Prise en charge et traitement de la neurofibromatose de type I
Résumé
La neurofibromatose de type I (NF1) est un trouble autosomique dominant associé à un syndrome suppresseur de tumeurs bénignes et malignes, affectant principalement la peau et le système nerveux. La NF1, le syndrome neurocutané le plus courant et qui fait l’objet de la présente revue, a une fréquence d’environ 1/1 900-1/3 500 personnes dans le monde entier. Les signes de la maladie peuvent apparaître dès la naissance et se manifester avec l’âge, ce qui a des conséquences négatives sur de nombreux domaines cliniques et des répercussions profondes sur la qualité et l’espérance de vie du patient.2 Compte tenu de sa nature évolutive et de sa variabilité clinique marquée, la NF1 justifie une approche pluridisciplinaire de la prise en charge et du traitement.
Références
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