Product image of Kwas Kargluminowy (Carglumic Acid) supplied by GNH India

Kwas Kargluminowy

Active Ingredient:
Carglumic Acid
Origin:
EU

Kwas Kargluminowy (Carglumic Acid)

Carglumic Acid, the active ingredient in Kwas Kargluminowy, is a carbamyl phosphate synthetase I activator presented as an oral formulation for oral use. It treats hyperammonemia caused by N‑acetylglutamate synthase deficiency and other urea cycle disorders, and improves metabolic stability through enhanced ammonia clearance. GNH India supplies Kwas Kargluminowy as a licensed, GDP‑compliant international pharmaceutical supplier serving hospitals worldwide and clinics, ensuring consistent quality and regulatory compliance across markets. Our distribution network includes transplant centres and specialty pharmacies, facilitating timely access to this essential therapy.

Manufacturer / TM Owner

Waymade B.V.

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Indications & Clinical Uses

  • Carglumic Acid is used to manage hyperammonemia within the urea cycle disorder spectrum.
  • Hyperammonemia due to N‑acetylglutamate synthase deficiency: lowers plasma ammonia concentrations.
  • Urea cycle disorders (e.g., carbamoyl phosphate synthetase I deficiency): supports the urea cycle to enhance ammonia detoxification.
  • Hyperammonemia in organic acidemias: assists in reducing elevated ammonia levels.

How It Works

  • Carglumic Acid activates carbamoyl‑phosphate synthetase I, the first enzyme of the mitochondrial urea cycle. By mimicking the allosteric activator N‑acetylglutamate, it increases the synthesis of carbamoyl‑phosphate, facilitating the conversion of ammonia to urea and promoting its excretion. This restoration of enzymatic activity improves the overall capacity of the urea cycle to detoxify ammonia, and the enhanced flux through the cycle reduces systemic ammonia accumulation and mitigates neurotoxic effects.

Side Effects

Carglumic Acid may be associated with several adverse reactions, which are categorized by frequency and severity.

Common Side Effects

  • Nausea
  • Vomiting
  • Diarrhea
  • Headache

Serious or Rare Side Effects

  • Allergic reactions (including rash or anaphylaxis)
  • Metabolic acidosis
  • Hepatic dysfunction

Precautions & Warnings

  • When prescribing Carglumic Acid, consider the following precautions.
  • Renal function: assess baseline renal function and monitor during therapy.
  • Pregnancy and lactation: safety not established; use only if potential benefit justifies risk.
  • Pediatric patients: dose may require adjustment based on weight and metabolic status.
  • Concomitant medications: review for potential interactions that may affect ammonia metabolism.
  • Store at room temperature: keep in original container, protect from moisture and light.

Avoid Interactions

Carglumic Acid can interact with other medicines; awareness of interaction severity is important.

Major Interactions (Avoid)

  • Sodium phenylbutyrate: may diminish the ammonia‑lowering effect of Carglumic Acid.
  • Carbamazepine: may increase risk of hepatic toxicity when combined.

Moderate Interactions (Monitor Closely)

  • Diuretics (e.g., furosemide): may alter electrolyte balance, requiring monitoring.
  • Liver enzyme inducers (e.g., rifampicin): may reduce drug exposure.
  • Protein‑rich meals: may increase ammonia load, affecting efficacy.

Frequently Asked Questions

Kwas Kargluminowy contains Carglumic Acid and is prescribed to treat hyperammonemia associated with N‑acetylglutamate synthase deficiency and other urea cycle disorders. By enhancing the urea cycle, it helps lower elevated blood ammonia levels, reducing the risk of neurological complications in affected patients.

Carglumic Acid acts as an allosteric activator of carbamoyl‑phosphate synthetase I, the first enzyme of the mitochondrial urea cycle. It mimics N‑acetylglutamate, increasing carbamoyl‑phosphate production, which drives the conversion of toxic ammonia into urea for safe excretion.

The dosage of Kwas Kargluminowy is individualized. A qualified prescriber determines the appropriate amount based on the patient’s age, weight, severity of hyperammonemia, and underlying metabolic condition. Patients should follow the prescribing information provided by their healthcare professional.

Safety data for Carglumic Acid in pregnancy and lactation are limited. It should only be used when the potential benefit to the mother outweighs any possible risk to the fetus or infant, and under close medical supervision.

To order, submit an enquiry on the product page at gnhindia.com, specifying the required quantity. GNH India works with hospitals, pharmacies and procurement teams worldwide, verifies trade or institutional credentials, and then provides pricing, availability, shipping details (including any cold‑chain requirements) and necessary import documentation.

The primary alternative class includes sodium phenylbutyrate and sodium benzoate, which act as ammonia scavengers. Carglumic Acid directly activates the urea cycle, offering a more physiological approach. In patients with N‑acetylglutamate synthase deficiency, Carglumic Acid often provides superior ammonia control compared with scavenger drugs.

Store the product at room temperature, below 25 °C, in its original packaging. Protect it from moisture and direct sunlight. Do not use if the packaging is damaged or if the product shows signs of degradation.

Kwas Kargluminowy is taken orally, usually with water. The exact administration schedule—such as with meals or on an empty stomach—should follow the prescribing clinician’s instructions to ensure optimal absorption and efficacy.

Serious adverse events, although rare, can include allergic reactions ranging from rash to anaphylaxis, metabolic acidosis, and hepatic dysfunction. Patients should be monitored for signs of these conditions, and treatment should be discontinued if severe reactions occur.

Carglumic Acid is contraindicated in patients with known hypersensitivity to the active ingredient or any excipients. Caution is also advised for individuals with severe hepatic impairment or uncontrolled metabolic disorders where the urea cycle cannot be effectively engaged.

Published by

GNH India Pharmaceuticals Limited

Licensed pharmaceutical supplier

Last updated:

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