All About Citrullinemia: How to Test For It and What You Can Do

While citrulline is a necessary component of the human- Vomiting
body that helps to process ammonia in the urea cycle,- Altered behavior
some people have conditions that make processing- Seizure
the amino acid a real problem.- Coma
Citrullinemia is a genetic disorder that is inherited from- Mental retardation
your parents. The disorder is caused by a lack of anCitrullinemia is diagnosed typically at birth, but it can be
enzyme that is necessary in order to process anddiscovered later on during tests that identify the levels
remove nitrogen from the body. This enzyme is calledof citrulline present in the body. These tests usually
argininosuccinate synthetase. Without this enzyme, theinvolve chromatography.
nitrogen accumulates in the blood in the form ofThe key to successful citrullinemia treatment is early
ammonia. The condition of having too much ammoniadiagnosis. Prompt treatment can help prevent the
in the blood is called hyperammonemia.accumulation of ammonia in the blood and even death.
There are two types of citrullinemia: Type 1 and TypeThe most common treatments used include:
2. Citrullinemia Type 1 is diagnosed in infants and- Low-protein diet
children. Common symptoms include:- Monitoring blood tests
- Vomiting- Medications
- LethargyA low-protein diet is probably the most important part
- Lack of appetiteof treatment. You will more than likely need to eliminate
- Comafoods like fish, eggs, poultry, meat, milk, cheese,
- Enlarged liverlegumes, nuts, and beans. However, protein is still a
- Poor growthnecessary part of your diet. You cannot eliminate it
- Breathing problemscompletely. This is why having an experienced
Citrullinemia Type 2 is diagnosed in adults and ismetabolic doctor treating you is so important. A
typically caused by the onset of a liver disorder duringdietician is equally important in ensuing that you or your
infancy called neonatal intrahepatic cholestasis.child obtains the nutrients he or she needs without
Common symptoms present in adults include:exacerbating the disease.
- Ammonia intoxication