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pseudocholinesterase deficiency; butyrylcholinesterase deficiency
Pathology:
- delayed metabolism of:
- succinylcholine, mivacurium, procaine, & cocaine
Epidemiology:
- most common in people of European descent
- rare in Asians
Clinical manifestations:
- recognized most often when respiratory paralysis unexpectedly persists for a prolonged period of time following administration of standard doses of succinylcholine
Laboratory:
- pseudocholinesterase in serum/plasma
- dibucaine number
- clinically significant effects generally are not observed until the plasma cholinesterase activity is reduced to < 75% of normal
Management:
- ventilatory support until diffusion of succinylcholine from the myoneural junction permits return of neuromuscular function of skeletal muscle
- counseling: avoid cocaine
Related
pseudocholinesterase (cholinesterase-2, acylcholine acylhydrolase, butyrylcholinesterase, BuChE, BCHE)
General
inborn error of metabolism
References
- Alexander DR
Pseudocholinesterase Deficiency
http://emedicine.medscape.com/article/247019-overview