The role of liver transplantation in urea cycle disorders.

Liver transplantation has an important role in the management of patients with urea cycle disorders, particularly those with severe variants, progressive liver disease, and some patients who have proved very difficult to control with conventional therapy. As a result of technical advances in liver transplantation, the outlook, both morbidity and mortality, is better and continues to improve. Patients can almost always have a normal diet and the quality of life for survivors is generally excellent.