Biomedical subjects
Publications and source records attributed to K Hasler.
31 patients with the diagnosis or presumed diagnosis of von Willebrand's disease were reinvestigated by means of Ristocetin cofactor-activity and factor VIII-associated antigen. In addition a family with a variant of von Willebrand's disease is described. Ristocetin cofactor-activity was found the most reliable test, its value for the diagnosis of mild forms and of variants of von Willebrand's disease is further established by these results.
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A family is described in which 5 out of 8 children had a marked bleeding disorder. The children showed prolonged bleeding times, abnormal platelet retention upon passage of blood through a glass bead column, the Willebrand factor activity as measured by ristocetin in a washed platelet system was low. Factor VIII/von Willebrand factor protein levels were normal even so the factor VIII-procoagulant activity. Even the parents and one child without any bleeding tendency and normal bleeding times had a reduced Willebrand factor activity. In all these patients evidence of an abnormal protein was observed on crossed antigen-antibody electrophoresis indicating a qualitative defect of the factor VIII/von Willebrand factor protein.
In bromocarbamide intoxication in humans, as in rabbits, the predominant feature is the endothelial damage characterized by desquamation and vacuolisation, followed by interstitial oedema. Consumption coagulopathy, as observed in some cases of human bromocarbamide intoxication and also in our experimental model, can be prevented by anti-coagulant therapy with heparin and also, as could be shown in rabbits, with aggregation-inhibiting agents. These findings strongly suggest that consumption coagulopathy is only a secondary phenomenon which develops in the course of primary endothelial damage. Similarities in the histological findings of endothelial damage between bromocarbamide intoxication in humans and in rabbits and the so-called "Adalin purpura", which can be observed after chronic use of bromocarbamide, are discussed.
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