Pseudocholinesterase Deficiency Treatment & Management

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

Medical Care

  • Pseudocholinesterase deficiency is a clinically silent condition in individuals who are not exposed to exogenous sources of choline esters.
  • Patients with prolonged paralysis following administration of succinylcholine can be treated in the following ways:
    • Prophylactic transfusion of fresh frozen plasma can augment the patient's endogenous plasma pseudocholinesterase activity. This practice is not recommended because of the risk of iatrogenic viral infectious complications. However, perioperative transfusion of fresh frozen plasma administered to correct a coagulopathy may mask an underlying pseudocholinesterase deficiency.
    • Mechanical ventilatory support is the mainstay of treatment until respiratory muscle paralysis spontaneously resolves. Recovery eventually occurs as a result of passive diffusion of succinylcholine away from the neuromuscular junction.
    • Administration of cholinesterase inhibitors, such as neostigmine, is controversial for reversing succinylcholine-related apnea in patients who are pseudocholinesterase deficient. The effects may be transient, possibly followed by intensified neuromuscular blockade.
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Consultations

  • Consultation with a geneticist may help to identify the specific atypical genotype alleles contributing to pseudocholinesterase deficiency.
  • Because the DNA sequence of the pseudocholinesterase gene and its amino acid structure is known, atypical alleles now can be identified by PCR amplification studies using DNA extracted from leukocytes in a blood sample.
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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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