Resistance to Rocuronium in a Patient with Propionic Acidemia Undergoing Appendectomy for Acute Appendicitis: A Case Report with Literature Review
1University Medical Centre Ljubljana, Slovenia and University of Ljubljana, Faculty of Medicine, Slovenia
2University Medical Centre Ljubljana, Slovenia
DOI: 10.22514/sv.2020.16.0026 Vol.16,Issue 1,June 2020 pp.187-191
Published: 30 June 2020
Propionic acidemia is a rare metabolic disorder caused by a deficiency of the mitochondrial enzyme, propionyl-CoA carboxylase, which is required in the catabolic pathways of several amino acids, odd-chain fatty acids and the side chains of cholesterol. Clinically, the disorder may present with multisystemic manifestations including metabolic ketoacidosis, hyperlactatemia, hyperammonemia, developmental delay and neurological dysfunction. The key principle in managing or preventing acute exacerbations consists of protein restricted diet and avoidance of factors that may potentiate relevant catabolic pathways. We describe an unusual case of a female patient with propionic acidemia undergoing appendectomy for acute appendicitis, who was resistant to a nondepolarizing neuromuscular blocker (rocuronium). In literature, there has been reported cases of resistance to nondepolarizing neuromuscular blockers associated with certain pathologic conditions or medications, but such resistance has not been previously described in the context of propionic acidemia. Rocuronium resistance in our index patient could be attributable to a sum of potential etiologic factors including the use of valproic acid, and upper motor neuron damage, a rare complication of chronic propionic acidemia.
Propionic academia, Nondepolarizing muscle relaxants, Rocuronium, Appendicitis, Anesthesia
Jurij Janež,Armand Dominik Škapin. Resistance to Rocuronium in a Patient with Propionic Acidemia Undergoing Appendectomy for Acute Appendicitis: A Case Report with Literature Review. Signa Vitae. 2020. 16(1);187-191.
 Rafique M. Emerging trends in management of propionic acidemia. Arq Bras Endocrinol Metabol. 2014;58:237-42.
 Ryu J, Shin YH, Ko JS, Gwak MS, Kim GS. Intractable metabolic acidosis in a child with propionic acidemia undergoing liver transplantation – a case report. Korean J Anesthesiol. 2013;65:257-61.
 Baumgartner MR, Hörster F, Dionisi-Vici C, Haliloglu G, Karall D, Chapman KA, et al. Proposed guidelines for the diagnosis and management of methylmalonic and propionic acidemia. Orphanet J Rare Dis. 2014;9:130.
 Harker HE, Emhardt JD, Hainline BE. Propionic acidemia in a four-month-old male: a case study and anesthetic implications. Anesth Analg. 2000;91:309-11.
 Soberón JR, Elliott CE, Bland KS, Weinberg GL. Peripheral nerve block in a patient with propionic acidemia. Reg Anesth Pain Med. 2014;39:560-1.
 Sebens EL. A child with propionic acidemia undergoing dental restorations: a case report. AANA J. 2011;79:295-299.
 Silva HM, Nassogne MC, Smets F, Stéphenne X, Scheers I, Veyckemans F, et al. Liver Transplantation for Propionic Acidemia. J Pediatr Gastroenterol Nutr. 2017;64:e73-e76.
 Deana C, Barbariol F, D’Incà S, Pompei L, Rocca GD. SUGAM-MADEX Versus Neostigmine After ROCURONIUM Continuous Infusion in Patients Undergoing Liver Transplantation. BMC Anes-thesiol. 2020;20:70.
 Fuchs-Buder T, Schlaich N, Ziegenfuss T. [Rocuronium for anes-thesia induction in elective procedures. Time course of muscular blockade and intubation after administration of 2-compartment ED95 (0.6 mg/kg) and dose reduction (0.4 mg/kg)]. Anaesthesist. 1999;48:164-8.
 Martyn JA, White DA, Gronert GA, Jaffe RS, Ward JM. Up-and-down Regulation of Skeletal Muscle Acetylcholine Receptors. Effects on Neuromuscular Blockers. Anesthesiology. 1992;76:822-43.
 Capuano A, Sullo MG, Rafaniello C, Sportiello L, Fusco P, De Vizia M, et al. Complete resistance after maximal dose of rocuronium. J Pharmacol Pharmacother. 2015;6:175-178.
 Soriano SG, Martyn JA. Antiepileptic-induced Resistance to Neu-romuscular Blockers: Mechanisms and Clinical Significance. Clin Pharmacokinet. 2004;43:71-81.
 Anderson GD. A Mechanistic Approach to Antiepileptic Drug Interactions. Ann Pharmacother. 1998;32:554-63.
 Lien CA, Eikermann M. 22 – Neuromuscular Blockers and Reversal Drugs. In: Hemmings HC, Egan TD, editors. Pharmacology and Physiology for Anesthesia, 2nd ed. Philadelphia, PA: Elsevier, Inc. 2019.p.428-454.
 Kim MH, Hwang JW, Jeon YT, Do SH. Effects of valproic acid and magnesium sulphate on rocuronium requirement in patients undergoing craniotomy for cerebrovascular surgery. Br J Anaesth. 2012;109:407-12.
Science Citation Index Expanded (SciSearch) The Science Citation Index (SCI) is a citation index originally produced by the Institute for Scientific Information (ISI) and created by Eugene Garfield. It (Science Citation Index Expanded) covers more than 8,500 notable and significant journals, across 150 disciplines in science and technology, from 1900 to the present.
Journal Citation Reports/Science Edition Journal Citation Reports/Science Edition aims to evaluate a journal’s value from multiple perspectives including the journal impact factor, descriptive data about a journal’s open access content as well as contributing authors, and provide readers a transparent and publisher-neutral data & statistics information about the journal.
Chemical Abstracts Service Source Index The CAS Source Index (CASSI) Search Tool is an online resource that can quickly identify or confirm journal titles and abbreviations for publications indexed by CAS since 1907, including serial and non-serial scientific and technical publications.
IndexCopernicus The Index Copernicus International (ICI) Journals database’s is an international indexation database of scientific journals. It covered international scientific journals which divided into general information, contents of individual issues, detailed bibliography (references) sections for every publication, as well as full texts of publications in the form of attached files (optional). For now, there are more than 58,000 scientific journals registered at ICI.
Geneva Foundation for Medical Education and Research The Geneva Foundation for Medical Education and Research (GFMER) is a non-profit organization established in 2002 and it works in close collaboration with the World Health Organization (WHO). The overall objectives of the Foundation are to promote and develop health education and research programs.
Scopus Scopus is Elsevier's abstract and citation database launched in 2004. Scopus covers nearly 36,377 titles (22,794 active titles and 13,583 Inactive titles) from approximately 11,678 publishers, of which 34,346 are peer-reviewed journals in top-level subject fields: life sciences, social sciences, physical sciences and health sciences.
Embase Embase (often styled EMBASE for Excerpta Medica dataBASE), produced by Elsevier, is a biomedical and pharmacological database of published literature designed to support information managers and pharmacovigilance in complying with the regulatory requirements of a licensed drug.
Editorial review: 1 - 2 days
Peer review: 1 - 2 months
Ahead of Print: within 2 months after being accepted
Notes: Your information is kept confide-ntial throughout the review process.