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Successful Use of Alternate Waste Nitrogen Agents and Hemodialysis in a Patient With Hyperammonemic Coma After Heart-Lung Transplantation
Gerard T. Berry, MD;
Nancy D. Bridges, MD;
Katherine L. Nathanson, MD;
Paige Kaplan, MD;
Robert R. Clancy, MD;
Gary R. Lichtenstein, MD;
Thomas L. Spray, MD
Arch Neurol. 1999;56:481-484.
Background Lethal hyperammonemic coma has been reported in 2 adults after lung transplantation. It was associated with a massive elevation of brain glutamine levels, while plasma glutamine levels were normal or only slightly elevated. In liver tissue, glutamine synthetase activity was markedly reduced, and the histologic findings resembled those of Reye syndrome. The adequacy of therapy commonly used for inherited disorders of the urea cycle has not been adequately evaluated in patients with this form of secondary hyperammonemia.
Objective To determine whether hemodialysis, in conjunction with intravenous sodium phenylacetate, sodium benzoate, and arginine hydrochloride therapy, would be efficacious in a patient with hyperammonemic coma after solid-organ transplantation.
Design Case report.
Setting A children's hospital.
Patient A 41-year-old woman with congenital heart disease developed a hyperammonemic coma with brain edema 19 days after undergoing a combined heart and lung transplantation.
Methods Ammonium was measured in plasma. Amino acids were quantitated in plasma and cerebrospinal fluid by column chromatography. The effectiveness of therapy was assessed by measuring plasma ammonium levels and intracranial pressure and performing sequential neurological examinations.
Results The patient had the anomalous combination of increased cerebrospinal fluid and decreased plasma glutamine levels. To our knowledge, she is the first patient with this complication after solid-organ transplantation to survive after combined therapy with sodium phenylacetate, sodium benzoate, arginine hydrochloride, and hemodialysis. Complications of the acute coma included focal motor seizures, which were controlled with carbamazepine, and difficulty with short-term memory.
Conclusions The aggressive use of hemodialysis in conjunction with intravenous sodium phenylacetate, sodium benzoate, and arginine hydrochloride therapy may allow survival in patients after solid-organ transplantation. An acute acquired derangement in extra–central nervous system glutamine metabolism may play a role in the production of hyperammonemia in this illness that resembles Reye syndrome, and, as in other hyperammonemic disorders, the duration and degree of elevation of brain glutamine levels may be the important determining factors in responsiveness to therapy.
From the Departments of Pediatrics (Drs Berry, Bridges, Nathanson, and Kaplan), Neurology (Dr Clancy), Medicine (Dr Lichtenstein), and Surgery (Dr Spray), University of Pennsylvania School of Medicine; the Department of Pediatrics, Divisions of Biochemical Development and Molecular Diseases (Drs Berry, Nathanson, and Kaplan) and Cardiology (Dr Bridges), and the Departments of Neurology (Dr Clancy) and Cardiothoracic Surgery (Dr Spray), The Children's Hospital of Philadelphia; and the Department of Medicine, Division of Gastroenterology, Hospital of the University of Pennsylvania (Dr Lichtenstein), Philadelphia.
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