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Pseudocholinesterase Deficiency Treatment & Management

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

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

See the list below:

  • 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, 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
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  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].

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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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