Serum Creatine Kinase Level as a Screening Test for Susceptibility to Malignant Hyperthermia

Abstract

One hundred thirty-one patients who were referred for muscle biopsy for in vitro contracture testing (IVCT) for susceptibility to malignant hyperthermia (MH) were studied. Serum creatine kinase (CK) levels were determined routinely before biopsy by the hospital clinical laboratories. Thirty-four had abnormal IVCTs (indicating susceptibility to MH) and 87 patients had normal IVCTs; all these 121 patients had normal CK levels. Ten additional patients had other muscle disorders (central core disease, hyperkalemic periodic paralysis, and Duchenne's muscular dystrophy) and abnormal IVCTs; six of the ten had elevated CK levels. We conclude that the serum CK level, as determined routinely, does not identify MH-susceptible individuals, but may indicate the presence of muscle disease. Therefore, the use of serum CK level as a screening or diagnostic test for susceptibility to MH should be abandoned.

Screening test for malignant hyperthermia in patients with persistent hyperCKemia: A pilot study

“…Conversely, however, in a group of patients with CK values only slightly above normal, only 1 MHS and 1 MHE individual (contracture after either caffeine or halothane) could be identified among the 37 patients examined . In summary, the predictive value of serum CK for a positive or negative result in diagnosis of MH susceptibility is generally low . However, for safety reasons, patients with persistently elevated CK levels without clinically obvious myopathy need special attention prior to anesthesia.…”

Evaluation of Malignant Hyperthermia Features in Patients with Pathogenic or Likely Pathogenic RYR1 Variants Disclosed through a Population Genomic Screening Program

“…Additionally, nearly half of the patients who had a recorded creatine kinase level had a maximum measurement over the reference range. As MH susceptibility has been associated with persistently elevated creatine kinase levels, [11][12][13] this finding raises the possibility that unselected individuals with a pathogenic or likely pathogenic RYR1 variant can still experience metabolic consequences of the variant even if they do not have a fulminant MH episode when exposed to triggering agents. Longitudinal evaluation, including in those who have not previously had creatine kinase measured, would be needed to assess this.…”

“…4 Guidelines for perioperative management of MH-susceptible patients intend to minimize morbidity and mortality. 5,6 Independent of triggering agent exposure, findings in MH-susceptible individuals continue to be studied, including exertional heat illness, 3,7 rhabdomyolysis, 8 neuromuscular symptoms, 9,10 chronically elevated serum creatine kinase, [11][12][13] and statin-associated muscle symptoms. 14 RYR1 and CACNA1S are actionable MH-associated genes on the American College of Medical Genetics and Genomics (Bethesda, Maryland) secondary findings list 15 and some commercially available genomic screening panels.…”

Malignant hyperthermia – what do we know in 2019?

“…No non-standard laboratory tests are needed prior to or after the anaesthetic procedure in MH susceptible patients [22]. It is not recommended to determine CK before anaesthesia to assess the risk of MHE or MH susceptibility [1,25]. An elevated serum level of CK may evidence muscle pathology yet has no connection with MH susceptibility [30].…”