2022年医学专题—新英格兰双语病例讨论2(1).ppt

,Bilingual case discussion2015-09-11,第一页，共五十四页。,A 35-year-old man with dyspnea,anemia,and renal failure,第二页，共五十四页。,several weeks ago,the patient felt fatigue.Two days before admission,dyspnea developed,decreased urination,without fever,cough,or chest pain,no hemoptysis咯血(k xi),chills,night sweats,headaches,visual loss,dry eyes,dry mouth,or joint pain.He was transferred by ambulance to the emergency department at this hospital,第三页，共五十四页。,Four years before admission,he had been evaluated because of back pain,fatigue,and a temperature of 38.6C.Urinalysis showed hematuria血尿(xu nio)and proteinuria；testing for rapid plasma reagin(RPR快速血浆反应素)was positive for antibodies to Treponema pallidum（梅毒螺旋体）.During the previous 6 months,the patient had had episodes of bilateral finger,ankle,and facial swelling,without pain or change in color.He had no history of recent travel,exposure to sick persons,blood transfusions,or previous surgery.He did not smoke,drink alcohol,or use illicit drugs.,第四页，共五十四页。,On examination,T 36.9C,BP 173/89 mm Hg,P 95b/m,R36 b/m,and S02 88%(ambient air).The skin and conjunctivae 结膜(jim)were pale,and there were hypopigmented macules 色素减退斑on the right temple and both lower cheeks and hyperpigmented macules 色素沉着斑on the bridge of the nose.There were bibasilar rales双肺底湿罗音 in the lungs,and the remainder of the examination was normal.,第五页，共五十四页。,第六页，共五十四页。,第七页，共五十四页。,第八页，共五十四页。,total and direct bilirubin胆红素,liver-function tests and lactate were normal.testing for rheumatoid factor,screening of the blood and urine for toxins were negative;Testing for hepatitis B and C viruses and autoantibodies against histones组蛋白 was negative.Serum protein electrophoresis 电泳(din yn)revealed a diffuse increase in the IgG level.,第九页，共五十四页。,Transthoracic cardiac ultrasonography revealed normal global cardiac function and right-ventricular size,no evidence of a pericardial effusion心包(xnbo)积液,and findings that were consistent with pulmonary Edema.Ultrasonography of the abdomen revealed normal renal size,position,and echotexture回声特性 and normal arterial blood flow.An electrocardiogram(ECG)showed sinus tachycardia,counterclockwise rotation逆钟向,and nonspecific ST-segment and T-wave abnormalities.,第十页，共五十四页。,on admission,第十一页，共五十四页。,第十二页，共五十四页。,ground-glass opacities GGO,第十三页，共五十四页。,第十四页，共五十四页。,Bronchoscopic examination revealed thick,red mucus in the main-stem and right-lower-lobe bronchi;airways of the left lung were normal.Bronchoalveolar lavage on the right,with 300 and 24,500 red cells per cubic millimeter(in the first tube),975 and 1475 white cells per cubic millimeter(in fourth tubes)In the fourth tube,the white-cell differential count revealed 84%leukocytes白细胞.On the second day,the sputum culture grew very few klebsiella克雷伯,第十五页，共五十四页。,what do you think,第十六页，共五十四页。,Pulmonary hemorrhage？,第十七页，共五十四页。,PCP 卡氏肺孢子？（梅毒(mid)阳性、HIV）pulmonary embolism 肺栓塞？,第十八页，共五十四页。,Rapidly progressive glomerulonephritis Pulmonary hemorrhage,第十九页，共五十四页。,Ernest W.Goodpasture reported in 1919 on the autopsy findings in the case of an 18-year-old man who had died of massive lung hemorrhage and crescentic glomerulonephritis during the height of the influenza pandemic The term Goodpastures syndrome is applied to the combination of lung purpura and nephritis,regardless of the underlying pathogenesis.,第二十页，共五十四页。,(一)anti-GBM disease,antiglomerular basement membrane(GBM)antibodies(anti-GBM disease)anti-GBM antibody,第二十一页，共五十四页。,（二）vasculitisvasculitis,大血管炎 大动脉炎 巨细胞动脉炎中等血管炎 结节性多动脉炎 川崎病小血管炎 ANCA相关性血管炎 显微镜下型多血管炎（MPA）肉芽肿性多血管炎（GPA，Wegeners）嗜酸细胞性肉芽肿性多血管炎（EGPA，CSS）免疫(miny)复合物相关性小血管炎 冷球蛋白血管炎 IgA血管炎 低补体荨麻疹性血管炎（抗C1q血管炎）,第二十二页，共五十四页。,（三）infection,A variety of both systemic and pulmonary microbial infections can be accompanied by pulmonary hemorrhage and renal disease,including nephritis.In one exceptional case,legionnaires disease军团菌病.No infections could be implicated in this patient.,第二十三页，共五十四页。,第二十四页，共五十四页。,The antinuclear antibody titer was positive at 1:1280and had a homogeneous pattern.The titer forantibodies to double-stranded DNA was positiveat 1:80.,第二十五页，共五十四页。,clinical+imaging+pathology,第二十六页，共五十四页。,第二十七页，共五十四页。,肾小体结构(jigu)模式图,第二十八页，共五十四页。,第二十九页，共五十四页。,*足细胞（podocyte）有初、次级突起，其次级突起间相互(xingh)嵌合为栅栏状，之间有裂孔，上有裂孔膜。,第三十页，共五十四页。,滤过膜 filtration membrane 又称滤过屏障 filtration barrier，由有孔毛细血管(mo x xu un)内皮、基膜和足细胞裂孔膜构成。,第三十一页，共五十四页。,滤过(l u)屏障模式图,返回(fnhu),第三十二页，共五十四页。,diffusely thickened capillarywalls and mild endocapillary proliferation,Cellular crescents,第三十三页，共五十四页。,(arrows),tubular atrophy and interstitial inflammation,fragmented red cells,The GBM was markedly thickened,imparting a“wire loop”appearance白金(bijn)耳,第三十四页，共五十四页。,abundant granular immune-complex deposition in the mesangium and along the glomerular basement membrane in a classic“full house”pattern 满堂(mn tn)亮(anti-IgG immunofluorescence),Immunecomplex deposition is also seen along the tubular basement membrane,第三十五页，共五十四页。,abundant electron-dense depositsare seen in a mesangial,intramembranous,subepithelial,and