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[Congenital pseudocholinesterase deficiency].

Remigiusz Gelo¹, Anna Jurek-Gelo, Ryszard Wardaszko

¹II Klinika Anestezjologii i Intensywnej Terapii, Warszawski Uniwersytet Medyczny.

Anestezjologia Intensywna Terapia

|July 27, 2011

Summary

Congenital pseudocholinesterase (pChe) deficiency can cause prolonged muscle relaxation after mivacurium use. Prompt measurement of pChe activity is crucial for diagnosing this rare genetic condition.

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Area of Science:

Anesthesiology
Pharmacogenetics
Neuromuscular blockade

Background:

Congenital pseudocholinesterase (pChe) deficiency is a rare genetic disorder.
It affects the metabolism of certain muscle relaxants, including mivacurium.
This deficiency can lead to unexpectedly prolonged neuromuscular blockade.

Observation:

Two male patients (26 and 7 years old) experienced prolonged neuromuscular block after receiving mivacurium during surgery.
Neuromuscular transmission recovery took 7 and 4 hours, respectively, with no response to standard reversal agents.
Postoperative assays confirmed severe pChe deficiency in both patients.

Findings:

Severe pChe deficiency was identified in both patients, with enzyme activity significantly below normal ranges.
Family screening confirmed the genetic basis of the deficiency in both cases.
Mivacurium hydrolysis was impaired due to low pseudocholinesterase levels.

Implications:

Consider pChe deficiency in cases of prolonged muscle relaxation post-mivacurium.
Measuring pChe activity is essential for diagnosis.
Mandatory family screening is recommended upon confirmation of pChe deficiency.