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Lethal Hyperammonemia due to Ornithine Transcarbamylase Deficiency in a Patient with Severe Septic Shock

 Ji An Hwang  ;  Joo Han Song  ;  Young Seok Lee  ;  Kyung Soo Chung  ;  Song Yee Kim  ;  Eun Young Kim  ;  Ji Ye Jung  ;  Young Ae Kang  ;  Young Sam Kim  ;  Joon Chang  ;  Moo Suk Park 
 Korean Journal of Critical Care Medicine, Vol.31(2) : 140-145, 2016 
Journal Title
 Korean Journal of Critical Care Medicine 
Issue Date
cerebral edema ; hyperammonemia ; ornithine transcarbamylase deficiency ; respiratory failure ; septic shock.
Severe hyperammonemia can occur as a result of inherited or acquired liver enzyme defects in the urea cycle, among which ornithine transcarbamylase deficiency (OTCD) is the most common form. We report a very rare case of a 45-year-old Korean male who was admitted to the intensive care unit (ICU) due to severe septic shock with acute respiratory failure caused by Pneumocystis jiroveci pneumonia. During his ICU stay with ventilator care, the patient suffered from marked hyperammonemia (>1,700 μg/dL) with abrupt mental change leading to life-threatening cerebral edema. Despite every effort including continuous renal replacement therapy and use of a molecular adsorbent recirculating system (extracorporeal liver support?albumin dialysis) to lower his serum ammonia level, the patient was not recovered. The lethal hyperammonemia in the patient was later proven to be a manifestation of acquired liver enzyme defect known as OTCD, which is triggered by serious catabolic conditions, such as severe septic shock with acute respiratory failure.
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1. College of Medicine (의과대학) > Dept. of Internal Medicine (내과학교실) > 1. Journal Papers
Yonsei Authors
강영애(Kang, Young Ae) ORCID logo https://orcid.org/0000-0002-7783-5271
김송이(Kim, Song Yee) ORCID logo https://orcid.org/0000-0001-8627-486X
김영삼(Kim, Young Sam) ORCID logo https://orcid.org/0000-0001-9656-8482
김은영(Kim, Eun Young) ORCID logo https://orcid.org/0000-0002-3281-5744
박무석(Park, Moo Suk) ORCID logo https://orcid.org/0000-0003-0820-7615
송주한(Song, Joo Han)
장준(Chang, Joon) ORCID logo https://orcid.org/0000-0003-4542-6841
정경수(Jung, Kyung Soo) ORCID logo https://orcid.org/0000-0003-1604-8730
정지예(Jung, Ji Ye) ORCID logo https://orcid.org/0000-0003-1589-4142
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