(精选)【医学课件】止血、凝血机制教学课件.pptVIP

止血、凝血机制;正常止血机制;Normal hemostasis（止血）;;;+;Platelets adhere to VWF bound to sub-endothelium collagen;The platelet is activated and fibrinogen and other adhesive proteins engage with GpIIb/IIIa ;+;Thrombin binds to GpIba, cleaves PAR and GpV (not shown) and further activates platelets;;Thrombin bound to GpIba activates FXI, leading to further thrombin generation ;+;Thrombin proteolytically inactivates ADAMTS13, preventing destabilisation of the platelet plug ;+;The haemostatic plug expands in an unregulated manner;Cofactor competition between TM and fibrin abruptly halts plug expansion;+; ;VWF前体的结构;◆ VWF主要是由内皮细胞和巨核细胞合成，血浆中的VWF 主要来源于内皮细胞； ◆ VWF前体在分泌前经历一系列化学修饰，如内质网中 proVWF二聚体化、N-糖基化；高尔基体中O-糖基化、 N-侧链碳水化，蛋白水解、 proVWF二聚体多聚体化及 vWF多聚体形成等； ◆ 成熟的VWF多聚体或者直接分泌入血浆或储存在内皮细 胞Weibel-Palade小体或血小板α颗粒中； ◆ 分泌到血浆中的VWF多聚体或被肝脏清除或在足够剪切 力时被vWF切割酶ADAMTS13在VWFA2区Y1605-M1606处切 割； ;VWF的生理作用;血浆VWF水平的影响因素;◆ 分为1型、2型（2A、2B、2N、2M）、3型； ◆ 多为常染色体显性遗传，3型和2N型为常染色体隐形遗传； ◆ 皮肤紫癜、粘膜出血，特别是牙龈和鼻出血最常见，女性患 者表现为月经增多； ◆ 发病机制： VWF质或量的异常；;血栓性血小板减少性紫癜(TTP); ADAMTS-13 consist of a signal peptide (S), a short propeptide (P), a metalloprotease domain, a disintegrin-like domain (Dis), 8 thrombospondin-1 repeats (numbered 1–8), a Cys-rich (Cys) and spacer domain ， two CUB domains and metalloprotease domain (M). The M domain cleaves the Tyr1605–Met1606 bond within VWF domain A2; the Cys-rich and spacer domains interact with additional features of VWF domain A2.;TTP发病机制模式图;?;出血;Deficiency of factor VIII or IX results in recurrent joint and soft tissue bleeding.;carrier;SPANISH ROYALTY;