Hyperammonemic Coma in an Adult due to Ornithine Transcarbamylase Deficiency

Objective. To report an unusual cause of coma in an adult. Design. Case report. Setting. University teaching hospital. Patient. A previously healthy 53-year-old man initially presented with altered mental status and progressed to coma. He was found to be substantially hyperammonemic and did not impr...

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Main Authors: Daniel L. Roberts, David A. Galbreath, Bhavesh M. Patel, Timothy J. Ingall, Amer Khatib, Daniel J. Johnson
Format: Article
Language:English
Published: Wiley 2013-01-01
Series:Case Reports in Critical Care
Online Access:http://dx.doi.org/10.1155/2013/493216
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author Daniel L. Roberts
David A. Galbreath
Bhavesh M. Patel
Timothy J. Ingall
Amer Khatib
Daniel J. Johnson
author_facet Daniel L. Roberts
David A. Galbreath
Bhavesh M. Patel
Timothy J. Ingall
Amer Khatib
Daniel J. Johnson
author_sort Daniel L. Roberts
collection DOAJ
description Objective. To report an unusual cause of coma in an adult. Design. Case report. Setting. University teaching hospital. Patient. A previously healthy 53-year-old man initially presented with altered mental status and progressed to coma. He was found to be substantially hyperammonemic and did not improve with lactulose therapy and continuous venovenous hemodialysis. Results. Biochemical testing revealed previously undiagnosed ornithine transcarbamylase deficiency, and the patient responded to arginine, sodium phenylacetate, and sodium benzoate. Conclusion. Even in adult patients with no known history, inborn errors of metabolism must be considered in the differential diagnosis of unexplained coma. Defects of the urea cycle can present with an unprovoked hyperammonemic coma.
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spelling doaj-art-d2ed9666975d4a2496ec9548e4ae9cb52025-08-20T02:19:51ZengWileyCase Reports in Critical Care2090-64202090-64392013-01-01201310.1155/2013/493216493216Hyperammonemic Coma in an Adult due to Ornithine Transcarbamylase DeficiencyDaniel L. Roberts0David A. Galbreath1Bhavesh M. Patel2Timothy J. Ingall3Amer Khatib4Daniel J. Johnson5Division of Hospital Internal Medicine, Department of Medicine, Mayo Clinic, Phoenix, AZ 85054, USAKansas City Family Medical Clinic, Kansas City, MO 64114, USADepartment of Critical Care Medicine, Mayo Clinic, Phoenix, AZ 85054, USADepartment of Neurology, Mayo Clinic, Phoenix, AZ 85054, USADivision of Gastroenterology, Faculty of Medicine, University of Jordan, Amman 11942, JordanDepartment of Surgery, Mayo Clinic, Phoenix, AZ 85054, USAObjective. To report an unusual cause of coma in an adult. Design. Case report. Setting. University teaching hospital. Patient. A previously healthy 53-year-old man initially presented with altered mental status and progressed to coma. He was found to be substantially hyperammonemic and did not improve with lactulose therapy and continuous venovenous hemodialysis. Results. Biochemical testing revealed previously undiagnosed ornithine transcarbamylase deficiency, and the patient responded to arginine, sodium phenylacetate, and sodium benzoate. Conclusion. Even in adult patients with no known history, inborn errors of metabolism must be considered in the differential diagnosis of unexplained coma. Defects of the urea cycle can present with an unprovoked hyperammonemic coma.http://dx.doi.org/10.1155/2013/493216
spellingShingle Daniel L. Roberts
David A. Galbreath
Bhavesh M. Patel
Timothy J. Ingall
Amer Khatib
Daniel J. Johnson
Hyperammonemic Coma in an Adult due to Ornithine Transcarbamylase Deficiency
Case Reports in Critical Care
title Hyperammonemic Coma in an Adult due to Ornithine Transcarbamylase Deficiency
title_full Hyperammonemic Coma in an Adult due to Ornithine Transcarbamylase Deficiency
title_fullStr Hyperammonemic Coma in an Adult due to Ornithine Transcarbamylase Deficiency
title_full_unstemmed Hyperammonemic Coma in an Adult due to Ornithine Transcarbamylase Deficiency
title_short Hyperammonemic Coma in an Adult due to Ornithine Transcarbamylase Deficiency
title_sort hyperammonemic coma in an adult due to ornithine transcarbamylase deficiency
url http://dx.doi.org/10.1155/2013/493216
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