Jump To


Generic name: glycerol phenylbutyrate

What is Ravicti used for?

Ravicti is used to treat urea cycle problems.


RAVICTI (glycerol phenylbutyrate) is a clear, colorless to pale yellow oral liquid. It is insoluble in water and most organic solvents, and it is soluble in dimethylsulfoxide (DMSO) and greater than 65% acetonitrile.

Glycerol phenylbutyrate is a nitrogen-binding agent. It is a triglyceride containing 3 molecules of PBA linked to a glycerol backbone, the chemical name of which is benzenebutanoic acid, 1′, 1′ ′ –(1,2,3-propanetriyl) ester with a molecular weight of 530.67. It has a molecular formula of C33H38O6. The structural formula is:


Mechanism of Action

UCDs are inherited deficiencies of enzymes or transporters necessary for the synthesis of urea from ammonia (NH3, NH4+). Absence of these enzymes or transporters results in the accumulation of toxic levels of ammonia in the blood and brain of affected patients. RAVICTI is a triglyceride containing 3 molecules of PBA. PAA, the major metabolite of PBA, is the active moiety of RAVICTI. PAA conjugates with glutamine (which contains 2 molecules of nitrogen) via acetylation in the liver and kidneys to form PAGN, which is excreted by the kidneys (Figure 1). On a molar basis, PAGN, like urea, contains 2 moles of nitrogen and provides an alternate vehicle for waste nitrogen excretion.

Figure 1: RAVICTI Mechanism of Action

Figure 1

Before taking Ravicti, tell your doctor:

  • If you are allergic to Ravicti; any part of this medicine; or any other drugs, foods, or substances. Tell your doctor about the allergy and what signs you had.
  • If you are breast-feeding. Do not breast-feed while you take Ravicti.

This medicine may interact with other drugs or health problems.

Tell your doctor and pharmacist about all of your drugs (prescription or OTC, natural products, vitamins) and health problems. You must check to make sure that it is safe for you to take Ravicti with all of your drugs and health problems. Do not start, stop, or change the dose of any drug without checking with your doctor.

What are some things I need to know or do while I take Ravicti?

  • Tell all of your health care providers that you take Ravicti. This includes your doctors, nurses, pharmacists, and dentists.
  • Follow the diet plan that your doctor told you about.
  • Have blood work checked as you have been told by the doctor. Talk with the doctor.
  • Tell your doctor if you are pregnant or plan on getting pregnant. You will need to talk about the benefits and risks of using Ravicti while you are pregnant.

How is Ravicti best taken?

Use Ravicti as ordered by your doctor. Read all information given to you. Follow all instructions closely.

For all patients taking Ravicti:

  • Take with food.
  • This medicine may be given with formula.
  • Measure liquid doses carefully. Use the measuring device that comes with Ravicti. If there is none, ask the pharmacist for a device to measure Ravicti.
  • Those who have feeding tubes may use Ravicti. Use as you have been told. Flush the feeding tube after Ravicti is given.


  • If giving Ravicti to a child who is breast-feeding, give just before your child breast-feeds.

What do I do if I miss a dose?

  • Take a missed dose as soon as you think about it.
  • If it is close to the time for your next dose, skip the missed dose and go back to your normal time.
  • Do not take 2 doses at the same time or extra doses.

What are the side effects of Ravicti that I need to call my doctor about immediately?

WARNING/CAUTION: Even though it may be rare, some people may have very bad and sometimes deadly side effects when taking a drug. Tell your doctor or get medical help right away if you have any of the following signs or symptoms that may be related to a very bad side effect:

For all patients taking Ravicti:

  • Signs of an allergic reaction, like rash; hives; itching; red, swollen, blistered, or peeling skin with or without fever; wheezing; tightness in the chest or throat; trouble breathing, swallowing, or talking; unusual hoarseness; or swelling of the mouth, face, lips, tongue, or throat.
  • Shortness of breath.
  • Cough.
  • Flushing.
  • Headache.
  • Upset stomach or throwing up.
  • Feeling confused.
  • Memory problems or loss.
  • Change in hearing.
  • Change in taste.
  • Feeling dizzy, sleepy, tired, or weak.
  • Passing out.
  • Shakiness.
  • Seizures.
  • A burning, numbness, or tingling feeling that is new or worse.

Children younger than 2 years of age:

  • Low white blood cell counts have happened with Ravicti. This may lead to a higher chance of getting an infection. Call your child’s doctor right away if your child has signs of infection like fever, chills, or sore throat.

Children younger than 2 months of age:

  • Signs of dehydration like dry skin, mouth, or eyes; thirst; fast heartbeat; dizziness; fast breathing; or confusion.
  • Signs of too much acid in the blood (acidosis) like confusion; fast breathing; fast heartbeat; a heartbeat that does not feel normal; very bad stomach pain, upset stomach, or throwing up; feeling very sleepy; shortness of breath; or feeling very tired or weak.
  • Feeling agitated.
  • Low blood cell counts have happened with Ravicti. If blood cell counts get very low, this can lead to bleeding problems, infections, or anemia. Call your child’s doctor right away if your child has signs of infection like fever, chills, or sore throat; any unexplained bruising or bleeding; or if your child feels very tired or weak.

What are some other side effects of Ravicti?

All drugs may cause side effects. However, many people have no side effects or only have minor side effects. Call your doctor or get medical help if any of these side effects or any other side effects bother you or do not go away:

For all patients taking Ravicti:

  • Stomach pain or diarrhea.
  • Not hungry.
  • Gas.
  • Heartburn.

Children younger than 2 years of age:

  • Constipation.
  • Runny or stuffy nose.
  • Small round bumps on the skin.

Children younger than 2 months of age:

  • Problems with feeding.
  • Feeling irritable.

These are not all of the side effects that may occur. If you have questions about side effects, call your doctor. Call your doctor for medical advice about side effects.

You may report side effects to the FDA at 1-800-332-1088. You may also report side effects at https://www.fda.gov/medwatch.



  • NDC 75987-050-06
    (glycerol phenylbutyrate) Oral Liquid
  • 1.1 grams per mL
  • For Oral Use Only
    25 mL
  • Pharmacist:
    Dispense the enclosed
    Medication Guide to
    patient and/or caregiver.
  • Rx Only

If overdose is suspected:

If you think there has been an overdose, call your poison control center or get medical care right away. Be ready to tell or show what was taken, how much, and when it happened.


Read Next Article

PHP Code Snippets Powered By : XYZScripts.com