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Pseudocholinesterase deficiency is an inherited or acquired condition in which the metabolism of succinylcholine, mivacurium, or ester local anesthetics is potentially impaired. In this review, genetic inheritance, variants, and testing are examined. Additionally, acquired conditions and drugs that influence enzyme activity, as well as possible treatments of the condition, are reviewed.
The review of the literature was conducted by searching PubMed and Ovid Medline databases, with no limitation on date of publication. The search was limited to English-language journals only. Additional articles of relevance were obtained from reference lists of previously searched articles and via Internet searches. Numerous keywords were used in the search, and a second search was undertaken to find specific citations about acquired conditions and drugs of relevance. Nearly 250 articles were obtained and examined for importance. Fifty articles appear in the review, including case reports, research studies, and review articles.
Keywords: Atypical pseudocholinesterase, BChE, butyrylcholinesterase, genetic variants, pseudocholinesterase.
Butyrylcholinesterase-also known as pseudocholinesterase, serum cholinesterase, plasma cholinesterase, and false cholinesterase-was so named due to its ability to hydrolyze buty - rylcholine faster than other esters.1 Pseudocholines ter ase is produced in the liver and found in most tissues, with the exception of red blood cells. The presence of pseudocholines ter ase in the body has been established for well over half a century. Although potential functions of this enzyme are debated to this day, its role in the metabolism of choline esters is universally recognized.
Definition of Pseudocholinesterase Deficiency
Pseudocholinesterase deficiency is a genetic or acquired alteration in the metabolism of choline esters such as succinylcholine, mivacurium, and ester-linked local anesthetics. The most described consequence of pseudocholinesterase deficiency in the literature is prolonged paralysis and apnea after administration of succinylcholine or mivacurium. The latter drug is no longer produced in the United States but is used elsewhere in the world. Less frequently described are adverse outcomes with the use of ester local anesthetics, particularly chloroprocaine.
Individuals can live their entire lives with pseudocholines ter ase deficiency and not experience any untoward health effects, and the presence of the genetic defect is not realized until one is exposed to succinylcholine or mivacurium. In fact, a pseudocholinester ase-deficient group of individuals in the Vysya community of India were studied, and the determination...