Hello, The main goal, of course, is to protect the brain. We want to prevent a Hyperammonemia crisis and to prevent hyperammonemia or other problems secondary to a urea cycle disorder, it is important that patients consume a low-protein diet. The acute form of the disease might require IV rescue medications such as nitrogen scavenging medicines. In more chronic cases, oral nitrogen scavengers such as arginine or citrulline can be used. Carglumic acid also may be effective in preventing hyperammonemia in patients who have certain urea cycle defects. Antibiotics may be used to help sterilize the gut from urea-splitting bacteria. Severe acute presentations may require invasive techniques such as hemodialysis. After that initial acute presentation, we expect that these other methods will help manage the ammonia level. Liver transplantation is the ultimate therapeutic intervention for our patients, especially patients who present in the first few weeks of life. We perform quite a few in our institution. Of course, the overall goal of management is to protect the brain. Anything that we can do to keep the ammonia levels down in the chronic form of the disease, and to bring the ammonia down as quickly as possible in the acute form, is absolutely necessary. Sometimes just a simple intervention is needed. We have specialists standing by to help with these patients. A simple intervention such as administering IV dextrose with the appropriate electrolytes while the child is in transit to the specialist centre might be lifesaving, and certain brain-saving, and will bring down the ammonia level. Preventing catabolism, or turning around the catabolic spiral, can make all the difference in the world for these patients, and the sooner we do that, the better. For clinicians outside a specialist centre, urgently contacting a centre with experience in treating patients with hyperammonemia is crucial. Hope I have answered your query. Let me know if I can assist you further. Take care Regards, Dr Sanjay Kini, General & Family Physician