Pseudocholinesterase Deficiency Considerations: A Case Study

Abstract

Pseudocholinesterase deficiency, sometimes called butyrylcholinesterase deficiency, is a rare disorder in which the neuromuscular blocking drugs succinylcholine and mivacurium cannot be metabolized properly in the blood plasma. This disorder can either be acquired as a result of certain comorbidities or it can be inherited genetically. Anesthesia providers must understand the pathophysiology of pseudocholinesterase deficiency and be prepared to safely and effectively manage patients who show signs and symptoms consistent with the disorder after the use of the indicated neuromuscular blocking drugs. This article summarizes the pharmacologic and physiologic data relevant to understanding the basic pathophysiology associated with pseudocholinesterase deficiency and illustrates a case study of a young woman suspected of having the disorder after a prolonged delay in emergence from general anesthesia.

Neuromuscular Blockade Monitoring: Having It but Knowing When Not to Trust It

“…opioids and benzodiazepines), hypoxemia, hypercarbia, hypoglycemia, and new neurologic deficits should be ruled out. Before considering BChe deficit, other causes for intraoperative prolonged NMB need to be accessed such as electrolytic changes (hypokalemia, hypocalcemia, and hypermagnesemia) and hypothermia [ 2 ].…”

“…Although the most common genetic variant is the K-variant with a frequency of about 20% in Caucasians, the atypical variant has the most clinical significance. The silent variants with no enzyme activity are very rare [ 2 ].…”

“…Acquired reduced BChE activity may also occur in patients with chronic liver disease, renal and heart failure, malnutrition, malignancies, severe burn injuries, or because of drug interactions (oral contraceptives, cyclophosphamide). A physiological decrease in BChe activity of 20% in the first trimester of pregnancy is maintained until delivery [ 1 , 2 ].…”

“…Before considering BChe deficit, other causes for intraoperative prolonged NMB need to be accessed such as electrolytic changes (hypokalemia, hypocalcemia, and hypermagnesemia) and hypothermia [2].…”

“…Congenital BchE deficiency is an autosomal recessive trait that affects approximately one in 2000 to 5000 individuals. Several genetic variations have been identified with different diagnostic and clinical effects [ 2 ].…”