carglumic acid


Also found in: Wikipedia.

carglumic acid

(car-gloo-mik as-id) ,

Carbaglu

(trade name)

Classification

Therapeutic: hyperammonemia treatments
Pharmacologic: enzyme activators
Pregnancy Category: C

Indications

Treatment (acute/maintenance) of hyperammonemia due to deficiency of hepatic enzyme N-acetylglutamate synthase (NAGS). During acute hyperammonemia, additional treatments should be used including alternate pathway medications, hemodialysis, and protein restriction.

Action

Serves as an analogue of N-acetyl glutamate (NAG), the natural activator of cabamoyl phosphate synthetase 1 (CPS 1) in the liver. CPS 1 helps to convert ammonia into urea. Carglumic acid replaces NAG in NAGS deficiency patients by activating CPS 1.

Therapeutic effects

↓ ammonia levels.

Pharmacokinetics

Absorption: Some absorption follows oral administration; bioavailability unknown.
Distribution: .
Metabolism and Excretion: Some metabolism by intestinal bacteria; 9% excreted unchanged in urine, 60% excreted unchanged in feces. Metabolic end product (CO2) eliminated via lungs.
Half-life: 5.6 hr.

Time/action profile (↓ ammonia levels)

ROUTEONSETPEAKDURATION
POwithin 24 hr3 daysunknown

Contraindications/Precautions

Contraindicated in: Lactation: Breastfeeding not recommended.
Use Cautiously in: Obstetric: Due to risk of neurologic complications in NAGS patients, carglumic acid must be used throughout pregnancy.

Adverse Reactions/Side Effects

Central nervous system

  • headache (most frequent)
  • drowsiness
  • weakness

Ear, Eye, Nose, Throat

  • nasopharyngitis (most frequent)

Gastrointestinal

  • abdominal pain (most frequent)
  • diarrhea (most frequent)
  • vomiting (most frequent)
  • abnormal taste

Dermatologic

  • rash

Hematologic

  • anemia (most frequent)

Metabolic

  • ↓ weight

Miscellaneous

  • infections (most frequent)
  • fever (most frequent)

Interactions

Drug-Drug interaction

None noted.

Route/Dosage

Oral (Adults) 100–250 mg/kg/day initially, given in 2–4 divided doses immediately before meals/feedings, adjusted to maintain normal plasma ammonia levels based on age. Dose should be rounded to the nearest 100 mg.
Oral (Children) 100–250 mg/kg/day initially, given in 2–4 divided doses immediately before meals/feedings.

Availability

Tablets: 200 mg

Nursing implications

Nursing assessment

  • Assess for hyperammonemia (neurological status, plasma ammonia levels) during therapy.
  • Lab Test Considerations: Monitor plasma ammonia levels periodically during therapy. Maintain within normal range for age via individual dose adjustment.
    • May cause ↓ hemoglobin.

Potential Nursing Diagnoses

Activity intolerance

Implementation

  • Concommitant administration of other ammonia-lowering therapies is recommended.
  • Oral: Disperse each 250 mg tablet in at least 2.5 mL of water immediately before use and take immediately; do not crush or swallow whole. Tablets may not dissolve completely in water; undissolved particles may remain in mixing container. Rinse mixing container with additional volumes of water to ensure complete delivery of dose; swallow immediately. Administer via oral syringe or nasogastric tube.
    • Refrigerate before opening. After first opening of container, do not refrigerate. Keep container tightly closed to protect from moisture. Write date of opening on tablet container; discard 1 mo after first opening. Do not use after expiration date stated on container.

Patient/Family Teaching

  • Instruct patient to take carglumic acid as directed.
  • Advise patient that during acute hyperammonemic episodes, protein restriction and hypercaloric intake is recommended to block ammonia generating catabolic pathways. When plasma ammonia levels have normalized, protein intake can usually be ↑ with goal of unrestricted protein intake.
  • Advise female patients to notify health care professional if pregnancy is planned or suspected or if breastfeeding Due to risk of neurologic complications in NAGS patients, carglumic acid must be used throughout pregnancy.

Evaluation/Desired Outcomes

  • Normalization of plasma ammonia levels.
References in periodicals archive ?
MRRSE Research has compiled a new comprehensive report on the global Carglumic Acid Market for the forecast period 2017 to 2026.
Summary: MRRSE Research has compiled a new comprehensive report on the global Carglumic Acid Market for the forecast period 2017 to 2026.
Carbamoyl glutamate (carglumic acid) was administered by enteral route at a dose of 25 mg/kg for six hours after the 100 mg/kg loading.
announces the successful development of a new, fast dissolving formulation of Carglumic acid tablets stable at room temperature.
The three endocrine products are carglumic acid (Carbaglu; C) for treatment of hyperammonemia due to the deficiency of the hepatic enzyme N-acetylglutamate synthase (NAGS); tesamorelin (Egrifta; X) a growth hormone--releasing factor analog for reduction of excess abdominal fat in HIV-infected patients; and velaglucerase alfa (VPRIV; B) given for long-term enzyme replacement in patients with Gaucher disease.
Italian pharmaceutical company Recordati (Milan:REC.MI)A declared on Friday that Carbaglu (carglumic acid) has been approved in the United States.
[UKPRwire, Mon Jun 17 2019] MRRSE Research has compiled a new comprehensive report on the global Carglumic Acid Market for the forecast period 2017 to 2026.
[ClickPress, Mon Jun 17 2019] MRRSE Research has compiled a new comprehensive report on the global Carglumic Acid Market for the forecast period 2017 to 2026.
[USPRwire, Fri Feb 22 2019] Market Research Reports Search Engine (MRRSE) has recently updated its massive report catalogue by adding a fresh study titled " Carglumic Acid Market Examination & Future Forecast Analyzed till 2026 - MRRSE ".
Tender of 4 lots broken down as follows: - lot 1: carglumic acid, - lot 2: daptomycin, - lot 3: bortezomib, - lot 4: pemetrexed.
Summary: The report's beginning chapter presents an executive summary of global market for carglumic acid. A precis of global carglumic acid market is delivered in this chapter.
@ https://www.futuremarketinsights.com/askus/rep-gb-6359 In terms of product type, the global amino acid metabolism disorder treatment market has been segmented into arginine, folic acid, vitamin B6 and B12, thiamine, vitamin D, betaine, carglumic acid, sapropterin dihydrocholoride, and others.