DISEASE VON WILLEBRAND

Von Willebrand disease (angiohemophilia) - a hereditary disease (I type - SR, II, and III types -
p), a syndrome characterized by bleeding mixed (sinyachkovo-gematomnogo) type.

Etiology and pathogenesis.'s Disease von Willebrand sick persons of both sexes. The disease is
caused by violation of the synthesis of plasma-protein complex of blood coagulation factor VIII
(von Willebrand factor) that leads to the pathology of platelet aggregation. There are several
types of von Willebrand disease, the most difficult type III flows. Von Willebrand factor is
synthesized in endothelial cells and megakariotsi the field and performs a dual function: involved
in the cascade of blood clotting, causing the stability of factor VIII, and plays an important role
in vascular-platelet hemostasis, providing the adhesion of platelets to subendote-lialnym
structures damaged vessel and contributing to the aggregation platelets.

Clinical picture. The first manifestations of the disease in patients with severe to occur in
children during the first year of life. Spontaneously bleeding from the mucous membranes of the
nose, mouth and water services are possible in puberty menorrhagia. There are often
subcutaneous hemorrhage in the form of ecchymosis, petechiae, and sometimes - deep
hematoma, hemarthrosis, which makes this disease is similar to hemophilia, and distinguishes it
from trombastenii Glyantsmanna and thrombocytopenic purpura. Such operations, as tonzil-
lektomiya and extraction of teeth, accompanied by profuse bleeding, which can lead to the
development of acute posthemorrhagic anemia. Unlike hemophilia, von Willebrand disease in
post-operative bleeding or injury begins immediately, not after some time, and after stopping the
bleeding is not repeated.

Diagnosis. Diagnostic criteria for von Willebrand's disease: family history, a mixed type of
bleeding, increased bleeding time, decrease the concentration of factor VIII and von Willebrand
factor in blood clotting, reduced platelet aggregation and adhesiveness (retention) under the
influence ristotsitina (ristomitsina).

Treatment. In the treatment of von Willebrand disease using the same drugs as the treatment of
hemophilia. Preference to cryoprecipitate, fresh frozen plasma. Highly purified preparations of
blood coagulation factor VIII ineffective because it does not contain von Willebrand factor. You
can use a synthetic analogue of ADH - desmopressin. If you have bleeding (except kidney!) Use
aminokaprono-vuyu acid. When menorrhagia shown mestranol.