Ammobula Powder contains Sodium Phenylbutyrate, a salt form of phenylbutyric acid, primarily used as an ammonia scavenger in the treatment of certain rare urea cycle disorders (UCDs). These are genetic conditions where the body is unable to efficiently remove ammonia—a waste product from protein metabolism—leading to potentially life-threatening consequences.

Sodium phenylbutyrate is a lifesaving therapy, especially in children, that helps control blood ammonia levels, thereby preventing neurological damage.

Mechanism of Action

Sodium Phenylbutyrate acts as a prodrug that undergoes metabolism in the liver to form phenylacetate, which then conjugates with glutamine to form phenylacetylglutamine. This compound is then excreted in the urine.

The mechanism in simple terms:

• Bypasses the defective urea cycle

• Promotes alternative pathway for nitrogen elimination

• Removes two nitrogen atoms per molecule of phenylacetylglutamine, similar to how urea would normally do.

  Uses / Indications

  Primary Indication:

  • Urea Cycle Disorders (UCDs)
    Used in patients who cannot adequately eliminate nitrogen waste, typically as an adjunct to dietary protein restriction and other therapies.

  Who needs it?

  • Infants, children, or adults with inherited enzyme deficiencies (e.g., CPS1, OTC, ASS1)

  • Patients showing signs of hyperammonemia such as vomiting, lethargy, confusion, or seizures