title: Hyperammonemia due to urea cycle disorders: a potentially fatal condition in the intensive care setting.
abstract: Disorders of the urea cycle are secondary to a defect in the system that converts ammonia into urea, resulting in accumulation of ammonia and other products. This results in encephalopathy, coma, and death if not recognized and treated rapidly. Late-onset urea cycle disorders may be precipitated by acute disease and can be difficult to recognize because patients are already ill. Diagnosis of urea cycle disorders is based on clinical suspicion and determination of blood ammonia in suspected patients with neurological symptoms in the intensive care setting. Treatment is based on the removal of ammonia by dialysis or hemofiltration, reduction of the catabolic state, abolishment of nitrogen administration, and use of pharmacological nitrogen scavenging agents.
authors.affiliation: Emergency Medicine Department, University of Sao Paulo, Sao Paulo, 05508-070 Brazil ; Faculdade de Medicina, USP, Av Dr Arnaldo 455 Room 3189 LIM 51, Sao Paulo, 05508-070 Brazil.
authors.forename: Marcel Cerqueira Cesar
authors.initials: MC
authors.lastname: Machado
authors.affiliation: Emergency Medicine Department, University of Sao Paulo, Sao Paulo, 05508-070 Brazil ; Faculdade de Medicina, USP, Av Dr Arnaldo 455 Room 3189 LIM 51, Sao Paulo, 05508-070 Brazil.
authors.forename: Fabiano
authors.initials: F
authors.lastname: Pinheiro da Silva
keywords: Hyperammonemia; Intensive care unit; Neurological disorders