Nutritional management of patients with urea cycle disorders

Summary The nutritional management of patients with urea cycle disorders (UCDs) involves restriction of dietary protein along with provision of adequate protein‐free energy, essential amino acid supplements, and vitamins and minerals in combination with nitrogen‐scavenging drugs. The present paper discusses nutrition therapy for a range of circumstances: during an acute hyperammonaemic episode and at hospital discharge; before, during, and after surgery; and for lifelong chronic management of UCDs.