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Ehlers–Danlos syndrome and related disorders 

Ehlers–Danlos syndrome and related disorders
Ehlers–Danlos syndrome and related disorders

Roger Smith

and Paul Wordsworth

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date: 20 September 2020

Ehlers–Danlos syndrome (EDS) is a group of disorders with soft hyperextensible skin and excessive joint mobility largely due to mutations in the genes for the fibrillin collagens. This chapter deals with the diagnosis, causes and management. The skeleton is mainly affected in EDS6 and 7. New mutations on non-collagen genes (TNXB, B4GALT7) are described. Vascular EDS is difficult to treat.

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