Pronunciation: kar-GLOO-mik AS-id
Class: Metabolic agent
- Tablets, dispersible, oral 200 mg
Carglumic acid is a synthetic structural analogue of N-acetylglutamate, which is an essential allosteric activator of carbamoyl phosphate synthetase 1 in liver mitochondria, which converts ammonia into urea.
T max is 3 h.
Vd is 2,657 L.
Metabolized by intestinal bacterial flora, resulting in carbon dioxide eliminated through the lungs.
Terminal half-life is 5.6 h. Total Cl is 5.7 L/min and renal Cl is 290 mL/min. Approximately 9% of the dose is excreted unchanged in the urine and up to 60% is excreted unchanged in the feces.
Indications and Usage
Adjunctive therapy in the treatment of acute hyperammonemia and maintenance therapy of chronic hyperammonemia caused by deficiency of the hepatic enzyme N-acetylglutamate synthase.
None well documented.
Dosage and AdministrationAdults and Children
PO 100 to 250 mg/kg/day initially divided into 2 to 4 doses for acute hyperammonemia. Maintenance dosage is usually less than 100 mg/kg/day. Titrate to a target normal plasma ammonia level for age. Coadministration of other ammonia-lowering therapies is recommended.
- Tablets should not be swallowed whole or crushed. Disperse tablets in water (minimum of 2.5 mL) immediately before use.
- Tablets may be administered orally or through a nasogastric (NG) tube.
- Divide the total daily dose into 2 to 4 doses and administer immediately before meals or feedings.
- Each divided dose should be rounded to the nearest 100 mg.
- Mix each 200 mg tablet in at least 2.5 mL of water for adults, and in 2.5 mL of water for children to yield a concentration of 80 mg/mL.
- Shake gently for quick dispersal and administer the dispersion immediately through the NG tube. Flush with additional water to clear the NG tube.
- Disperse each 200 mg tablet in at least 2.5 mL of water for adults, and in 2.5 mL of water for children to yield a concentration of 80 mg/mL.
- To ensure complete delivery of the dose, the container should be rinsed with additional volumes of water and the contents swallowed immediately.
- For children, draw up the appropriate volume of dispersion in syringe and administer immediately. Refill the syringe with a minimum of 1 to 2 mL of water and administer immediately.
Before opening tablets, store refrigerated between 36° and 46°F. After opening the container, do not refrigerate or store above 86°F. Protect from moisture. Write the date of opening on the container and discard 1 mo after first opening.
None well documented.
Asthenia, headache, somnolence.
Abdominal pain, diarrhea, dysgeusia, vomiting.
Anemia, decreased hemoglobin.
Anorexia, decreased weight.
Nasopharyngitis, pneumonia, tonsillitis.
Ear infection, infection, influenza, pyrexia.
To assess patient response to treatment, ongoing monitoring of plasma ammonia levels, neurological status, laboratory tests, and clinical response in patients is recommended. Maintain plasma ammonia levels within the normal range for age via individual dosage adjustment.
Category C .
Safety and efficacy have been established.
Treatment may require dialysis, preferably hemodialysis, to remove a large burden of ammonia. Uncontrolled hyperammonemia can rapidly result in brain injury/damage or death, and prompt use of all therapies necessary to reduce plasma ammonia levels is essential.
Protein restriction and hypercaloric intake is recommended to block ammonia-generating catabolic pathways during acute hyperammonemic episodes. When plasma ammonia levels have normalized, protein intake can usually be increased with the goal of unrestricted protein intake.
Increased body temperature, increased bronchial secretion, profuse sweating, restlessness, tachycardia.
- Advise patients to administer the tablets immediately before meals or feedings.
- Instruct patients that the tablets should not be swallowed whole or crushed and that each tablet should be dispersed in a minimum of 2.5 mL of water.
- Inform patients that not all of the tablet will dissolve completely in water and undissolved particles of the tablet may remain in the mixing container. The mixing container should be rinsed with additional volumes of water and the contents swallowed immediately.
- Instruct patients on the proper storage of the tablets. Before opening the tablets, they should be stored in a refrigerator and kept in a tightly closed container to protect from moisture. After the first opening of the container, do not refrigerate the tablets or store above 86°F. Write the date of opening on the tablet container and discard 1 mo after the first opening.
- Inform patients that when plasma ammonia levels have normalized, dietary protein intake can usually be increased with the goal of unrestricted protein intake.
- Advise patients that breast-feeding is not recommended.
- Inform patients that the most common adverse reactions are abdominal pain, anemia, diarrhea, ear infection, headache, nasopharyngitis, pyrexia, tonsillitis and vomiting.
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