Pseudocholinesterase (butyrylcholinesterase) deficiency is an inherited or acquired condition in which the serum pseudocholinesterase levels are absent or lower than normal. The enzyme is produced by the liver; decreased levels of the enzyme in an individual cause increased sensitivity to anesthetic agents, like succinylcholine and mivacurium. Pseudocholinesterase deficiency is caused by butyrylcholinesterase (BCHE) gene mutation, a gene that provides instructions for making the pseudocholinesterase enzyme. Succinylcholine is a depolarizing muscle relaxant that provides a quicker onset and a brief duration of muscle relaxation during general anesthesia. In this article, we would like to discuss a case report of prolonged intubation and ventilation in a patient with pseudocholinesterase deficiency and the necessity of succinylcholine during intubation in comparison to possible alternatives (rocuronium).
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http://dx.doi.org/10.7759/cureus.10721 | DOI Listing |
Cureus
October 2024
Anesthesiology, East Suffolk and North Essex NHS Foundation Trust, Colchester, GBR.
Suxamethonium is commonly used as a muscle relaxant during electroconvulsive therapy (ECT). Prolonged apnoea associated with suxamethonium, mostly caused by pseudocholinesterase deficiency, is rare, but it can sometimes pose a great challenge in managing emergency situations due to patients' inability to breathe; occasionally, it can result in serious complications, including death. We report a case of a young male who had no prior exposure to suxamethonium and developed prolonged apnoea after induction with it.
View Article and Find Full Text PDFMedicine (Baltimore)
October 2024
Department of Cardiovascular, People's Hospital of Changshou Chongqing, Chongqing, China.
Rationale: Congenital butyrylcholinesterase deficiency (BCHED) is a rare autosomal recessive genetic disorder caused by a pathogenic mutation in the BCHE gene. Patients with BCHED may experience prolonged apnea or even death after the application of drugs such as succinylcholine. We aimed to identify the genetic basis of disease in a patient presenting with butyrylcholinesterase deficiency in order to confirm the diagnosis, expand BCHE gene mutation spectrum, and elucidate potential genotype-phenotype associations to inform management.
View Article and Find Full Text PDFNeurodiagn J
September 2024
Department of SurgeryAxis Neuromonitoring, Richardson, Texas.
S D Med
June 2024
Avera Sacred Heart Hospital, Yankton, South Dakota.
Background: Pseudocholinesterase (butyrylcholinesterase) deficiency is an acquired or inherited condition in which decreased plasma levels of the pseudocholinesterase enzyme lead to an inability to metabolize the neuromuscular blocking agents succinylcholine and mivacurium, prolonging their paralytic effects. This often results in delayed extubation and additional intensive care requirements in the postoperative period.
Case Description: We describe a case of suspected pseudocholinesterase deficiency in a previously healthy 59-year-old female who underwent a left thyroid lobectomy and isthmusectomy.
AANA J
April 2024
is a Professor, Department of Anesthesiology, The University of North Carolina at Chapel Hill, Chapel Hill, North Carolina.
Administration of succinylcholine to patients with a variant in the butyrylcholinesterase (BChE) gene increases the risk of anesthesia emergence prior to recovery from neuromuscular blockade (NMB). Application of quantitative neuromuscular monitoring (NMM) can identify residual NMB. We present two patients with abnormal BChE gene variants.
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