OTC deficiency is a disorder of the urea cycle X-linked. It is manifested in men as severe hyperammonemia in the first days of life. In women the disease is milder severity. Various conditions cause decompensation with hyperammonemia. It could be fatal or cause permanent neurological damage. We report a 36 years old woman admitted for surgery, she suffered a decompensation in conjunction with surgical wound infection. Hyperammonemia caused neurological deterioration with decreased level of consciousness, tetraparesis and neurogenic dysphagia. The treatment consisting of low-protein diet, ammonium chelating drugs and dialytic measures, was effective in controlling hyperammonaemia and improving neurological status. This case illustrates the importance of nutritional support of patients with disorders of the urea cycle in the hospital because the descompensations are more frecuent here.
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