Urea cycle disorders therapy

Urea cycle disorders (UCDs) Hyperammonemic encephalopathy, sometimes fatal, has been reported following initiation of valproate therapy in patients with UCDs, a group of uncommon genetic abnormalities, particularly ornithine transcarbamylase deficiency. Patients who develop symptoms of unexplained hyperammonemic encephalopathy while receiving valproate therapy should receive prompt treatment (including discontinuation of valproate therapy) and be evaluated for underlying urea cycle disorders (see Precautions). 

Brusilow SW, Maestri NE Urea cycle disorders diagnosis, pathophysiology and therapy. Adv Pediatr 43 127-170,1996. 

Batshaw ML, MacArthur RB, Tuchman M. Alternative pathway therapy for urea cycle disorders twenty... 

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