A new form of Ehlers-Danlos syndrome: fibronectin corrects defective platelet function
MA Arneson, DE Hammerschmidt, LT Furcht, RA King - Jama, 1980 - jamanetwork.com
In a kindred with a mild, recessively inherited variant of the EhlersDanlos syndrome (EDS), a
platelet aggregation defect segregated concordantly with skin and joint abnormalities. This
defect was partially corrected in vitro by addition of normal plasma or cryoprecipitate. The
plasma of the patients with EDS failed to support the aggregation of normal gel-filtered
platelets in response to collagen; this defect was completely corrected by the addition of
normal human fibronectin. Since fibronectin is an important adhesive glycoprotein in …
platelet aggregation defect segregated concordantly with skin and joint abnormalities. This
defect was partially corrected in vitro by addition of normal plasma or cryoprecipitate. The
plasma of the patients with EDS failed to support the aggregation of normal gel-filtered
platelets in response to collagen; this defect was completely corrected by the addition of
normal human fibronectin. Since fibronectin is an important adhesive glycoprotein in …