Perioperative management of living‐donor liver transplantation for methylmalonic acidemia

Summary Methymalonic acidemia (MMA) is a hereditary metabolic disorder characterized by a defect of the methylmalonyl‐CoA mutase that breaks down propionate. The efficacy of liver transplantation for MMA was recently reported. However, the anesthetic management of liver transplant for MMA is not clear. The aim of this article is to describe an anesthetic management algorithm of liver transplant for MMA by reviewing our cases of liver transplant for MMA. Fourteen patients received a liver transplant; three cases showed metabolic decompensation during the transplant and two of the patients died. In the two patients who expired, propofol was used for maintenance anesthesia and preoperative continuous hemodiafiltration was used to reduce plasma methylmalonic acid level in one case, and to control severe metabolic decompensation before transplant for the other case. Their renal function was also worse than others and they were already experiencing metabolic decompensation before induction of anesthesia. Based on our experience of these 14 cases, we have established an anesthetic algorithm for patients with MMA undergoing liver transplant or other procedures. There are three important points in our experience: propofol should be avoided, dextrose infusion therapy should be continued to prevent metabolic decompensation, and liver transplant or other procedures should be avoided during metabolic decompensation.
Source: Pediatric Anesthesia - Category: Anesthesiology Authors: Tags: Special Interest Article Source Type: research

Related Links:

Publication date: Available online 25 February 2020Source: MitochondrionAuthor(s): Viraj Muthye, Dennis V. Lavrov
Source: Mitochondrion - Category: Biochemistry Source Type: research
Publication date: Available online 24 February 2020Source: MitochondrionAuthor(s): Deepika Kundu, Ritu Pasrija
Source: Mitochondrion - Category: Biochemistry Source Type: research
Publication date: Available online 24 February 2020Source: Microvascular ResearchAuthor(s): A. Alper Öztürk, İrem Namlı, Kadri Güleç, H. Tuba Kıyan
Source: Microvascular Research - Category: Biochemistry Source Type: research
Publication date: Available online 25 February 2020Source: Polymer TestingAuthor(s): Cijun Shuai, Xun Yuan, Wenjing Yang, Shuping Peng, Chongxian He, Pei Feng, Fangwei Qi, Guoyong Wang
Source: Polymer Testing - Category: Chemistry Source Type: research
Publication date: Available online 25 February 2020Source: Polymer TestingAuthor(s): Sattaiah Naidu K, Abhijeet S. Kate, Vikas Kshirsagar, R. Ganeshan, Tukaram Gunale, Bing Zhou, Samir Anapat, Yusuf Sulub, Arun Kumar, Narayana Rao
Source: Polymer Testing - Category: Chemistry Source Type: research
Publication date: April 2020Source: Phytochemistry Letters, Volume 36Author(s): Zeynep Dogan, Kan’ichiro Ishiuchi, Toshiaki Makino, Iclal Saracoglu
Source: Phytochemistry Letters - Category: Chemistry Source Type: research
Publication date: Available online 24 February 2020Source: Computational and Theoretical ChemistryAuthor(s): F. Paularokiadoss, A. Sekar, Thayalaraj Christopher Jeyakumar
Source: Computational and Theoretical Chemistry - Category: Chemistry Source Type: research
Soft Matter, 2020, Advance Article DOI: 10.1039/C9SM02494H, PaperGhazi Ben Messaoud, Patrick Le Griel, Daniel Hermida-Merino, Niki Baccile The structure-properties relationship of lipid lamellar hydrogels composed of a biobased microbial glucolipid biosurfactant is studied against pH, temperature and shear rate usingin situ rheo-SAXS experiments. To cite this article before page numbers are assigned, use the DOI form of citation above. The content of this RSS Feed (c) The Royal Society of Chemistry
Source: RSC - Soft Matter latest articles - Category: Chemistry Authors: Source Type: research
Soft Matter, 2020, Accepted Manuscript DOI: 10.1039/D0SM00001A, Review ArticleCharles E Sing, Sarah L Perry Complex coacervation is an associative, liquid-liquid phase separation that can occur in solutions of oppositely-charged macromolecular species, such as proteins, polymers, and colloids. This process results in a coacervate phase,... The content of this RSS Feed (c) The Royal Society of Chemistry
Source: RSC - Soft Matter latest articles - Category: Chemistry Authors: Source Type: research
Phys. Chem. Chem. Phys., 2020, Accepted Manuscript DOI: 10.1039/C9CP05699H, PaperMeng-Meng Wang, Yan-Xia Zhao, Xun-Lei Ding, Wei Li, Sheng-Gui He The ability of transition metals to activate methane is quite different, and it is attractive to find the most suitable metal for the direct conversion of methane to value-added chemicals.... The content of this RSS Feed (c) The Royal Society of Chemistry
Source: RSC - Phys. Chem. Chem. Phys. latest articles - Category: Chemistry Authors: Source Type: research
More News: Anesthesia | Anesthesiology | Dialysis | Diprivan | Hemodiafiltration | Liver | Liver Transplant | Pediatrics | Propofol | Transplant Surgery | Transplants | Urology & Nephrology