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Carglumic Acid Dosage

Medically reviewed on May 29, 2018.

Applies to the following strengths: 200 mg

Usual Adult Dose for:

Usual Pediatric Dose for:

Additional dosage information:

Usual Adult Dose for Hyperammonemia

Initial dose: 100 to 250 mg/kg/day orally divided into 2 to 4 doses (rounded to the nearest 100 mg)
Maintenance dose: 10 to 100 mg/kg/day orally divided into 2 to 4 doses (rounded to the nearest 100 mg)

Therapeutic monitoring: Plasma ammonia levels should be closely monitored; titrate dose to maintain plasma ammonia levels within the normal range

Comments:
-Therapy should be initiated as soon as the diagnosis of N-acetylglutamate synthase (NAGS) deficiency is suspected.
-During acute episodes, concomitant administration with other ammonia lowering therapies, such as alternate pathway medications, hemodialysis, and dietary protein restriction, is recommended.
-During maintenance therapy, concomitant administration with other ammonia lowering therapies and dietary protein restriction may be needed based on plasma ammonia levels.

Uses: As adjunctive therapy for the treatment of acute hyperammonemia due to the deficiency of the hepatic enzyme N-acetylglutamate synthase (NAGS).

Usual Pediatric Dose for Hyperammonemia

Initial dose: 100 to 250 mg/kg/day orally divided into 2 to 4 doses (rounded to the nearest 100 mg)
Maintenance dose: 10 to 100 mg/kg/day orally divided into 2 to 4 doses (rounded to the nearest 100 mg)

Therapeutic monitoring: Plasma ammonia levels should be closely monitored; titrate dose to maintain plasma ammonia levels within the normal range for age, taking into account clinical condition (e.g. nutritional requirements, protein intake, growth parameters, etc.)

Comments:
-Therapy should be initiated as soon as the diagnosis of N-acetylglutamate synthase (NAGS) deficiency is suspected; this may be as soon as birth.
-During acute episodes, concomitant administration with other ammonia lowering therapies, such as alternate pathway medications, hemodialysis, and dietary protein restriction, is recommended.
-During maintenance therapy, concomitant administration with other ammonia lowering therapies and dietary protein restriction may be needed based on plasma ammonia levels.

Uses: As adjunctive therapy for the treatment of acute hyperammonemia due to the deficiency of the hepatic enzyme N-acetylglutamate synthase (NAGS).

Renal Dose Adjustments

Data not available

Liver Dose Adjustments

Data not available

Precautions

CONTRAINDICATIONS:
-None

Safety and efficacy have not been established in geriatric population.

Consult WARNINGS section for additional precautions.

Dialysis

Data not available

Other Comments

Administration advice:
-Tablets must be dispersed in water; do not swallow whole or crushed
-Take orally prior to meals or feedings
-Follow preparation instructions below; use in foods or liquids other than water has not been studied and is not recommended

Oral Administration:
-Add approximately 2.5 mL of water in cup for each 200-mg tablet and each half (100-mg) tablet needed for the prescribed dose
-Add tablet(s) to the water and carefully stir tablet and water mixture
-Swallow mixture immediately; rinse cup with additional water and swallow immediately; repeat as needed until no pieces of tablet are left in cup
-The mixture has a slightly acid taste (slightly sour)

Nasogastric (NG) Tube Administration:
-Add approximately 2.5 mL of water in cup for each 200-mg tablet and each half (100-mg) tablet needed for the prescribed dose
-Add tablet(s) to the water and carefully stir tablet and water mixture
-Draw mixture into a catheter-tip syringe
-Administer mixture immediately through the NG tube; flush immediately with 1 or 2 mL of additional water to clear NG tube; flush tube as needed until no pieces of tablet are left in syringe or NG tube

Oral Syringe Administration:
-Add approximately 2.5 mL of water in cup for each 200-mg tablet and each half (100-mg) tablet needed for the prescribed dose
-Add tablet(s) to the water and carefully stir tablet and water mixture
-Draw mixture into oral syringe
-Administer mixture immediately; refill oral syringe with a minimum of 1 to 2 mL of additional water and administer immediately; flush oral syringe until no pieces of tablet are left in syringe

Storage requirements:
Before use: Store in original container in the refrigerator 36F to 46F (2C to 8C)
After opening container: Store at room temperature; do not refrigerate
--Date container with date of opening; discard 1 month after opening
--Keep container tightly closed between openings to protect from moisture

General:
-This drug should be managed by a physician and medical team experienced in metabolic disorders.

Monitoring:
-Monitor plasma ammonia levels

Patient advice:
-Read the US FDA-approved patient labeling (Instructions for Use).
-Women should be advised not to breastfeed during use.

Further information

Always consult your healthcare provider to ensure the information displayed on this page applies to your personal circumstances.

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