Acute hyperammonaemia is a medical emergency. Most cases are resulted from severely impaired liver function but some cases of non-hepatic causes do occur. In patients with unexplained altered conscious state, immediate measurement of plasma ammonia level is a simple but important step in early diagnosis of hyperammonaemia and prompt reduction of ammonia level to minimise permanent brain damage. We reported a case of recurrent hyperammonaemia and cerebral dysfunction due to urinary tract infection with urinary retention in a patient without underlying liver disease.
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