七年制出血性疾病课件-PPT文档.ppt
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1、Classification of hemorrhagic diseases 1. Abnormality of blood vessel 2. Abnormality of platelets 3 . Abnormality of coagulation,Mechanism of cougulation,Coagulation factors,FI,fibrinogen FII,prothrombin FIII,tissue factor,tissue thromboplastin FIV,Ca+ FV, labile factor FVII, stable factor FVIII, an
2、tihemophilic globulin, AHG FIX, plasma thromboplastin component,PTC, Christmas factor FX, Stuart-Prowe factor FXI, plasma thromboplastin anticedent,PTA FXII, Hageman Factor FXIII, fibrin stablizing factor PK HMWK,Coagulation cascade theory,The mechanism of anticoagulation and fibrinolysis,The system
3、 of anticoagulation *Antithrombin (AT) *Protein C system *Tissue factor pathway inhibitor (TFPI) *Heparin,Fibrinolysis system *Plasminogen (PLG) *t-PA *u-PA *Plasmin-related inhibitor,Coagulation=Anticoagulation, Laboratory examination for hemorrhagic diseases 1. Platelet count 2. Bleeding time(BT)
4、3. Clot retraction test 4. Capillary fragility test If above items are abnormal ,that means abnormality of blood vessel or platelet .,5. Clotting time(CT) 6. Plasma prothrombin time (PT) 7. Thrombin clotting time(TT) 8. Activation partial thromboplastin time(APTT) Kaolin partial thromboplastin time(
5、KPTT) If above items are abnormal, that means abnormality of coagulation.,PT FVII deficiency APTT hemophilia or FXI deficiency PT deficiency of FV, FX, APTT FII, or fibrinogen abnormalities.,Acquired deficiencies of plasma coagulation are more frequent than congenital disorder; the most common disor
6、ders include : -Hemorrhagic diathesis of liver disease; -Disseminated intravascular coagulation (DIC), -Vitamin K deficiency of more than one clotting factor.,treatment,Requires replacement of the deficient protein using recombinant or purified plasma derived products or fresh plasma.,Disseminated I
7、ntravascular Coagulation,DIC,Definition,The syndrome of DIC is a pathological state in the development of diseases. DIC is always secondary to another disorder. It never occurs as a primary disease.,Etiology,Infectious diseases: 43% *bacteria infection *virus *protozoon malaria,Etiology,Malignant tu
8、mor : 34%,Pathologic obsterics: 12% Operation and trauma: 5% Systemic disease,Pathogenesis,1.Damage of tissue release of tissue factor into blood activate extrinsic coagulation pathway 2.Damage of vascular epitheliaintrinsic coagulation system 3.Platelet activate 4.Activate fibrinolysindisturbance o
9、f coagulation and fibrinolysis,CoagulationAnticoagulation CoagulationAnticoagulation The disturbance of balance between coagulation and anticoagulation,Pathology and pathophysiology,Microthrombosis-the fundamental and specific change of pathology Abnormality of coagulation *hypercoagulable stage *co
10、nsumptive hypocoagulable stage *secondary hyperfibrinolytic stage Disturbance of microcirculation,Clinical manifestations,Bleeding tendency Shock or disturbance of microcirculation Embolism of microvasculature Microangiopathic hemolysis Manifestations of primary disease.,Clinical manifestations,Blee
11、ding tendency Shock or disturbance of microcirculation Embolism of microvasculature Microangiopathic hemolysis Manifestations of primary disease.,Clinical manifestations,Bleeding tendency Shock or disturbance of microcirculation Embolism of microvasculature Microangiopathic hemolysis Manifestations
12、of primary disease.,DIC,Clinical manifestations,Bleeding tendency Shock or disturbance of microcirculation Embolism of microvasculature Microangiopathic hemolysis Manifestations of primary disease.,Lab examination,1.platelet count 2.quantitative of plasma fibrinogen4g/L 3.3p test (+) or plasma FDP 2
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