Preferred Label : Ehlers-Danlos syndrome, dysfibronectinaemic type;
ICD-11 definition : Ehlers-Danlos syndrome, fibronectinemic type is a type of Ehlers-Danlos syndromes
(EDS), a heterogeneous group of inherited connective tissue disorders characterized
by variable joint hypermobility and cutaneous hyperextensibilityand is distinguished
by platelet dysfunction associated with a fibronectin abnormality.;
ICD-11 synonym : Ehlers-Danlos syndrome with platelet dysfunction from fibronectin abnormality; Ehlers-Danlos syndrome, fibronectin-deficient; Ehlers-Danlos syndrome type 10;
Origin ID : 1014914201;
Automatic exact mappings (from CISMeF team)
Ehlers-Danlos syndrome, fibronectinemic type is a type of Ehlers-Danlos syndromes
(EDS), a heterogeneous group of inherited connective tissue disorders characterized
by variable joint hypermobility and cutaneous hyperextensibilityand is distinguished
by platelet dysfunction associated with a fibronectin abnormality.