Pseudocholinesterase Deficiency Follow-up

  • Author: Daniel R Alexander, MD; Chief Editor: Allen R Wyler, MD   more...
 
Updated: Jan 13, 2012
 

Complications

  • The main complication resulting from pseudocholinesterase deficiency is the possibility of respiratory failure secondary to succinylcholine or mivacurium-induced neuromuscular paralysis.
  • Individuals with pseudocholinesterase deficiency also may be at increased risk of toxic reactions, including sudden cardiac death, associated with recreational use of cocaine.
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Prognosis

  • Prognosis for recovery following administration of succinylcholine is excellent when medical support includes close monitoring and respiratory support measures.
  • In nonmedical settings in which subjects with pseudocholinesterase deficiency are exposed to cocaine, sudden cardiac death can occur.
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Patient Education

  • Patients with known pseudocholinesterase deficiency may wear a medic-alert bracelet that will notify healthcare workers of increased risk from administration of succinylcholine.
  • These patients also may notify others in their family who may be at risk for carrying one or more abnormal pseudocholinesterase gene alleles.
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Contributor Information and Disclosures
Author

Daniel R Alexander, MD  Consulting Staff, Departments of Internal Medicine and Pathology, Franklin Square Hospital Center

Daniel R Alexander, MD is a member of the following medical societies: American College of Physicians-American Society of Internal Medicine, American Medical Association, American Society for Clinical Pathology, College of American Pathologists, and MedChi

Disclosure: Nothing to disclose.

Specialty Editor Board

Scott C Dulebohn, MD  Neurological Surgeon, Appalachian Neurosurgical

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Allen R Wyler, MD  Former Medical Director, Northstar Neuroscience, Inc

Allen R Wyler, MD is a member of the following medical societies: American Academy of Neurological and Orthopaedic Surgeons, American Association of Neurological Surgeons, and Society of Neurological Surgeons

Disclosure: Nothing to disclose.

Paolo Zamboni, MD  Professor of Surgery, Chief of Day Surgery Unit, Chair of Vascular Diseases Center, University of Ferrara, Italy

Paolo Zamboni, MD is a member of the following medical societies: American Venous Forum and New York Academy of Sciences

Disclosure: Nothing to disclose.

Chief Editor

Allen R Wyler, MD  Former Medical Director, Northstar Neuroscience, Inc

Allen R Wyler, MD is a member of the following medical societies: American Academy of Neurological and Orthopaedic Surgeons, American Association of Neurological Surgeons, and Society of Neurological Surgeons

Disclosure: Nothing to disclose.

References

  1. Leadingham CL. A case of pseudocholinesterase deficiency in the PACU. J Perianesth Nurs. Aug 2007;22(4):265-71; quiz 272-4. [Medline].

  2. Williams J, Rosenquist P, Arias L, McCall WV. Pseudocholinesterase deficiency and electroconvulsive therapy. J ECT. Sep 2007;23(3):198-200. [Medline].

  3. Soliday FK, Conley YP, Henker R. Pseudocholinesterase deficiency: a comprehensive review of genetic, acquired, and drug influences. AANA J. Aug 2010;78(4):313-20. [Medline].

  4. Duysen EG, Li B, Carlson M, Li YF, Wieseler S, Hinrichs SH, et al. Increased hepatotoxicity and cardiac fibrosis in cocaine-treated butyrylcholinesterase knockout mice. Basic Clin Pharmacol Toxicol. Dec 2008;103(6):514-21. [Medline].

  5. Li B, Duysen EG, Carlson M, Lockridge O. The butyrylcholinesterase knockout mouse as a model for human butyrylcholinesterase deficiency. J Pharmacol Exp Ther. Mar 2008;324(3):1146-54. [Medline].

  6. Cerf C, Mesguish M, Gabriel I, et al. Screening patients with prolonged neuromuscular blockade after succinylcholine and mivacurium. Anesth Analg. Feb 2002;94(2):461-6, table of contents. [Medline].

  7. Dietz AA, Rubinstein HM, Lubrano T. Colorimetric determination of serum cholinesterase and its genetic variants by the propionylthiocholine-dithiobis(nitrobenzoic acid)procedure. Clin Chem. Nov 1973;19(11):1309-13. [Medline].

  8. Jatlow P, Barash PG, Van Dyke C. Cocaine and succinylcholine sensitivity: a new caution. Anesth Analg. May-Jun 1979;58(3):- Van Dyke C. [Medline].

  9. Jensen FS, Viby-Mogensen J. Plasma cholinesterase and abnormal reaction to succinylcholine: twenty years' experience with the Danish Cholinesterase Research Unit. Acta Anaesthesiol Scand. Feb 1995;39(2):150-6. [Medline].

  10. Kalow W, Genest K. A method for the detection of atypical forms of human serum cholinesterase; determination of dibucaine numbers. Can J Biochem Physiol. Jun 1957;35(6):339-46. [Medline].

  11. Lange D, du Pasquier Y. [Study of cellular immunity in the course of nephro-epithelioma. Preliminary study concerning 12 cases (author's transl)]. J Urol Nephrol (Paris). Jul-Aug 1975;81(7-8):543-8. [Medline].

  12. Lehmann H, Liddell J, M - 196907. Human cholinesterase (pseudocholinesterase): genetic variants and their recognition. Br J Anaesth. Mar 1969;41(3):235-44. [Medline].

  13. Lovely MJ, Patteson SK, Beuerlein FJ, Chesney JT. Perioperative blood transfusion may conceal atypical pseudocholinesterase. Anesth Analg. Mar 1990;70(3):326-7. [Medline].

  14. Maiorana A, Roach RB Jr. Heterozygous pseudocholinesterase deficiency: a case report and review of the literature. J Oral Maxillofac Surg. Jul 2003;61(7):845-7. [Medline].

  15. Pantuck EJ. Plasma cholinesterase: gene and variations. Anesth Analg. Aug 1993;77(2):380-6. [Medline].

 
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Noninvasive ventilation. A bilevel positive airway pressure (BIPAP) prototype is shown here. Expiratory positive airway pressure is the expiratory pressure setting that determines the amount of positive end-expiratory pressure that is applied. The inspiratory positive airway pressure setting is the pressure support. The device can be used in spontaneous mode or timed mode (with a mandatory backup respiratory frequency).
Table 1. Atypical Gene Alleles for the Pseudocholinesterase Genotype
EaAtypical dibucaine-resistant variantPoint mutation
EfFluoride-resistant variantPoint mutation
EsSilent variantFrameshift mutation
*These alleles may occur either in the homozygous form or in any heterozygous combination with each other, with the normal Eu allele, or with a number of additional rare variant abnormal alleles
Table 2. Reaction Times for Acholest Test Paper
Reaction TimePseudocholinesterase Enzyme Activity
< 5 minAbove normal
5-20 minNormal
20-30 minBorderline low
>30 minBelow normal
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