Urea Cycle Disorder (UCD) can become life-threatening, if left untreated.
The initial set of treatments for a child include:
- Dialysis for removing ammonia from their blood.
- Feeding supplements of sugars, fats, and amino acids
- Medicines to remove extra nitrogen.
- Frequent blood tests will be needed throughout their life to check ammonia levels.
Long-term treatment will include:
- A low protein, high-calorie diet
- Medications to remove nitrogen
- Supplements of amino acids
- Drinking plenty of water
- A liver transplant can reverse the symptoms of a urea cycle disorder.
Treatment with drugs include:
- Sodium phenylbutyrate or buphenyl, which provides alternative ammonia removal pathways and prevents hyperammonemia. Sodium benzoate is also used for continual ammonia removal from the bloodstream.
- Lactulose and Neosporin, when taken orally, can help prevent ammonia production by bacteria in the colon. Multivitamins, calcium and antioxidant supplements are also prescribed.
- Antacids are often used to relieve gastrointestinal side effects of these drugs such as acid reflux and stomach ache.
Cycle of biochemical reactions,Lethargy,Coma
Sodium phenylbutyrate or buphenyl,Lactulose,Neosporin,Pharmaceutical grade L-citrulline supplements,L-arginine,Antacids
In the neonatal period: lethargy, seizures, hypotonia (poor muscle tone), respiratory distress, and coma,In childhood: hyperactive behavior, screaming and self-injurious behavior, and refusal to eat meat or other high-protein foods,In Adulthood: stroke-like symptoms, episodes of lethargy, and delirium