Drug management in emergent liver transplantation of mitochondrial disorder carriers: Review of the literature

Youri Vater, Gregory Dembo, Kenneth Martay, Yifat Klein, Alex Vitin, Avi A. Weinbroum

Research output: Contribution to journalArticle

1 Citation (Scopus)

Abstract

Background: Mitochondrial respiratory-chain disorders (MRCD) lead to progressive disabling of neurological and cellular conditions that involve muscles, brain, kidney, and liver dysfunction. Affected individuals may need surgery, including orthotopic liver transplantation (OLT). Surgery poses anesthesia challenges because of the prolonged use of anesthetic drugs and sedatives, which may inhibit oxidative phosphorylation, mimic mitochondrial cytopathic disorders, or unveil them ex novo. Materials and methods: We conducted a multilingual PubMed search of surgical and non-surgical anesthesia reports between the years 1992 and 2008, where anesthetic drugs were used in MRCD patients, especially for those undergoing urgent OLTs. Results: There were 51 case reports of 210 anesthesia and critical care interventions in patients with MRCD, a large part of them were children. Data pertaining to the safe usage of anesthesia and perioperative drugs were limited and conflicting. We found no article that addressed the issue of perioperative handling of urgent OLT in MRCD patients. We therefore suggest our own - although limited - experience for such occasions. Conclusion: There are no randomized, controlled, trial-based indications regarding safe anesthetic drugs to be used perioperatively in MRCD carriers. Consultation among geneticists, anesthesiologists, intensivists, and surgeons is essential in patients with known/suspected metabolic syndrome for planning appropriate perioperative care.

Original languageEnglish (US)
Pages (from-to)E43-E53
JournalClinical Transplantation
Volume24
Issue number2
DOIs
StatePublished - Mar 1 2010

Fingerprint

Mitochondrial Diseases
Electron Transport
Liver Transplantation
Anesthesia
Anesthetics
Pharmaceutical Preparations
Perioperative Care
Oxidative Phosphorylation
Critical Care
Hypnotics and Sedatives
PubMed
Liver Diseases
Referral and Consultation
Randomized Controlled Trials
Kidney
Muscles
Brain

All Science Journal Classification (ASJC) codes

  • Transplantation

Cite this

Vater, Youri ; Dembo, Gregory ; Martay, Kenneth ; Klein, Yifat ; Vitin, Alex ; Weinbroum, Avi A. / Drug management in emergent liver transplantation of mitochondrial disorder carriers : Review of the literature. In: Clinical Transplantation. 2010 ; Vol. 24, No. 2. pp. E43-E53.
@article{d00dc4094c7c4ecb8d3148221d780aef,
title = "Drug management in emergent liver transplantation of mitochondrial disorder carriers: Review of the literature",
abstract = "Background: Mitochondrial respiratory-chain disorders (MRCD) lead to progressive disabling of neurological and cellular conditions that involve muscles, brain, kidney, and liver dysfunction. Affected individuals may need surgery, including orthotopic liver transplantation (OLT). Surgery poses anesthesia challenges because of the prolonged use of anesthetic drugs and sedatives, which may inhibit oxidative phosphorylation, mimic mitochondrial cytopathic disorders, or unveil them ex novo. Materials and methods: We conducted a multilingual PubMed search of surgical and non-surgical anesthesia reports between the years 1992 and 2008, where anesthetic drugs were used in MRCD patients, especially for those undergoing urgent OLTs. Results: There were 51 case reports of 210 anesthesia and critical care interventions in patients with MRCD, a large part of them were children. Data pertaining to the safe usage of anesthesia and perioperative drugs were limited and conflicting. We found no article that addressed the issue of perioperative handling of urgent OLT in MRCD patients. We therefore suggest our own - although limited - experience for such occasions. Conclusion: There are no randomized, controlled, trial-based indications regarding safe anesthetic drugs to be used perioperatively in MRCD carriers. Consultation among geneticists, anesthesiologists, intensivists, and surgeons is essential in patients with known/suspected metabolic syndrome for planning appropriate perioperative care.",
author = "Youri Vater and Gregory Dembo and Kenneth Martay and Yifat Klein and Alex Vitin and Weinbroum, {Avi A.}",
year = "2010",
month = "3",
day = "1",
doi = "10.1111/j.1399-0012.2009.01203.x",
language = "English (US)",
volume = "24",
pages = "E43--E53",
journal = "Clinical Transplantation",
issn = "0902-0063",
publisher = "Wiley-Blackwell",
number = "2",

}

Drug management in emergent liver transplantation of mitochondrial disorder carriers : Review of the literature. / Vater, Youri; Dembo, Gregory; Martay, Kenneth; Klein, Yifat; Vitin, Alex; Weinbroum, Avi A.

