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PCHES<br />

8518<br />

(0%-20%) and usually low levels of enzyme. Heterozygous patients have intermediate PCHE levels and<br />

response to inhibitors. The atypical gene is inherited in an autosomal recessive pattern. In a positive<br />

patient, family members should be tested. Several reports have shown that 65% to 75% of patients who<br />

respond abnormally to succinylcholine had at least 1 abnormal gene, had low activity due to an acquired<br />

deficiency such as liver disease, or had received an inappropriate dose of drug. The remaining 25% to<br />

35% of patients appeared to have the usual or normal genotype but nevertheless displayed long periods of<br />

apnea. Although reasons could not be established, it is possible that these cases represent unknown<br />

genotypes. Therefore, although many symptomatic patients will show moderate to significant resistance to<br />

dibucaine and low enzyme activity, not all will. In all cases, it is recommended that succinylcholine and<br />

mivacurium be avoided, or the dose greatly reduced.<br />

Reference Values:<br />

DIBUCAINE INHIBITION<br />

70-90%<br />

Congenital deficiency: 18-20%<br />

PSEUDOCHOLINESTERASE, TOTAL<br />

Males: 3,100-6,500 U/L<br />

Females<br />

18-49 years: 1,800-6,600 U/L<br />

> or =50 years: 2,550-6,800 U/L<br />

Reference values have not been established for patients that are

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