title: [Registration of peranesthetic cases of malignant hyperthermia in France. An update].
abstract: Sixty-two suspected crises of anaesthetic malignant hyperthermia (MH) were collected between 1969 and 1988 by a retrospective inquiry which lasted four years. 33 patients (53%) died whilst 29 survived. 20 cases were confirmed to be MH, either directly or indirectly by way of muscle biopsy and halothane and caffeine contracture tests carried out according to the European MH group protocol by two laboratories. This group included 11 of the deaths, one family member of whom, at least, is sensitive (MHS), 7 MHS survivors and 2 survivors too young to undergo muscle biopsy but belonging to MHS families. 21 cases were highly suspect of MH: 15 of the deaths which occurred in a typical way, and 6 patients of three different families who have suffered from anaesthetic deaths which, clinically, suggested MH. Another 15 were possible MH cases, all survivors, including one case of Steinert's disease and a brother of a case of central core disease. 2 cases were still being debated, because they had equivocal results for the caffeine test (MHEc); the last 4 had negative muscle biopsies and were excluded. 33 close relatives of the MH patients were diagnosed as MHS. 44 others were found to be free from the genetic predisposition. It was strongly recommended to yet 11 others that they carry the MHS card because they were MHEc. The clinical, surgical and anesthetic pictures were always as described in the literature. The anaesthetic protocols included inhalational agents in 90% of cases; these were combined with suxamethonium in 55% of cases. Dantrolene was only used in 32% of cases, and then at inadequate doses and very often too late; this probably explains the large number of treatment failures. The number of severe forms of MH was also very high in this series (70%). The need to increase the means of prevention and screening for MH in France is stressed.
authors.affiliation: CHRU de Grenoble, Département d'Anesthésie-Réanimation.
authors.forename: C
authors.initials: C
authors.lastname: Jacquot
authors.forename: P
authors.initials: P
authors.lastname: Stieglitz
authors.forename: G
authors.initials: G
authors.lastname: Kozak-Reiss
authors.forename: R
authors.initials: R
authors.lastname: Krivosic-Horber
authors.forename: M C
authors.initials: MC
authors.lastname: Laxenaire
authors.forename: A
authors.initials: A
authors.lastname: Lienhart
authors.forename: Y
authors.initials: Y
authors.lastname: Nivoche
mesh_terms: D000293:Adolescent; D000328:Adult; D000777:Anesthetics; D002648:Child; D002675:Child, Preschool; D003620:Dantrolene / Q000627:therapeutic use; D005260:Female; D005602:France; D006801:Humans; D007223:Infant; D007431:Intraoperative Complications / Q000453:epidemiology*; D008297:Male; D008305:Malignant Hyperthermia / Q000188:drug therapy / Q000453:epidemiology*; D008875:Middle Aged; D009135:Muscular Diseases / Q000209:etiology; D012042:Registries; D012189:Retrospective Studies; D013390:Succinylcholine; D011795:Surveys and Questionnaires
chemical_list: D000777:Anesthetics; D003620:Dantrolene; D013390:Succinylcholine
vernacular_title: Recensement des cas français d'hyperthermie maligne peranesthésique. Mise à jour.