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Title: Successful perioperative management of living-donor liver transplantation for a patient with severe methylmalonic acidemia: a case report
Authors: Hirotsu, Akiko  kyouindb  KAKEN_id
Kusudo, Eriko
Mori, Natsumi
Miyai, Yoshimitsu
Suzuki, Kengo
Kawamoto, Shuji  kyouindb  KAKEN_id  orcid https://orcid.org/0000-0003-3172-0855 (unconfirmed)
Fukuda, Kazuhiko  kyouindb  KAKEN_id
Author's alias: 廣津, 聡子
楠戸, 絵梨子
宮井, 善三
鈴木, 堅悟
川本, 修司
福田, 和彦
Keywords: Methylmalonic acidemia
Liver transplantation
Metabolic acidosis
Perioperative management
Issue Date: 27-Dec-2018
Publisher: Springer Nature
Journal title: JA Clinical Reports
Volume: 4
Thesis number: 83
Abstract: [Background]Methylmalonic acidemia (MMAemia) is a rare hereditary disease affecting organic acid metabolism. It causes recurrent metabolic acidosis and secondary mitochondrial dysfunction, resulting in a poor prognosis. Liver transplantation (LT) has been performed to facilitate the metabolism of organic acids and improve the prognosis of MMAemia. However, there have been few reports on perioperative management of LT. [Case presentation]A 22-month-old female with severe MMAemia was scheduled to receive LT to relieve recurrent metabolic acidosis despite dietary and pharmacological treatment. General anesthesia was maintained without propofol or nitrous oxide, which can worsen MMAemia-induced metabolic acidosis during anesthesia for LT. Strict metabolic and respiratory management enabled the operation to be successfully performed without metabolic acidosis. [Conclusion]Perioperative management of LT for MMAemia is challenging for anesthesiologists because of the possibility of serious metabolic acidosis. We succeeded in preventing metabolic decompensation by avoiding the use of propofol and nitrous oxide.
Rights: © The Author(s). This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made.
URI: http://hdl.handle.net/2433/263330
DOI(Published Version): 10.1186/s40981-018-0221-y
PubMed ID: 32026114
Appears in Collections:Journal Articles

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