This reduces the stress on the hampered urea cycle. It is imperative that protein restriction be done with guidance from a health care professional who can make certain to balance the amount of protein needed for growth with that which will cause symptoms.

It is extremely important for people with these disorders to AVOID FASTING.

Patients with urea cycle abnormalities must also be very careful under times of stress, such as infections. Stress, such as fever, can cause the body to break down its own proteins and exceed the capacity of the abnormal urea cycle to dispose of the by-products. 

A sick-day plan should be formulated with your doctor to avoid all protein, consume high carbohydrate drinks, and stay hydrated.

Most patients with urea cycle disorders require hospitalization at some point in their illness. During such times, they may be treated with medicines that help the body dispose of nitrogen-containing wastes. Dialysis may be required to help rid the body of excess ammonia during extreme illness.

Prognosis

The outcome depends on which of the urea cycle abnormalities a person has, how severe it is, how early it is discovered, and how closely the protein-restricted diet is followed. Babies diagnosed in the first week of life and are immediately placed on a diet do well. 

Continued adherence to the diet can lead to normal adult intelligence. Repeated episodes of not following the diet or stress-induced symptoms can lead to repeated brain swelling and irreversible brain damage.

It should be expected that major stresses, such as surgery or accidents, can be complicated for these patients. Extreme care is required to avoid problems during such periods.

Complications

• confusion progressing to disorientation
• coma
• death
• repeated increases in blood ammonia level

Call Your Health Care Provider If:

If your child has a test which shows increased ammonia in the blood, request an evaluation by a biochemical geneticist or metabolic specialist. If there is a family history of urea cycle disorder, seek genetic counseling during family planning. A dietician is instrumental in planning and updating a protein-restricted diet as a child grows.

Ency. home > Disease > H > Hereditary urea cycle abnormality

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