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On the other hand, mild elevations in the level of ammonia that did not cause any symptoms have been observed in patients treated with VA in whom medication did not need to be discontinued.13 Thus, we do not believe that levels of ammonia need to be monitored in all patients treated with VA, although they should be monitored in those who develop neurological symptoms.14 Several factors may be associated to the development of VHE, but the most important are polytherapy and deficiency of ornithine transcarbamylase (OTC) and of carnitine