淀粉样变性课件.pptxVIP

淀粉样变性 amyloidosis;淀粉样变性amyloidosis;淀粉样物质的形成;淀粉样物质构成;;; 淀粉样变性分型;继发性肾淀粉样变病因;鉴别 原发继发;AL型淀粉样变性病是异常的轻链蛋白和少数重链蛋白沉积造成的，部分病例可发现多发性骨髓瘤等单克隆免疫球蛋白异常沉积。 血清淀粉样相关蛋白降解产物组成的淀粉样蛋白A型(AA型) (见于继发性淀粉样变和部分家族性淀粉样变) ;肉眼：粉红或灰白石蜡样增加肾脏体积的慢性肾脏疾病皮质有灰白色淀粉样沉积物;普通光镜：心肌纤维之间有不定形的浅红色沉淀物 淀粉样蛋白的特征;刚果红染色:淀粉样物质经染色后应为橙红色，偏振光下，淀粉样物质为“苹果绿”双折射;;病理机制;肾淀粉样变;;心脏淀粉样变性(Cardiac Amyloidosis);ATTR型;SSA型;;病生理;临床表现;;心脏淀粉样变性确定诊断的依据;心脏淀粉样变性治疗;;;Electrocardiography Transthoracic echocardiography Cardiac magnetic resonance imaging cMRI）better characterizes myocardial involvement, functional abnormalities and amyloid deposition due to its high spatial resolution. Nuclear imaging has a role in the diagnosis of SSA Cardiac biomarkers ;;Light-chain amyloidosis　AL ;diagnosis;Diagnostic criteria for cardiac involvement;current staging systemthe Mayo Clinic staging;Another biomarker — midregional proadrenomedullin（肾上腺髓质前体素中段）;Electric disturbances(90 %） ;Holter;Echocardiographical findings;?right atrial enlargement and dilated vena cave reflecting　right ventricular (RV) filling pressure; ?increased interatrial septal thickness ?increased RV free wall thickness ( 7 mm) with RV systolicand diastolic dysfunctions associated with worse survival ?left and right valve thickening, usually responsible for　mild regurgitation; ?reduced aortic ejection time ( 273 ms） ?small pericardial effusion in 50% of cases atrial thrombi 　 AL patients were younger and had less atrial fibrillation than those with other types of amyloidosis; however,　 the AL group had significantly more intracardiac thrombi　and more fatal embolic events;Diagnostic and prognostic value of newechocardiographical techniques;Cardiac magnetic resonance imaging　cMRI;;Nuclear imaging;Cardiac complications ;Management and treatment of LA;;Prognosis ;Cardiac supportive treatment;Hereditary transthyretin-related　amyloidosis　ATTR;;The diagnosis strategy for ATTR combines pathology and molecular genetic testing. Cardiac medical supportive treatment