Protein C deficiency--response to danazol and DDAVP.
We studied a Spanish family in which one of the female members presented recurrent thrombophlebitis in both legs after three different deliveries. Biological and antigenic activity of protein C was decreased (35% and 42% respectively). Reduced protein C levels were also observed in 6 other family members. Administration of danazol (600 mg/day) in two patients with protein C deficiency elevated this protein and discontinuation of the drug resulted in a reduction of protein C to pretreatment values. The proposita showed a normal fibrinolytic activity and infusion of DDAVP produced a similar response of FVIII/VWF and plasminogen activator to those observed in healthy subjects.
D000990:Antithrombin III / Q000032:analysis; D003613:Danazol; D003894:Deamino Arginine Vasopressin; D005170:Factor X / Q000032:analysis; D005260:Female; D005342:Fibrinolysis; D006023:Glycoproteins / Q000032:analysis / Q000172:deficiency* / Q000235:genetics; D006801:Humans; D008297:Male; D010375:Pedigree; D011245:Pregnadienes; D011486:Protein C; D011516:Prothrombin / Q000032:analysis; D012016:Reference Values; D013924:Thrombophlebitis / Q000097:blood* / Q000235:genetics
D006023:Glycoproteins; D011245:Pregnadienes; D011486:Protein C; D000990:Antithrombin III; D011516:Prothrombin; D005170:Factor X; D003894:Deamino Arginine Vasopressin; D003613:Danazol