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

DC Field Value Language
dc.contributor.author강영애-
dc.contributor.author김송이-
dc.contributor.author김영삼-
dc.contributor.author김은영-
dc.contributor.author박무석-
dc.contributor.author송주한-
dc.contributor.author장준-
dc.contributor.author정경수-
dc.contributor.author정지예-
dc.date.accessioned2017-10-26T07:40:00Z-
dc.date.available2017-10-26T07:40:00Z-
dc.date.issued2016-
dc.identifier.issn2383-4870-
dc.identifier.urihttps://ir.ymlib.yonsei.ac.kr/handle/22282913/152351-
dc.description.abstractSevere 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.-
dc.description.statementOfResponsibilityopen-
dc.languageEnglish-
dc.publisherKorean Society of Critical Care Medicine-
dc.relation.isPartOfKorean Journal of Critical Care Medicine-
dc.rightsCC BY-NC-ND 2.0 KR-
dc.rights.urihttps://creativecommons.org/licenses/by-nc-nd/2.0/kr/-
dc.titleLethal Hyperammonemia due to Ornithine Transcarbamylase Deficiency in a Patient with Severe Septic Shock-
dc.typeArticle-
dc.publisher.locationKorea-
dc.contributor.collegeCollege of Medicine-
dc.contributor.departmentDept. of Internal Medicine-
dc.contributor.googleauthorJi An Hwang-
dc.contributor.googleauthorJoo Han Song-
dc.contributor.googleauthorYoung Seok Lee-
dc.contributor.googleauthorKyung Soo Chung-
dc.contributor.googleauthorSong Yee Kim-
dc.contributor.googleauthorEun Young Kim-
dc.contributor.googleauthorJi Ye Jung-
dc.contributor.googleauthorYoung Ae Kang-
dc.contributor.googleauthorYoung Sam Kim-
dc.contributor.googleauthorJoon Chang-
dc.contributor.googleauthorMoo Suk Park-
dc.identifier.doi10.4266/kjccm.2016.31.2.140-
dc.contributor.localIdA00626-
dc.contributor.localIdA00707-
dc.contributor.localIdA00811-
dc.contributor.localIdA01457-
dc.contributor.localIdA02062-
dc.contributor.localIdA03472-
dc.contributor.localIdA03570-
dc.contributor.localIdA03735-
dc.contributor.localIdA00057-
dc.relation.journalcodeJ01995-
dc.relation.journalsince2004~-
dc.relation.journalbefore~2003 Korean Journal of Critical Care Medicine (대한중환자의학회지)-
dc.subject.keywordcerebral edema-
dc.subject.keywordhyperammonemia-
dc.subject.keywordornithine transcarbamylase deficiency-
dc.subject.keywordrespiratory failure-
dc.subject.keywordseptic shock.-
dc.contributor.alternativeNameKang, Young Ae-
dc.contributor.alternativeNameKim, Song Yi-
dc.contributor.alternativeNameKim, Young Sam-
dc.contributor.alternativeNameKim, Eun Young-
dc.contributor.alternativeNamePark, Moo Suk-
dc.contributor.alternativeNameSong, Joo Han-
dc.contributor.alternativeNameChang, Joon-
dc.contributor.alternativeNameJung, Kyung Soo-
dc.contributor.alternativeNameJung, Ji Ye-
dc.contributor.affiliatedAuthorKim, Song Yi-
dc.contributor.affiliatedAuthorKim, Young Sam-
dc.contributor.affiliatedAuthorKim, Eun Young-
dc.contributor.affiliatedAuthorPark, Moo Suk-
dc.contributor.affiliatedAuthorSong, Joo Han-
dc.contributor.affiliatedAuthorChang, Joon-
dc.contributor.affiliatedAuthorJung, Kyung Soo-
dc.contributor.affiliatedAuthorJung, Ji Ye-
dc.contributor.affiliatedAuthorKang, Young Ae-
dc.citation.volume31-
dc.citation.number2-
dc.citation.startPage140-
dc.citation.endPage145-
dc.identifier.bibliographicCitationKorean Journal of Critical Care Medicine, Vol.31(2) : 140-145, 2016-
dc.date.modified2017-10-24-
dc.identifier.rimsid48086-
dc.type.rimsART-
Appears in Collections:
1. College of Medicine (의과대학) > Dept. of Internal Medicine (내과학교실) > 1. Journal Papers

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