Medical Care

Pseudocholinesterase deficiency is a clinically silent condition seen 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.

Consultation with a geneticist may help 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 polymerase chain reaction (PCR) amplification studies using DNA extracted from leukocytes in a blood sample.

Questions

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Media Gallery

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).

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Sections Pseudocholinesterase Deficiency
Overview
DDx
Workup
Treatment
Questions & Answers
Tables
References

Ea	Atypical dibucaine-resistant variant	Point mutation
Ef	Fluoride-resistant variant	Point mutation
Es	Silent variant	Frameshift 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.

Reaction Time	Pseudocholinesterase Enzyme Activity
< 5 min	Above normal
5-20 min	Normal
20-30 min	Borderline low
>30 min	Below normal

Pseudocholinesterase Deficiency Treatment & Management

Medical Care

References

Tables

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