pseudocholinesterase deficiency; butyrylcholinesterase deficiency

Epidemiology

• most common in people of European descent
• rare in Asians

Pathology

• delayed metabolism of:
  • succinylcholine, mivacurium, procaine, & cocaine

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

More general terms

• inborn error of metabolism

Additional terms

• pseudocholinesterase (cholinesterase-2, acylcholine acylhydrolase, butyrylcholinesterase, BuChE, BCHE)