Urea Cycle Disease

When the body's urea cycle does not have enough enzymes to remove the correct amount of ammonia from the bloodstream, a urea cycle disorder occurs. There is no cure for urea cycle disease.

Cause

• Biochemical steps in the body remove nitrogen from the blood and convert it to urea, which is expelled from the body through urine. Urea cycle disorders allow too much nitrogen to accumulate and become ammonia, producing a condition called hyperammonemia, which is toxic to the body.

Warning

• This accumulation of ammonia travels through the bloodstream to the brain, leading to irreversible brain damage, coma and sometimes death.

Symptoms

• Symptoms associated with urea cycle diseases include slurred speech, vomiting, unsteady gait, combativeness, decrease in mental awareness and loss of consciousness.

Heredity

• There is often a hereditary component to urea cycle disorders. Diseases contributing to urea cycle disorders include argininosuccinic aciduria, arginase deficiency, CPS deficiency and citrullinemia.

Statistics

• One in every 30 thousand newborns has a urea cycle disease, according to the National Institutes of Health.

Treatment

• Treatment for urea cycle disorders includes a lower protein diet, reducing stress and hospitalizations to remove excess nitrogen-containing waste products from the body.