Purpose: In 1992, 1812 individuals (I.2% of the population) were labelled at risk for malignant hyperthermia (MH) ~n seven families from Abitibi-Temiscamingue, To evaluate the effective risk in this population, a multidisciplinary study was undertaken which included clinical, genealogical and molecular aspects. This paper presents the clinical aspects of the study. Method: For each of the 1546 individuals reached, all anaesthetic exposures were screened for elements relevant to MH. Malignant hyperthermia events were analyzed with "the clinical grading scale." All 44 reports of caffeine halothane contracture tests were reappraised, Finally, a genealogical study was done to complete each family tree up to the initial French settlers in order to identify links between these seven families through common ancestors. Results: Following this reassessment, the families were compared and classified into four groups. Two families (1097 individuals) are not considered to be at a higher risk for MH than the population in general. Two families are styli considered possibly at risk. finally, one family (402 individuals) is highly at risk and two other families are probably at nsk. Family trees did not show any link up to the colonization of Abitibi-Temiscamingue in the beginning of this Century but common ancestors were found around the 9 th generation, Conclusion: Th~s chnical reassessment will help to focus education and prevention on a much smaller group of tndividuals still considered potentially at risk for MH, By adequate evaluation of phenotypes, combined with the use of a genealogical approach, it will be possible to target families for molecular research.Objectifs : En 1992, 1812 Jndividus (soit 1.2% de la population) 6taient etiquetes comme susceptibles fi I'hyperthermie maligne (HM) darts sept families de rAbitibi-Temiscamingue./kiln d'&aluer le risque reel de cette population, une etude multidisoplinaire rut initiee incluant les volets clinique, genealogique et moleculaire. Cet article presente les aspects cliniques de cette etude. M&hode : Pour chacun des 1546 individus rejoints, routes les procedures anesthesiques furent evaluees afin de retrouver des slgnes revelateurs d'HM, Les episodes d'HM furent examines ~. I'aide d'une 6chelle de gradation clinique. Les 44 rapports rews& de tests de contracture cafeine-halothane furent pris en consideration, Finalement, une etude genealogique de chaque ascendance, jusqu'aux premiers arnvants fran(jais, rut menee afin d'identifier les liens entre ces sept families & travers des anc&res communs, R~sultats : Suite ~ la rews~on clinique, les families furent comparees et classees en quatre groupes. Deux farnilles (I 097 individus) ne sont pas conslderees plus ~ risque que la population en general, Deux autres families sont encore consid&ees comme ayant un nsque possible, finalement, une famille (402 individus) est consid&ee hautement nsque et deux autres famtlles sont ctassees comme ayant un risque probable, rexamen des ascendances montre qu'on ne retrouve pas d'ancCtres commun...
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