**5. General management**

When managing a patient with hyperammonemia, several factors should be taken into consideration, including the onset (acute vs. chronic), presence or absence of symptoms, degree or severity of the hyperammonemia, and the underlying cause. Acute severe hyperammonemia carries the highest risk of adverse outcomes and is potentially fatal. Thus, all measures must be taken to lower the ammonia level rapidly [9]. The following are essential lines of general management [2, 8–10]:


If hyperammonemia is still not explained, one needs to consider investigating for underlying urea cycle disorders as soon as possible. As mentioned above, some of the cases of non-hepatic hyperammonemia in adults could be related to undiagnosed urea cycle disorder, which becomes unmasked under stressful conditions. Therefore, it might be prudent to involve a biochemical geneticist (metabolic) physician early on to provide some guidance on further investigations and management.


#### **Table 2.**

*General and initial lines of management for hyperammonemia.*

