Malignant ventricular dysrhythmias in a patient with isovaleric acidemia receiving general and local anesthesia for suction lipectomy

Weinberg, Guy; Laurito, Charles E.; Geldner, Peter; Pygon, Bernard H.; Burton, Barbara K.

doi:10.1016/s0952-8180(97)00187-6

Cited by 70 publications

(46 citation statements)

References 13 publications

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“…Acute episodes may be misdiagnosed as diabetic ketoacidosis due to hyperglycemia, acidosis and the apparent presence of blood and urinary ketones [Attia et al, 1996]. Acute pancreatitis, myeloproliferative syndrome, Fanconi syndrome, and cardiac arrhythmias have been reported [Arnold et al, 1986;Gilbert-Barness and Barness, 1999;Kahler et al, 1994;Weinberg et al, 1997]; abnormalities of the globus pallidus can be seen [Sogut et al, 2004]. The variability of this disorder is highlighted by the diagnosis of IVA in a previously well 18-year-old man who developed acute nausea, vomiting, and mental status changes during basic training camp for the United States Air Force [Feinstein and O'Brien, 2003].…”

Section: Clinical Presentationmentioning

confidence: 99%

Isovaleric acidemia: New aspects of genetic and phenotypic heterogeneity

Vockley

Ensenauer

2006

American J of Med Genetics Pt C

196

161

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Isovaleric acidemia (IVA) is an autosomal recessive inborn error of leucine metabolism caused by a deficiency of the mitochondrial enzyme isovaleryl-CoA dehydrogenase (IVD) resulting in the accumulation of derivatives of isovaleryl-CoA. It was the first organic acidemia recognized in humans and can cause significant morbidity and mortality. Early diagnosis and treatment with a protein restricted diet and supplementation with carnitine and glycine are effective in promoting normal development in severely affected individuals. Both intra-and inter-familial variability have been recognized. Initially, two phenotypes with either an acute neonatal or a chronic intermittent presentation were described. More recently, a third group of individuals with mild biochemical abnormalities who can be asymptomatic have been identified through newborn screening of blood spots by tandem mass spectrometry. IVD is a flavoenzyme that catalyzes the conversion of isovaleryl-CoA to 3-methylcrotonyl-CoA and transfers electrons to the electron transfer flavoprotein. Human IVD has been purified from tissue and recombinant sources and its biochemical and physical properties have been extensively studied. Molecular analysis of the IVD gene from patients with IVA has allowed characterization of different types of mutations in this gene. One missense mutation, 932C>T (A282V), is particularly common in patients identified through newborn screening with mild metabolite elevations and who have remained asymptomatic to date. This mutation leads to a partially active enzyme with altered catalytic properties; however, its effects on clinical outcome and the necessity of therapy are still unknown. A better understanding of the heterogeneity of this disease and the relevance of genotype/phenotype correlations to clinical management of patients are among the challenges remaining in the study of this disorder in the coming years.

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Section: Clinical Presentationmentioning

confidence: 99%

Isovaleric acidemia: New aspects of genetic and phenotypic heterogeneity

Vockley

Ensenauer

2006

American J of Med Genetics Pt C

196

161

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“…6,7,21 Although published case reports [21][22][23][24][25] indicate that one must always be aware of the risk of bupivacaine toxicity, considered and Volume 124, Number 4 • Bupivacaine in Tumescent Liposuction appropriate use of bupivacaine within the field of pain management has shown that the drug can provide safe, long-acting pain control. 10 -20 This dichotomy underscores how important it is to develop good evidence-based practice with regard to preparation and administration of wetting solutions for tumescent liposuction.…”

Section: Discussionmentioning

confidence: 99%

Intraoperative Use of Bupivacaine for Tumescent Liposuction: The Robert Wood Johnson Experience

Failey

Vemula²,

Borah³

et al. 2009

Plastic and Reconstructive Surgery

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A review of the experience at Robert Wood Johnson University Hospital reveals that the intraoperative use of bupivacaine for tumescent liposuction in 27 cases appeared to be as safe as other tumescent additives. There did not appear to be a significant difference in the incidence of adverse events or postoperative length of stay for patients who underwent liposuction with bupivacaine compared with other wetting solutions. Among a subset of patients who underwent concurrent abdominoplasty and liposuction procedures, patients who received bupivacaine spent significantly less time in the hospital postoperatively than those who did not receive it. Conducting future prospective studies involving larger samples among multiple centers is an essential next step to confirm these findings.

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“…Consistent with this hypothesis, supplementation with individual agents including AICAR, 26 ATP, 41 and Akt inhibitors including rapamycin and LY294002 25 modify toxicity with bupivacaine in a synergistic or antagonistic fashion. Furthermore, metabolic conditions including diabetes 42,43 and/or deficiency of specific enzymes or cofactors including carnitine 44,45 increase susceptibility to local anesthetic toxicity. Controlling for these effects adds a layer of complexity to the necessary scientific studies, requiring controls for both local anesthetic and for lipid emulsion.…”

Section: Local Anesthestic Systemic Toxicitymentioning

confidence: 99%

The Mechanisms Underlying Lipid Resuscitation Therapy

Fettiplace

Weinberg

2018

Regional Anesthesia and Pain Medicine

111

104

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The experimental use of lipid emulsion for local anesthetic toxicity was originally identified in 1998. It was then translated to clinical practice in 2006 and expanded to drugs other than local anesthetics in 2008. Our understanding of lipid resuscitation therapy has progressed considerably since the previous update from the American Society of Regional Anesthesia and Pain Medicine, and the scientific evidence has coalesced around specific discrete mechanisms. Intravenous lipid emulsion therapy provides a multimodal resuscitation benefit that includes both scavenging (eg, the lipid shuttle) and nonscavenging components. The intravascular lipid compartment scavenges drug from organs susceptible to toxicity and accelerates redistribution to organs where drug (eg, bupivacaine) is stored, detoxified, and later excreted. In addition, lipid exerts nonscavenging effects that include postconditioning (via activation of prosurvival kinases) along with cardiotonic and vasoconstrictive benefits. These effects protect tissue from ischemic damage and increase tissue perfusion during recovery from toxicity. Other mechanisms have diminished in favor based on lack of evidence; these include direct effects on channel currents (eg, calcium) and mass-effect overpowering a block in mitochondrial metabolism. In this narrative review, we discuss these proposed mechanisms and address questions left to answer in the field. Further work is needed, but the field has made considerable strides towards understanding the mechanisms.

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Malignant ventricular dysrhythmias in a patient with isovaleric acidemia receiving general and local anesthesia for suction lipectomy

Cited by 70 publications

References 13 publications

Isovaleric acidemia: New aspects of genetic and phenotypic heterogeneity

Isovaleric acidemia: New aspects of genetic and phenotypic heterogeneity

Intraoperative Use of Bupivacaine for Tumescent Liposuction: The Robert Wood Johnson Experience

The Mechanisms Underlying Lipid Resuscitation Therapy

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