pseudocholinesterase deficiency

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pseu·do·cho·lin·es·ter·ase de·fi·cien·cy

[MIM*177400]

an autosomal dominant disorder manifested by exaggerated responses to drugs ordinarily hydrolyzed by serum pseudocholinesterase (for example, succinylcholine); believed to entail production of a variant enzyme that is less active than the normal enzyme in hydrolyzing appropriate substrates, but also abnormally resistant to the effects of anticholinesterases, caused by mutation in the pseudocholinesterase E1 gene (CHE1) on 3q.

pseu·do·cho·lin·es·ter·ase de·fi·cien·cy

(sūdō-kōlin-estĕr-ās dĕ-fishĕn-sē) [MIM*177400]

Disorder with exaggerated responses to drugs ordinarily hydrolyzed by serum pseudocholinesterase.

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References in periodicals archive ?

A diagnosis of atypical pseudocholinesterase or pseudocholinesterase deficiency was suspected at this time.

(2) If any of these milder variants are combined with an acquired quantitative pseudocholinesterase deficiency such as has been shown in pregnancy, an additive effect resulting in a significantly prolonged response to succinylcholine would be expected.

Prolonged paralysis following emergent cesarean section with succinylcholine despite normal dibucaine number

Rocuronium antagonized by sugammadex for series of electroconvulsive therapy in a patient with pseudocholinesterase deficiency. J ECT 2011; 27:e47-e48.

The selective combined use of rocuronium and sugammadex would be particularly advantageous in replacing multiple boluses of succinylcholine

Complete pseudocholinesterase deficiency: genetic and immunologic characterization.

A patient with prolonged paralysis

Use with caution in patients with severe hepatic disease, pseudocholinesterase deficiency, or a history of sensitivity to amide-type anesthetics.

Zingo, Risperdal

A 17-year-old girl with a history of von Willebrand' s disease and pseudocholinesterase deficiency was referred to our department with a 1-year history of a progressively enlarging tongue mass.

Intralingual dermoid cysts: A report of two new cases

Batistaki et al recently reported a patient with another contraindication to succinylcholine, pseudocholinesterase deficiency, where rocuronium (0.4 mg/kg) and sugammadex (2 mg/kg) were used to avoid prolonged apnoea during this patient's ECT (4).

Rocuronium antagonized by sugammadex for series of electroconvulsive therapy (ECT) in a patient with pseudocholinesterase deficiency. J ECT 2011; 27:e47-48.

With the advent of sugammadex, a succinylcholine-free anaesthetic in electroconvulsive therapy may be the future standard