Close
New

Medscape is available in 5 Language Editions – Choose your Edition here.

 

carglumic acid (Rx)Brand and Other Names:Carbaglu

 
 
 

Dosing & Uses

AdultPediatric

Dosage Forms & Strengths

dispersible tablet

  • 200mg
more...

Hyperammonemia

Initial dose range: 100-250 mg/kg/day PO/NG divided in 2-4 doses before meals; round total daily dose to nearest 100 mg  

Maintenance: Generally <100 mg/kg/day in chronic hyperammonemia

Use of other ammonia-lowering therapies (eg, diet, hemodialysis) with carglumic acid during episodes of acute hyperammonemia is recommended

Administration

Disperse 200-mg dispersible tab in 2.5 mL water (resulting in 80 mg/mL) and administer immediately

Do not swallow whole or crush

Adjust dose according to ammonia levels and symptoms

Dosage Forms & Strengths

dispersible tablet

  • 200mg
more...

Hyperammonemia

Initial dose range: 100-250 mg/kg/day PO/NG divided in 2-4 doses before meals; round total daily dose to nearest 100 mg  

Maintenance: Generally <100 mg/day in chronic hyperammonemia

Use of other ammonia-lowering therapies (eg, diet, hemodialysis) with carglumic acid during episodes of acute hyperammonemia is recommended

Other Information

Disperse 200-mg dispersible tab in 2.5 mL water (resulting in 80 mg/mL) and administer immediately

Do not swallow whole or crush

Adjust dose according to ammonia levels and symptoms

Next

Adverse Effects

Note: Adverse effects are derived from retrospective case series of 23 patients

>10%

Vomiting (26%)

Abdominal pain (17%)

Pyrexia (17%)

Tonsillitis (17%)

Anemia (13%)

Ear infection (13%)

Diarrhea (13%)

Nasopharyngitis (13%)

Headache (13%)

1-10%

Asthenia (9%)

Somnolence (9%)

Dysgeusia (9%)

Hyperhidrosis (9%)

Weakness (9%)

Pneumonia (9%)

Anorexia (9%)

Weight loss (9%)

Influenza (9%)

Rash (9%)

Previous
Next

Warnings

Contraindications

None

Cautions

Monitor serum ammonia levels and adjust dose to maintain within normal range for age

Treat acute hyperammonemia episode as life-threatening emergency

During acute hyperammonemic episodes, protein restriction and hypercaloric intake recommended to block ammonia generating catabolic pathways; when normalized, protein intake can usually be increased with goal of unrestricted protein intake

Previous
Next

Pregnancy & Lactation

Pregnancy

Pregnancy Category: C; no adequate, well controlled studies or available human data in pregnant women

Decreased survival and growth occurred in offspring born to animals that received carglumic acid at doses similar to the maximum recommended starting human dose during pregnancy and lactation

Because untreated NAGS deficiency results in irreversible neurologic damage and death, women with NAGS must remain on treatment throughout pregnancy

Lactation

Unknown whether distributed in human breast milk, breastfeeding not recommended

Excreted in rat milk, and increased mortality and impairment of body weight gain occurred in neonatal rats nursed by mothers receiving carglumic acid

Because many drugs are excreted in human milk and because of the potential for serious adverse reactions in nursing infants, human breastfeeding is not recommended since treatment is continuous and life-long for NAGS deficiency patients

Pregnancy Categories

A:Generally acceptable. Controlled studies in pregnant women show no evidence of fetal risk.

B:May be acceptable. Either animal studies show no risk but human studies not available or animal studies showed minor risks and human studies done and showed no risk.

C:Use with caution if benefits outweigh risks. Animal studies show risk and human studies not available or neither animal nor human studies done.

D:Use in LIFE-THREATENING emergencies when no safer drug available. Positive evidence of human fetal risk.

X:Do not use in pregnancy. Risks involved outweigh potential benefits. Safer alternatives exist.

NA:Information not available.

more...
Previous
Next

Pharmacology

Mechanism of Action

Structural analogue of N-acetylglutamate, which enters cells and enables activation of CPS I (first enzyme of urea cycle) in vivo; therefore, decreases hyperammonemia by converting ammonia into urea

Pharmacokinetics

Half-Life: 5.6 hr

Bioavailability: Absolute biovailability is not known

Vd: 2657 L

Peak Plasma Time: 3 hr (range: 2-4 hr)

Metabolism: portion metabolized by intestinal bacterial flora; likely end product is carbon dioxide eliminated via lungs

Clearance: 5.7 L/min (total); 290 mL/min (renal)

Excretion: unchanged in feces (60%) and urine (9%)  

Previous
Next

Images

Previous
Next

Formulary

FormularyPatient Discounts

To view formulary information first create a list of plans. Your list will be saved and can be edited at any time.

Adding plans allows you to:

  • View the formulary and any restrictions for each plan.
  • Manage and view all your plans together – even plans in different states.
  • Compare formulary status to other drugs in the same class.
  • Access your plan list on any device – mobile or desktop.

The above information is provided for general informational and educational purposes only. Individual plans may vary and formulary information changes. Contact the applicable plan provider for the most current information.

Tier Description
1 This drug is available at the lowest co-pay. Most commonly, these are generic drugs.
2 This drug is available at a middle level co-pay. Most commonly, these are "preferred" (on formulary) brand drugs.
3 This drug is available at a higher level co-pay. Most commonly, these are "non-preferred" brand drugs.
4 This drug is available at a higher level co-pay. Most commonly, these are "non-preferred" brand drugs or specialty prescription products.
5 This drug is available at a higher level co-pay. Most commonly, these are "non-preferred" brand drugs or specialty prescription products.
6 This drug is available at a higher level co-pay. Most commonly, these are "non-preferred" brand drugs or specialty prescription products.
NC NOT COVERED – Drugs that are not covered by the plan.
Code Definition
PA Prior Authorization
Drugs that require prior authorization. This restriction requires that specific clinical criteria be met prior to the approval of the prescription.
QL Quantity Limits
Drugs that have quantity limits associated with each prescription. This restriction typically limits the quantity of the drug that will be covered.
ST Step Therapy
Drugs that have step therapy associated with each prescription. This restriction typically requires that certain criteria be met prior to approval for the prescription.
OR Other Restrictions
Drugs that have restrictions other than prior authorization, quantity limits, and step therapy associated with each prescription.
Add or Remove Plans
Plans for
Select State:
Non-Medicare PlansMedicare Plans

Select a box to add or remove a plan.

Select a class to view formulary status for similar drugs

Additional Offers
Email to Patient

From:

To:

The recipient will receive more details and instructions to access this offer.

By clicking send, you acknowledge that you have permission to email the recipient with this information.

Email Forms to Patient

From:

To:

The recipient will receive more details and instructions to access this offer.

By clicking send, you acknowledge that you have permission to email the recipient with this information.

Previous
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2016 by WebMD LLC. This website also contains material copyrighted by 3rd parties.