The urea cycle is a chain of reactions occurring in the liver using a variety of enzymes. These enzymes play an important role in the removal of byproducts of protein metabolism (nitrogen) from the blood. Urea cycle disorders (UCDs) are a group of inherited metabolic disorders named based on the enzyme that is affected in the cycle. Normally, excess nitrogen enters the urea cycle to produce urea, a nontoxic compound that is excreted in urine. When one of the enzymes in the urea cycle is not working, urea cannot be made. The excess nitrogen forms into ammonia and circulates in the blood. Ammonia is toxic to the body. When a person with this disorder eats more protein than the body can effectively use, the excess nitrogen cannot be removed and ammonia levels in the blood rise. Excess nitrogen can also come from the breakdown of protein in muscles and other body stores, which may occur during times of stress or illness.
The primary nutritional management for UCDs is to limit the amount of protein in the diet and often provide a UCD formula to support adequate intakes of protein, especially essential amino acids.
Vitaflo USA offers formulas for the dietary management of Urea Cycle Disorders from childhood through adulthood. If you have additional questions about Vitaflo’s range of UCD products, please feel free to contact us.