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  1. Ehlers-Danlos Syndrome Type VI (EDS VI) : problems of diagnosis and management.

    Article - En anglais

    Ehlers-Danlos Syndrome Type VI (EDS VI) is a rare autosomal recessively inherited connective tissue disorder, which poses several problems of diagnosis and management.

    We report on a patient who developed severe kyphoscoliosis long before the diagnosis was reached.

    We conclude that early biochemical diagnosis and a timely operative procedure by extensive posterior instrumentation is the basis for successful management of this disorder.

    Mots-clés Pascal : Ehlers Danlos syndrome, Pathogénie, Complication, Cyphoscoliose, Dépistage, Diagnostic, Traitement, Etude cas, Enfant, Homme, Rachis dorsal, Peau pathologie, Tissu élastique pathologie, Système ostéoarticulaire pathologie, Maladie système, Maladie héréditaire, Rachis pathologie, Déformation

    Mots-clés Pascal anglais : Ehlers Danlos syndrome, Pathogenesis, Complication, Kyphoscoliosis, Medical screening, Diagnosis, Treatment, Case study, Child, Human, Dorsal spine, Skin disease, Elastic tissue disease, Diseases of the osteoarticular system, Systemic disease, Genetic disease, Spine disease, Deformation

    Logo du centre Notice produite par :
    Inist-CNRS - Institut de l'Information Scientifique et Technique

    Cote : 98-0344387

    Code Inist : 002B07. Création : 27/11/1998.