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Eur J Clin Invest 36: 850-859

Learning objectives Describe Ehlers Danlos syndrome Recognize the symptoms and signs of Ehlers Danlos syndrome Anesthetic management of a patient with Ehlers Danlos syndrome Definition and mechanisms Ehlers Danlos syndrome (EDS) comprises a group of clinically and genetically heterogeneous heritable connective tissue disorders, characterized by joint hypermobility and instability, skin texture anomalies, and vascular and internal organ fragility Clinical manifestations range from extremely mild phenotypes to life-threatening complications depending on the specific subtype The current Villefranche nosology recognizes six major subtypes, comprising classic, hypermobile, vascular, kyphoscoliotic, arthrochalasia, and dermatosparaxis, most of which are linked to mutations in one of the genes encoding for fibrillar collagen proteins or enzymes involved in post-translational modification of these proteins AD, autosomal dominant

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