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Ehlers-Danlos syndrome, fibronectinemic type

Synonym(s) EDS X
Ehlers-Danlos syndrome type 10
Ehlers-Danlos syndrome with platelet dysfunction from fibronectin abnormality
Ehlers-Danlos syndrome, fibronectin-deficient
Prevalence <1 / 1 000 000
Inheritance Autosomal recessive
Age of onset Adolescent
  • Q79.6
MeSH -
MedDRA -


Disease definition

Ehlers-Danlos syndromes (EDS) form a heterogeneous group of inherited connective tissue disorders characterized by variable joint hypermobility and cutaneous hyperextensibility. Type X is distinguished by platelet dysfunction associated with a fibronectin abnormality. Type X EDS has been described in only one family so far. Age of onset is about 13-25 years. Transmission is autosomal recessive.

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