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, Contexte : La mucoviscidose est une maladie autosomique récessive létale dont l'incidence et la prévalence sont élevées à La Réunion
, Objectifs : Décrire la morbi-mortalité des patients réunionnais atteints de mucoviscidose
Etude de cohorte observationnelle rétrospective, à partir des données du Registre Français de la Mucoviscidose sur une période de 12 ans, 2006. ,
, La proportion d'adultes a augmenté pendant l'étude mais représentait seulement 37% des patients en 2017, Résultats : 157 patients ont été inclus (87 hommes
, Seconde (VEMS) : durée de suivi (p<0,001), année de naissance (p<0,05), VEMS initial (p<0,001), génotype F508del/Y122X (p<0,05), colonisation chronique à Pseudomonas aeruginosa (p<0,001), bactériologie des crachats positive à mycobactérie atypique (p<0,05)
RGO) étaient les principales complications chroniques émergentes, avec 42% d'adultes diabétiques (22/53) et une prévalence du RGO de 49% (67/138) en 2017. Seize patients (10%) sont décédés au cours de l'étude, l'atteinte cardiorespiratoire représentait 56% des décès. La mortalité a baissé de 18 à 2,4/1000 pendant l'étude ,
, Discussion : La mortalité a diminué au cours de la dernière décennie chez les patients réunionnais atteints de mucoviscidose. Cependant, la survie restait inférieure à la métropole, même si la différence était moins marquée chez les patients les plus jeunes