지원사업
학술연구/단체지원/교육 등 연구자 활동을 지속하도록 DBpia가 지원하고 있어요.
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연구자들이 자신의 연구와 전문성을 널리 알리고, 새로운 협력의 기회를 만들 수 있는 네트워킹 공간이에요.
논문 기본 정보
- 자료유형
- 학술저널
- 저자정보
- 발행연도
- 2016.1
- 수록면
- 140 - 145 (6page)
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초록· 키워드
오류제보하기
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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등록된 정보가 없습니다.
참고문헌 (14)
참고문헌 신청
Gropman AL / 2007 / Neurological implications of urea cycle disorders / J Inherit Metab Dis 30:865~879
Shawcross D / 2005 / The pathophysiologic basis of hepatic encephalopathy: central role for ammonia and inflammation / Cell Mol Life Sci 62:2295~2304
Vaquero J / 2003 / Pathogenesis of hepatic encephalopathy in acute liver failure / Semin Liver Dis 23:259~269
Clay AS / 2007 / Hyperammonemia in the ICU / Chest 132:1368~1378
Bachmann C / 2003 / Physicians Guide to the Laboratory Diagnosis of Metabolic Diseases / Springer :261~276
Shawcross D / 2005 / The pathophysiologic basis of hepatic encephalopathy: central role for ammonia and inflammation / Cell Mol Life Sci 62:2295~2304
Vaquero J / 2003 / Pathogenesis of hepatic encephalopathy in acute liver failure / Semin Liver Dis 23:259~269
Clay AS / 2007 / Hyperammonemia in the ICU / Chest 132:1368~1378
Bachmann C / 2003 / Physicians Guide to the Laboratory Diagnosis of Metabolic Diseases / Springer :261~276
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