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논문 기본 정보
- 자료유형
- 학술저널
- 저자정보
- 발행연도
- 2020.1
- 수록면
- 472 - 477 (6page)
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Background: Familial amyloid polyneuropathy (FAP) is caused by mutation in a gene transcribing transport protein produced mainly by the liver. Liver transplantation is required to stop FAP progression, but the pathology causes anesthetic management challenges.Case: We report a case of domino living donor liver transplantation in an FAP patient. No intraoperative events occurred; however, during postoperative day 1 in the intensive care unit (ICU), the FAP patient underwent multiple cardiopulmonary resuscitation (CPR) sessions due to pulseless electrical activity following a sudden drop in blood pressure and ventricular tachycardia. Despite ICU management, the patient died after the third CPR session.Conclusions: Various anesthetic management techniques should be considered for FAP patients. Anesthetic management was carefully assessed with the use of isoflurane, isoproterenol, and an external patch. The cause of deterioration in the ICU is unclear, but further investigation is needed to prevent and better manage postoperative morbidity and mortality.
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참고문헌 (15)
참고문헌 신청
Andrade C / 1952 / A peculiar form of peripheral neuropathy; familiar atypical generalized amyloidosis with special involvement of the peripheral nerves / Brain 75:408~427
Benson MD / 2013 / Liver transplantation and transthyretin amyloidosis / Muscle Nerve 47:157~162
Sekijima Y / 2015 / Transthyretin(ATTR)amyloidosis : clinical spectrum, molecular pathogenesis and disease-modifying treatments / J Neurol Neurosurg Psychiatry 86:1036~1043
Adams D / 2000 / The course and prognostic factors of familial amyloid polyneuropathy after liver transplantation / Brain 123(Pt 7):1495~1504
Furtado A / 1997 / Sequential liver transplantation / Transplant Proc 29:467~468
Benson MD / 2013 / Liver transplantation and transthyretin amyloidosis / Muscle Nerve 47:157~162
Sekijima Y / 2015 / Transthyretin(ATTR)amyloidosis : clinical spectrum, molecular pathogenesis and disease-modifying treatments / J Neurol Neurosurg Psychiatry 86:1036~1043
Adams D / 2000 / The course and prognostic factors of familial amyloid polyneuropathy after liver transplantation / Brain 123(Pt 7):1495~1504
Furtado A / 1997 / Sequential liver transplantation / Transplant Proc 29:467~468
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