions affecting use, especially:
Sensitivity to sacrosidase
Other medical problems, especially diabetes mellitus
Proper use of this medication
» Taking dose with meals or snacks; taking half of dose at beginning of meal or snack and remainder at end
» Taking mixed in 2 to 4 ounces of milk, water, or infant formula; not heating or mixing with hot beverages; not mixing with fruit juice
» Importance of following special diet if ordered by physician
» Proper dosing
Missed dose: Not taking missed dose at all; not doubling doses
» Proper storage
General Dosing Information
It is recommended that sacrosidase oral solution be diluted with 2 to 4 ounces of milk, water, or infant formula for administration {01}. Dilution in fruit juice is not recommended because the acidity may reduce the enzyme activity {01}.
It is recommended that the beverage in which sacrosidase oral solution is diluted be at room temperature {01}. The beverage should not be heated after addition of sacrosidase because of the potential for decreased potency {01}.
Oral Dosage Forms
SACROSIDASE ORAL SOLUTION
Usual adult and adolescent dose
Sucrase deficiency
Oral, 17,000 International Units (IU) (diluted with two to four ounces of water or milk) with each meal or snack {01}.
Note: It is recommended that approximately one half of the dose be taken at the beginning of each meal or snack and the remainder of the dose be taken at the end {01}.
Usual pediatric dose
Sucrase deficiency
Children weighing more than 15 kg (33 lbs): Oral, 17,000 IU (diluted with two to four ounces of water or milk) with each meal or snack {01}.
Children weighing up to 15 kg (33 lbs): Oral, 8500 IU (diluted with two to four ounces of water or milk) with each meal, snack, or infant formula {01}.
Note: It is recommended that approximately one half of the dose be taken at the beginning of each meal or snack and the remainder of the dose taken at the end {01}.
Strength(s) usually available
U.S.—
8500 International Units (IU) per mL (Rx) [Sucraid (glycerol 50%)]
Note: 22 drops of the oral solution from the container tip are equivalent to 1 mL {01}.
Packaging and storage:
Store between 2 and 8 °C (36 and 46 °F). Protect from light. Protect from heat. {01}
Preparation of dosage form:
Fully soluble in and may be mixed in water, milk, or infant formula {01}. Sacrosidase oral solution should not be heated or mixed in warm or hot liquids {01}.
Stability:
It is recommended that the oral solution be discarded 4 weeks after the container is first opened because of the potential for bacterial growth {01}.
Incompatibilities:
It is recommended that sacrosidase not be mixed or consumed with fruit juice because the acidity of the juice may reduce the enzyme activity {01}.
Developed: 01/12/1999
References
- Sucraid package insert (Orphan Medical—US), Rev 4/98.
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