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Ehlers-Danlos syndrome, fibronectinemic type

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.


  • 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, Adult
  • ICD-10: Q79.6
  • OMIM: 225310
  • UMLS: C1857038
  • MeSH: -
  • GARD: 8508
  • MedDRA: -

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