病理学局循障碍2013秋五年制3-1.ppt

* Esophageal varices resulting from portal hypertension caused by cirrhosis of the liver. With high pressure, varices ruptures readily. * Seen here is the anus and perianal region with prominent prolapsed true hemorrhoids. Chronic constipation, chronic diarrhea, pregnancy, and portal hypertension enhance hemorrhoid formation. Hemorrhoids can itch and bleed slowly(usually bright red blood, during defaecation). * 骨骼肌肉系统：主要有四肢细长，蜘蛛指（趾），双臂平伸指距大于身长，双手下垂过膝，下半身比上半身长。长头畸形、面窄、高腭弓、耳大且低位。皮下脂肪少，肌肉不发达，胸、腹、臂皮肤皱纹。肌张力低，呈无力型体质。韧带、肌腱及关节囊伸长、松弛，关节过度伸展。有时见漏斗胸、鸡胸、脊柱后凸、脊柱侧凸、脊椎裂等。  　　2.眼：主要有晶体状脱位或半脱位、高度近视、白内障、视网膜剥离、虹膜震颤等。男性多于女性。  　　3.心血管系统：约80%的患者伴有先天性心血管畸形。常见主动脉进行性扩张、主动脉瓣关闭不全，由于主动脉中层囊样坏死而引起的主动脉窦瘤、夹层动脉瘤及破裂。二尖瓣脱垂、二尖瓣关闭不全亦属本征重要表现。可合并先天性房间隔缺损、室间隔缺损、法乐氏四联症、动脉导管未闭、主动脉缩窄等。也可合并各种心律失常如传导阻滞、预激综合症、房颤、房扑等。  * Aortic dissection * The coagulation cascade. Note the common link between the intrinsic and extrinsic pathways at the level of factor IX activation. Factors in red boxes represent inactive molecules; activated factors are indicated with a lower case a and a green box. PL, phospholipid surface; HMWK, high-molecular-weight kininogen. Not shown are the anticoagulant inhibitory pathways. * Schematic illustration of some of the pro- and anticoagulant activities of endothelial cells. Not shown are the pro- and antifibrinolytic properties. vWF, von Willebrand factor; PGI2, prostacyclin; NO, nitric oxide; t-PA, tissue plasminogen activator. Thrombin receptor is referred to as protease activated receptor (PAR; see text). * Platelet adhesion and aggregation. von Willebrand factor functions as an adhesion bridge between subendothelial collagen and the GpIb platelet receptor complex (the functional complex is composed of GpIb in association with factors V and IX). Aggregation involves linking platelets via fibrinogen bridges bound to the platelet GpIIb-IIIa receptors. Marfan’s syndrome -- myxomatous degeneration of aorta wall 血管壁的完整性和/或通透性的改变 出血 hemorrhage 水肿 edema局部循环血量的异常