All patients reported and external bleeding from the mucous membranes and internal different localization frequently vnutricerepnae bruises. Inherited deficit protrombina also described as a mixed deficit of 7, 9, 10, and protein C and S. There are cases of congenital desprotrombinemii where pronounced tendency to bleeding with protrombina level in the blood. Factor VII is converted to its active form by tkanevomu factor (TF), and set TF : VIIa is the initiator of the liquidation process. The F VII is a rare disease (1 case per 500,000 population), and its symptoms are varied. Patients with an activity factor of less than 1% suffer serious violations hypercoagulable similar manifestations of severe hemophilia A. In the factor VII more than 5% of patients had only minor bleeding episodes mucous membranes. There are reports about the possibility of trombozov against deficit F VII, as well as described socetanny deficit factors VII, IX and X. Factor X is a zimogen with molecular weight in 56 Kda consisting of two chains, which circulates freely in the plasma. His aguirovannaya form is the ferment of protrombinaza complex, which is a catalyst for the conversion process in protrombina trombin. Innate deficit this factor is rare and is transmitted to autosomno-retessivno the mechanism. Those deficits described in amiloidoze, acute respiratory infections and type leikozah mieloblastoza acute. The type and frequency of bleeding usually on the level of the deficit factor. The most frequent symptoms include gemartrozy, menstrual bleeding and bruises spontaneous, less commonly gematuria and central nervous system. The factor XI is a very different clinical picture, but bleeding usually develops only in connection with surgery or injury. For women, the disease is rarely profuznami menstrualnami bleeding. The father of autosomno-retessivno the and the most common among Ashkenazi Jews, where the frequency of pathology reaches 8%. Unlike classical hemophilia, the disease is no match between the factors in the blood and the severity of bleeding. Vazopatia Gemorragicaki vasculit. The most frequent disease Shenlein-genoha (haemorrhagic immune mikrotrombovskulit). The pathogenesis of the disease is Shenlein-genoha immunopatologicaki process. In fact, with this pathology it is a third type of allergic reactions - going Artusa. This disease is nasledstvenno-obuslovlennam, it is inherited by autosomno-rezesivne type. Meet with the same frequency for both boys and girls. Most episodes of the disease observed in the spring and autumn are the children of pre-school and school-age children. The defeat receptacles can be approached in a systematic fashion, but more often than not (in most cases), it is located in one of a system. Clinical manifestations : * cutaneous form is characterized by the appearance of predominantly razgibationah and lateral surfaces of the legs, buttocks, and are rarely found on the body haemorrhagic disease, the size of which prossianogo grain to 5 kopeecna coin. Makulezno-papuleznaya disappear. Sometimes rashes about everything. The elements combined with the swelling of the lower leg and feet external genitals, a century-type Квинке oedema. Haemorrhagic elements in the most severe cases, nekrotiziruyutza, which is one of the criteria for the seriousness of the course of the disease.
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