carbaglu
carbaglu Uses, Dosage, Side Effects, Mechanism of Action, How Long Does it Take to Work? carbaglu is a brand name. Its generic name is Carglumic acid.
Carglumic Acid
carbaglu (10-carboxy-7,8-dihydropteroic acid) is a synthetic derivative of the naturally-occurring amino acid, para-aminobenzoic acid (PABA). It is used as a medication primarily to treat hyperammonemia, or abnormally high levels of ammonium (NH4+) in the body. It is also sometimes used to treat urea cycle disorders (UCDs).
Uses for Carglumic Acid
carbaglu is mainly indicated for the treatment of hyperammonemia due to deficiency of N-acetylglutamate synthetase (NAGS) and ornithine transcarbamylase (OTC) caused by urea cycle disorders.[1] It is believed to have been beneficial in even low levels of hyperammonemia and is usually used in combination with drugs such as sodium benzoate and sodium phenylbutyrate.
Mechanism of Action
carbaglu acts as an inhibitor of the enzyme methionine adenosyltransferase (MAT), which is believed to be the cause of the increased levels of ammonia in the body in hyperammonemia associated with urea cycle disorders.[2] This reduces the amount of ammonia in the bloodstream and helps clear the body of it. carbaglu also helps the body to use free ammonia molecules by functioning as a methyl group donor in the recycling of glutamic acid and urea cycle intermediates.
How Long Does it Take to Work?
carbaglu has been shown to take effect within hours to days, depending on the severity of the hyperammonemia present. Symptoms can start to improve shortly after starting treatment with carglumic acid.[3]
Absorption, Route of Elimination, Dosage, Administration
carbaglu is taken orally, usually in the form of tablets. It is absorbed from the intestine and distributed throughout the body. It reaches a peak concentration in the plasma about 3-5 hours after administration. It is also metabolized and eliminated mainly through the kidneys, with a half-life of about 2-3 hours. The typical loading dose is 300-600 mg/kg once daily, for 1-2 days, followed by a maintenance dose of 50-100 mg/kg/day.
Side Effects, Toxicity, Precaution, Interaction, Disease Interaction, Drug Interaction, Food Interactions, Pregnancy Use, Lactation Use
Common side effects of taking carglumic acid include fatigue, nausea, vomiting, diarrhoea, anorexia, constipation, dizziness, headache, insomnia, and abdominal pain. The most serious toxicity is hyperammonemia, which can be caused by taking too much carglumic acid. It is important to monitor the levels of ammonia in the body while taking carglumic acid. Patients on the drug should be regularly monitored for signs of elevated ammonia levels. Also, carglumic acid may interact with a variety of medications, including cephalosporin antibiotics, antiretrovirals, and some anticonvulsants. It may also interact with certain foods and dietary supplements. The use of carglumic acid in pregnant and lactating women has not been studied, and so it is not recommended for these two groups.
Acute Overdose, Contraindication, Use Direction, Storage Condition, Volume of Distribution, Half Life, Clearance
Acute overdose of carglumic acid has not been reported, and there are no known contraindications to its use. carbaglu should be used in the direction prescribed by the doctor for the particular condition. It should be stored at room temperature away from light and moisture, and should be kept out of the reach of children. The volume of distribution of carglumic acid is 0.11 L/kg. The half-life of carglumic acid is approximately 2.3-3.3 hours, and its clearance is 6.9-19.2 mL/min/kg.
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