title: Malignant hyperthermia, coexisting disorders, and enzymopathies: risks and management options.
abstract: Clinical episodes and abnormal laboratory tests compatible with a diagnosis of malignant hyperthermia have been observed in patients with a diversity of syndromes, enzymopathies, and coexisting disorders thereby raising the likelihood of causal associations and heightened perioperative risk in others carrying a shared diagnosis. In the present review, we survey available published series, case reports, and the results of contracture testing in patients identified by others to be potentially predisposed to malignant hyperthermia. For most conditions, evidence for a causal relationship with malignant hyperthermia susceptibility is weak. The review concludes with suggestions for clinical management when evidence for or against an association is uncertain.
authors.affiliation: Department of Anesthesiology, University of Wisconsin School of Medicine and Public Health, B6/319 Clinical Science Center, 600 Highland Ave., Madison, WI 53792-3272, USA. jfbenca@wisc.edu
authors.forename: Joan
authors.initials: J
authors.lastname: Benca
authors.forename: Kirk
authors.initials: K
authors.lastname: Hogan
mesh_terms: D000659:AMP Deaminase / Q000172:deficiency; D000758:Anesthesia / Q000009:adverse effects*; D001176:Arthrogryposis / Q000150:complications; D002334:Carnitine O-Palmitoyltransferase / Q000172:deficiency; D003402:Creatine Kinase / Q000097:blood; D019317:Evidence-Based Medicine; D006012:Glycogen Storage Disease Type V / Q000150:complications; D006801:Humans; D008305:Malignant Hyperthermia / Q000175:diagnosis / Q000209:etiology* / Q000517:prevention & control*; D008661:Metabolism, Inborn Errors / Q000150:complications* / Q000201:enzymology / Q000235:genetics; D009140:Musculoskeletal Diseases / Q000150:complications*; D009634:Noonan Syndrome / Q000150:complications; D010013:Osteogenesis Imperfecta / Q000150:complications; D018579:Patient Selection; D018570:Risk Assessment; D012307:Risk Factors; D053498:Sarcoplasmic Reticulum Calcium-Transporting ATPases / Q000235:genetics
chemical_list: D002334:Carnitine O-Palmitoyltransferase; D003402:Creatine Kinase; D000659:AMP Deaminase; D053498:Sarcoplasmic Reticulum Calcium-Transporting ATPases; C506943:ATP2A1 protein, human