When there is too much ammonia in the blood, it’s called hyperammonemia (hi-per-am-oh-NEE-me-uh). Hyperammonemia can be very harmful to the body, and especially to the brain. Over time, high ammonia blood levels can lead to behavioral problems, tiredness (fatigue), and even serious health problems like seizures and coma.
A person’s sex (male or female) is decided by their chromosomes. Each pair of chromosomes carries genes that define which traits are passed from parent to child, such as hair and eye color. There are 2 types of chromosomes which determine your sex at birth: X chromosomes and Y chromosomes. A female has 2 X chromosomes and a male has 1 X and 1 Y chromosome. When someone passes a UCD down through a gene that’s located on the X chromosome, they are called X-linked carriers.
Autosomal recessive is when both parents have a UCD gene. When this happens, see the chances of having a child with a UCD:
For all other UCDs except ornithine transcarbamylase (OTC) deficiency, both parents carry a gene that doesn’t work.
Different UCD genes cause different types of UCDs. In people with UCDs, ammonia cannot leave the body because it is not processed correctly. See how the urea cycle works.
There are 8 types of urea cycle disorders. They are named after the protein in the urea cycle that is not working correctly:
*Important Limitation of Use: It is not known if RAVICTI is safe and effective for the treatment of N-acetylglutamate synthase (NAGS) deficiency.
If you or your child has been diagnosed with a UCD, it’s important to pay attention to other relatives, especially children, who may have similar signs and symptoms.
OTC deficiency is most commonly passed from a mother to her child through her X chromosome. Some mothers who are carriers may not know they have the OTC gene until their child is diagnosed. Mothers who are OTC carriers may have subtle signs and symptoms, which should not be ignored. Those subtle symptoms can include headaches, confusion, and trouble concentrating. If you experience these symptoms, you should talk with your doctor to see if you may need treatment.
What is the most important safety information I should know about RAVICTI?
RAVICTI may cause serious side effects, including:
Nervous system side effects (Neurotoxicity) Phenylacetate (PAA), a breakdown product of RAVICTI, may cause nervous system side effects. Call your doctor or get medical help right away if you have any of these symptoms while taking RAVICTI:
Your doctor may do blood tests to measure the amount of PAA in your blood during your treatment with RAVICTI.
What is RAVICTI?
Do not take RAVICTI if you are allergic to phenylbutyrate. Call your doctor or go to the nearest hospital emergency room if you have wheezing, shortness of breath, cough, low blood pressure, flushing, nausea or a rash while taking RAVICTI.
Before taking RAVICTI, tell your doctor about all of your medical conditions, including if you:
What are possible side effects of RAVICTI?
RAVICTI may cause serious side effects, including:
See "What is the most important information I should know about RAVICTI?"
The most common side effects of RAVICTI in adults include:
The most common side effects of RAVICTI in children 2 years to 17 years of age include:
The most common side effects of RAVICTI in children 2 months to less than 2 years of age include:
The most common side effects of RAVICTI in children less than 2 months of age include:
These are not all of the possible side effects of RAVICTI. Call your doctor for medical advice about side effects. You may report side effects to FDA at 1-800-FDA-1088.
For additional Important Safety Information, click here for the Medication Guide and discuss with your doctor.
What is the most important safety information I should know about BUPHENYL?
BUPHENYL may cause serious side effects, including:
Nervous system side effects (Neurotoxicity) Phenylacetate (PAA), a breakdown product of BUPHENYL, may cause nervous system side effects. Call your doctor or get medical help right away if you have any of these symptoms while taking BUPHENYL:
What is BUPHENYL?
Do not take BUPHENYL if you are allergic to phenylbutyrate. Call your doctor or go to the nearest hospital emergency room if you experience an allergic reaction while taking BUPHENYL.
Before taking BUPHENYL, tell your doctor about all of your medical conditions, including if you:
What are possible side effects of BUPHENYL?
BUPHENYL may cause serious side effects, including:
See “What is the most important information I should know about BUPHENYL?”
The most common side effects of BUPHENYL include:
These are not all of the possible side effects of BUPHENYL. Call your doctor for medical advice about side effects. You may report side effects to FDA at 1-800-FDA-1088.
For additional Important Safety Information, click here for the Patient Package Insert and discuss with your doctor.
What is the most important safety information I should know about RAVICTI?
RAVICTI may cause serious side effects, including:
Nervous system side effects (Neurotoxicity) Phenylacetate (PAA), a breakdown product of RAVICTI, may cause nervous system side effects. Call your doctor or get medical help right away if you have any of these symptoms while taking RAVICTI:
Your doctor may do blood tests to measure the amount of PAA in your blood during your treatment with RAVICTI.
What is RAVICTI?
Do not take RAVICTI if you are allergic to phenylbutyrate. Call your doctor or go to the nearest hospital emergency room if you have wheezing, shortness of breath, cough, low blood pressure, flushing, nausea or a rash while taking RAVICTI.
Before taking RAVICTI, tell your doctor about all of your medical conditions, including if you:
What are possible side effects of RAVICTI?
RAVICTI may cause serious side effects, including:
See "What is the most important information I should know about RAVICTI?"
The most common side effects of RAVICTI in adults include:
The most common side effects of RAVICTI in children 2 years to 17 years of age include:
The most common side effects of RAVICTI in children 2 months to less than 2 years of age include:
The most common side effects of RAVICTI in children less than 2 months of age include:
These are not all of the possible side effects of RAVICTI. Call your doctor for medical advice about side effects. You may report side effects to FDA at 1-800-FDA-1088.
For additional Important Safety Information, click here for the Medication Guide and discuss with your doctor.
What is the most important safety information I should know about RAVICTI?
RAVICTI may cause serious side effects, including:
Nervous system side effects (Neurotoxicity) Phenylacetate (PAA), a breakdown product of RAVICTI, may cause nervous system side effects. Call your doctor or get medical help right away if you have any of these symptoms while taking RAVICTI:
Your doctor may do blood tests to measure the amount of PAA in your blood during your treatment with RAVICTI.
What is RAVICTI?
Do not take RAVICTI if you are allergic to phenylbutyrate. Call your doctor or go to the nearest hospital emergency room if you have wheezing, shortness of breath, cough, low blood pressure, flushing, nausea or a rash while taking RAVICTI.
Before taking RAVICTI, tell your doctor about all of your medical conditions, including if you:
What are possible side effects of RAVICTI?
RAVICTI may cause serious side effects, including:
See "What is the most important information I should know about RAVICTI?"
The most common side effects of RAVICTI in adults include:
The most common side effects of RAVICTI in children 2 years to 17 years of age include:
The most common side effects of RAVICTI in children 2 months to less than 2 years of age include:
The most common side effects of RAVICTI in children less than 2 months of age include:
These are not all of the possible side effects of RAVICTI. Call your doctor for medical advice about side effects. You may report side effects to FDA at 1-800-FDA-1088.
For additional Important Safety Information, click here for the Medication Guide and discuss with your doctor.