In: Clinical Transplantation, Vol. 24, No. 2, 01.03.2010, p. E43-E53.

Research output: Contribution to journalArticle

TY - JOUR

T1 - Drug management in emergent liver transplantation of mitochondrial disorder carriers

T2 - Review of the literature

AU - Vater, Youri

AU - Dembo, Gregory

AU - Martay, Kenneth

AU - Klein, Yifat

AU - Vitin, Alex

AU - Weinbroum, Avi A.

PY - 2010/3/1

Y1 - 2010/3/1

N2 - Background: Mitochondrial respiratory-chain disorders (MRCD) lead to progressive disabling of neurological and cellular conditions that involve muscles, brain, kidney, and liver dysfunction. Affected individuals may need surgery, including orthotopic liver transplantation (OLT). Surgery poses anesthesia challenges because of the prolonged use of anesthetic drugs and sedatives, which may inhibit oxidative phosphorylation, mimic mitochondrial cytopathic disorders, or unveil them ex novo. Materials and methods: We conducted a multilingual PubMed search of surgical and non-surgical anesthesia reports between the years 1992 and 2008, where anesthetic drugs were used in MRCD patients, especially for those undergoing urgent OLTs. Results: There were 51 case reports of 210 anesthesia and critical care interventions in patients with MRCD, a large part of them were children. Data pertaining to the safe usage of anesthesia and perioperative drugs were limited and conflicting. We found no article that addressed the issue of perioperative handling of urgent OLT in MRCD patients. We therefore suggest our own - although limited - experience for such occasions. Conclusion: There are no randomized, controlled, trial-based indications regarding safe anesthetic drugs to be used perioperatively in MRCD carriers. Consultation among geneticists, anesthesiologists, intensivists, and surgeons is essential in patients with known/suspected metabolic syndrome for planning appropriate perioperative care.

AB - Background: Mitochondrial respiratory-chain disorders (MRCD) lead to progressive disabling of neurological and cellular conditions that involve muscles, brain, kidney, and liver dysfunction. Affected individuals may need surgery, including orthotopic liver transplantation (OLT). Surgery poses anesthesia challenges because of the prolonged use of anesthetic drugs and sedatives, which may inhibit oxidative phosphorylation, mimic mitochondrial cytopathic disorders, or unveil them ex novo. Materials and methods: We conducted a multilingual PubMed search of surgical and non-surgical anesthesia reports between the years 1992 and 2008, where anesthetic drugs were used in MRCD patients, especially for those undergoing urgent OLTs. Results: There were 51 case reports of 210 anesthesia and critical care interventions in patients with MRCD, a large part of them were children. Data pertaining to the safe usage of anesthesia and perioperative drugs were limited and conflicting. We found no article that addressed the issue of perioperative handling of urgent OLT in MRCD patients. We therefore suggest our own - although limited - experience for such occasions. Conclusion: There are no randomized, controlled, trial-based indications regarding safe anesthetic drugs to be used perioperatively in MRCD carriers. Consultation among geneticists, anesthesiologists, intensivists, and surgeons is essential in patients with known/suspected metabolic syndrome for planning appropriate perioperative care.

UR - http://www.scopus.com/inward/record.url?scp=77952926522&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=77952926522&partnerID=8YFLogxK

U2 - 10.1111/j.1399-0012.2009.01203.x

DO - 10.1111/j.1399-0012.2009.01203.x

M3 - Article

C2 - 20141521

AN - SCOPUS:77952926522

VL - 24

SP - E43-E53

JO - Clinical Transplantation

JF - Clinical Transplantation

SN - 0902-0063

IS - 2

ER -