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Hyperammonemia in the ICU^*

^* From the Department of Surgery (Dr. Clay), Duke University School of Medicine, Durham, NC; and the Division of Clinical Biochemical Genetics (Dr. Hainline), Department of Medical and Molecular Genetics, Indiana University School of Medicine, Bloomington, IN.

Correspondence to: Alison S. Clay, MD, FCCP, Department of Surgery and Medicine, Duke University Medical Center, Box 2945, Durham, NC 27710; e-mail: alison.clay{at}duke.edu

Abstract

Patients experiencing acute elevations of ammonia present to the ICU with encephalopathy, which may progress quickly to cerebral herniation. Patient survival requires immediate treatment of intracerebral hypertension and the reduction of ammonia levels. When hyperammonemia is not thought to be the result of liver failure, treatment for an occult disorder of metabolism must begin prior to the confirmation of an etiology. This article reviews ammonia metabolism, the effects of ammonia on the brain, the causes of hyperammonemia, and the diagnosis of inborn errors of metabolism in adult patients.

Key Words: adult • ammonia • hyperammonemia • inborn error of metabolism • total parenteral nutrition • urea cycle disorder