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KMID : 0604020160310020140
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Korean Journal of Critical Care Medicine
2016 Volume.31 No. 2 p.140 ~ p.0
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Lethal Hyperammonemia due to Ornithine Transcarbamylase Deficiency in a Patient with Severe Septic Shock
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Hwang Ji-An
Song Joo-Han
Lee Young-Seok
Chung Kyung-Soo
Kim Song-Yee
Kim Eun-Young
Jung Ji-Ye
Kang Young-Ae
Kim Young-Sam
Chang Joon
Park Moo-Suk
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Abstract
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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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KEYWORD
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cerebral edema, hyperammonemia, ornithine transcarbamylase deficiency, respiratory failure, septic shock
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