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Pseudocholinesterase Deficiency Follow-up

  • Author: Daniel R Alexander, MD; Chief Editor: Brian H Kopell, MD  more...
 
Updated: Sep 17, 2015
 

Complications

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  • 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

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  • 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

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  • 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, MedChi The Maryland State Medical Society

Disclosure: Nothing to disclose.

Specialty Editor Board

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

Disclosure: Received salary from Medscape for employment. for: Medscape.

Chief Editor

Brian H Kopell, MD Associate Professor, Department of Neurosurgery, Icahn School of Medicine at Mount Sinai

Brian H Kopell, MD is a member of the following medical societies: Alpha Omega Alpha, American Association of Neurological Surgeons, International Parkinson and Movement Disorder Society, Congress of Neurological Surgeons, American Society for Stereotactic and Functional Neurosurgery, North American Neuromodulation Society

Disclosure: Received consulting fee from Medtronic for consulting; Received consulting fee from St Jude Neuromodulation for consulting; Received consulting fee from MRI Interventions for consulting.

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

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

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

  4. Brozović G, Mazul Sunko B, Hafner T, Bekavac I. Allergic reaction to suxamethonium during emergency caesarean section and pseudocholinesterase deficiency in the same patient. Wien Klin Wochenschr. 2014 Jul. 126 (13-14):435-8. [Medline].

  5. Zoller M, Walther S. [Residual relaxant block due to pseudocholinesterase deficiency - First manifestation in an elderly patient]. Anasthesiol Intensivmed Notfallmed Schmerzther. 2014 Jan. 49 (1):8-11. [Medline].

  6. Lurati AR. Organophosphate exposure with pseudocholinesterase deficiency. Workplace Health Saf. 2013 Jun. 61 (6):243-5. [Medline].

  7. Araoud M, Mhenni H, Hellara I, Hellara O, Neffati F, Douki W, et al. [Plasma cholinesterase activity in hepatic diseases]. Ann Biol Clin (Paris). 2013 May-Jun. 71 (3):293-8. [Medline].

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

  9. 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. 2008 Dec. 103(6):514-21. [Medline].

  10. Chaudhary SC, Singh K, Sawlani KK, Jain N, Vaish AK, Atam V, et al. Prognostic significance of estimation of pseudocholinesterase activity and role of pralidoxime therapy in organophosphorous poisoning. Toxicol Int. 2013 Sep. 20(3):214-7. [Medline]. [Full Text].

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

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

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

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

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

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

  17. 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). 1975 Jul-Aug. 81(7-8):543-8. [Medline].

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

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

  20. Pantuck EJ. Plasma cholinesterase: gene and variations. Anesth Analg. 1993 Aug. 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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