• Brand Name – Carbaglu
  • Active Ingredient – carglumic acid
  • Strength – 200mg tablets
  • Originator Name/Marketing-authorisation Holder – Recordati Rare Diseases 


What is Carbaglu?

Carbaglu tablets contain 200 mg of carglumic acid. Carglumic acid is the active substance, is a Carbamoyl Phosphate Synthetase 1 activator and is soluble in boiling water, slightly soluble in cold water, and practically insoluble in organic solvents. Croscarmellose sodium, silica colloidal anhydrous, hypromellose, microcrystalline cellulose, sodium lauryl sulfate, and sodium stearyl fumarate are inactive ingredients of the medicine.

What are the uses of Carbaglu?

Carbaglu tablets are used in the treatment of hyperammonemia. There are two types of hyperammonemia:

1. Acute hyperammonemia

Professionals suggest Carbaglu as adjunctive therapy in pediatric and adult patients to treat acute hyperammonemia due to the deficiency of the hepatic enzyme N-acetyl glutamate synthase (NAGS). During acute hyperammonemia episodes, Clinicians recommend concomitant administration of Carbaglu with other ammonia lowering therapies, such as alternate pathway medications, hemodialysis, and dietary protein restriction.

2. Chronic hyperammonemia

Carbaglu is recommended as maintenance therapy in pediatric and adult patients to treat chronic hyperammonemia due to the hepatic enzyme N-acetyl glutamate synthase (NAGS) deficiency. During maintenance therapy, doctors may need other related ammonia lowering therapies and protein restriction based on plasma ammonia levels.

Dosage form and strength

Carbaglu tablet is a 200 mg white and elongated for oral suspension only.

Start the Carbaglu as soon as NAGS deficiency is diagnosed, which may be as soon as at birth.
Initial Dosage: The initial recommended daily dosage of Carbaglu in pediatric and adult patients for acute hyperammonemia is 100 mg/kg to 250 mg/kg divided into 2-4 doses and rounded to the nearest 100 mg (half of a Carbaglu tablet). Experts recommend the administration of other related ammonia lowering therapies.

Maintenance Dosage: The daily recommended maintenance dosage of Carglumic acid tablets in pediatric and adult patients is 10 mg/kg to 100 mg/kg divided into 2-4 doses and rounded to the nearest 100 mg (half Carglumic acid tablet).

Therapeutic Monitoring: Closely monitor the plasma ammonia levels. Then titrate the Carbaglu dosage to maintain the plasma ammonia level within the normal range according to the patient’s age, considering their clinical condition, e.g., nutritional requirements, protein intake, growth parameters, etc.


A report on the overdose states that “one patient was treated with 650 mg/kg/day of carglumic acid developed symptoms identified as a monosodium glutamate intoxication-like syndrome: tachycardia, profuse sweating, increased bronchial secretion, increased body temperature, and restlessness”.
These symptoms were resolved upon reduction of the dose. Repeated oral dosage of carglumic acid at 2000 mg/kg/day was fatal to most neonatal rats within 2-3 days of treatment.
A single oral administration of the Carglumic acid tablet was not fatal in adult rats at doses up to 2800 mg/kg.

Side effects

Every medicine has some side effects, but everyone doesn’t need to experience them. Intake of Carbaglu tablets also has some side effects that can be common or severe. And these are categorized as:

Vomiting, abdominal pain, pyrexia, tonsillitis, etc., are common side effects.

Anemia, Diarrhea, Ear infection, Infections, Nasopharyngitis, Hemoglobin decreased, Headache, etc., are uncommon side effects.

Dysgeusia, Asthenia, Hyperhidrosis, Influenza, Pneumonia, Weight decreased, Anorexia, Somnolence, Rash, etc., are rare side effects experienced by few patients only.

Precautions and Warnings

Professionals treat an episode of acute symptomatic hyperammonemia as a life-threatening emergency. Hyperammonemia treatment may require dialysis, preferably hemodialysis, to remove a large quantity of ammonia. Uncontrolled hyperammonemia can quickly result in death or brain injury/damage and prompt use of all therapies necessary to decrease plasma ammonia levels.

Healthcare professionals advise managing hyperammonemia due to NAGS deficiency in coordination with experienced medical personnel in metabolic disorders. The ongoing monitoring of plasma ammonia levels, neurological status, laboratory tests, and clinical reactions in patients receiving Cargulmic acid tablets is crucial to assess patient reaction to treatment.

Storage instructions
  • Before opening the medicine, refrigerate it at 2 – 8°C or 36 – 46°F.
    After the first opening of the container of medicine, follow the following steps:
  • Neither refrigerate the medicine nor store it above 30°C /86° F.
  • To protect the medicine from moisture, keep the container tightly closed.
  • Write the opening date on the tablet container.
  • Please do not use it after the expiration date as mentioned on the tablet container.
  • Discard the container after one month of opening.

Frequently asked questions (FAQs)

Carbaglu is a white, elongated tablet containing carglumic acid used for oral administration only.  

Carbaglu tablet contains carglumic acid as an active ingredient, croscarmellose sodium, hypromellose, microcrystalline cellulose, silica colloidal anhydrous, sodium lauryl sulfate, sodium stearyl fumarate are inactive ingredients. 

  1. Dissolve Carbaglu tablets in water. Do not swallow the tablet whole or crushed. 
  2. Mix each 200 mg tablet in a minimum of 2.5 mL of water to get 80 mg/mL concentration. 
  3. Carbaglu tablets do not dissolve completely in water, and undissolved particles of the tablet may remain in the mixing container.
  4. Take Carbaglu immediately before meals.
  5. There are no clinical studies that recommend using foods or liquids except water to prepare the dose.

Ikris Pharma Network is an authorized partner of “Recordati,” who can help access this medicine in India for the patient through the legal process. To know Carglumic acid price and more, contact Ikris at toll-free no. 18008891064 or write at

You can buy Carbaglu in India via Ikris Pharma Network, an authorized partner of “Recordati” for Carbaglu medicine.