Lethal Hyperammonemia due to Ornithine Transcarbamylase Deficiency in a Patient with Severe Septic Shock / 대한구급학회지
The Korean Journal of Critical Care Medicine
; : 140-145, 2016.
Article
in En
| WPRIM
| ID: wpr-770933
Responsible library:
WPRO
ABSTRACT
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.
Key words
Full text:
1
Index:
WPRIM
Main subject:
Ornithine
/
Ornithine Carbamoyltransferase
/
Pneumonia
/
Respiratory Insufficiency
/
Shock, Septic
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Urea
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Brain Edema
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Ventilators, Mechanical
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Renal Replacement Therapy
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Ornithine Carbamoyltransferase Deficiency Disease
Limits:
Humans
/
Male
Language:
En
Journal:
The Korean Journal of Critical Care Medicine
Year:
2016
Type:
Article