新英格蘭雙語病例討論2

Bilingualcasediscussion2021-09-11

A35-year-oldmanwithdyspnea,anemia,andrenalfailureseveralweeksago,thepatientfeltfatigue.Twodaysbeforeadmission,dyspneadeveloped,decreasedurination,withoutfever,cough,orchestpain,nohemoptysis咯血,chills,nightsweats,headaches,visualloss,dryeyes,drymouth,orjointpain.Hewastransferredbyambulancetotheemergencydepartmentatthishospital

Fouryearsbeforeadmission,hehadbeenevaluatedbecauseofbackpain,fatigue,andatemperatureof38.6°C.Urinalysisshowedhematuria血尿andproteinuria；testingforrapidplasmareagin(RPR快速血漿反響素)waspositiveforantibodiestoTreponemapallidum〔梅毒螺旋體〕.Duringtheprevious6months,thepatienthadhadepisodesofbilateralfinger,ankle,andfacialswelling,withoutpainorchangeincolor.Hehadnohistoryofrecenttravel,exposuretosickpersons,bloodtransfusions,orprevioussurgery.Hedidnotsmoke,drinkalcohol,oruseillicitdrugs.

Onexamination,T36.9°C,BP173/89mmHg,P95b/m,R36b/m,andS0288%(ambientair).Theskinandconjunctivae結(jié)膜werepale,andtherewerehypopigmentedmacules色素減退斑ontherighttempleandbothlowercheeksandhyperpigmentedmacules色素沉著斑onthebridgeofthenose.Therewerebibasilarrales雙肺底濕羅音inthelungs,andtheremainderoftheexaminationwasnormal.

totalanddirectbilirubin膽紅素,liver-functiontestsandlactatewerenormal.testingforrheumatoidfactor,screeningofthebloodandurinefortoxinswerenegative;TestingforhepatitisBandCvirusesandautoantibodiesagainsthistones組蛋白wasnegative.Serumproteinelectrophoresis電泳revealedadiffuseincreaseintheIgGlevel.Transthoraciccardiacultrasonographyrevealednormalglobalcardiacfunctionandright-ventricularsize,noevidenceofapericardialeffusion心包積液,andfindingsthatwereconsistentwithpulmonaryEdema.Ultrasonographyoftheabdomenrevealednormalrenalsize,position,andechotexture回聲特性andnormalarterialbloodflow.Anelectrocardiogram(ECG)showedsinustachycardia,counterclockwiserotation逆鐘向,andnonspecificST-segmentandT-waveabnormalities.onadmissionground-glassopacitiesGGOBronchoscopicexaminationrevealedthick,redmucusinthemain-stemandright-lower-lobebronchi;airwaysoftheleftlungwerenormal.Bronchoalveolarlavageontheright,with300and24,500redcellspercubicmillimeter(inthefirsttube),975and1475whitecellspercubicmillimeter(infourthtubes)Inthefourthtube,thewhite-celldifferentialcountrevealed84%leukocytes白細胞.Onthesecondday,thesputumculturegrewveryfewklebsiella克雷伯

whatdoyouthink

Pulmonaryhemorrhage？PCP卡氏肺孢子？〔梅毒陽性、HIV〕pulmonaryembolism肺栓塞？

RapidlyprogressiveglomerulonephritisPulmonaryhemorrhage

ErnestW.Goodpasturereportedin1919ontheautopsyfindingsinthecaseofan18-year-oldmanwhohaddiedofmassivelunghemorrhageandcrescenticglomerulonephritisduringtheheightoftheinfluenzapandemicThetermGoodpasture’ssyndromeisappliedtothecombinationoflungpurpuraandnephritis,regardlessoftheunderlyingpathogenesis.(一)anti-GBMdiseaseanti–glomerularbasementmembrane(GBM)antibodies(anti-GBMdisease)anti-GBMantibody〔二〕vasculitisvasculitis大血管炎大動脈炎巨細胞動脈炎中等血管炎結(jié)節(jié)性多動脈炎川崎病小血管炎ANCA相關(guān)性血管炎顯微鏡下型多血管炎〔MPA〕肉芽腫性多血管炎〔GPA，Wegener’s〕嗜酸細胞性肉芽腫性多血管炎〔EGPA，CSS〕免疫復合物相關(guān)性小血管炎冷球蛋白血管炎IgA血管炎低補體蕁麻疹性血管炎〔抗C1q血管炎〕〔三〕infectionAvarietyofbothsystemicandpulmonarymicrobialinfectionscanbeaccompaniedbypulmonaryhemorrhageandrenaldisease,includingnephritis.Inoneexceptionalcase,legionnaires’disease軍團菌病.Noinfectionscouldbeimplicatedinthispatient.Theantinuclearantibodytiterwaspositiveat1:1280andhadahomogeneouspattern.Thetiterforantibodiestodouble-strandedDNAwaspositiveat1:80.

clinical+imaging+pathology

腎小體結(jié)構(gòu)模式圖

*足細胞〔podocyte〕有初、次級突起，其次級突起間相互嵌合為柵欄狀，之間有裂孔，上有裂孔膜。③濾過膜filtration

membrane

又稱濾過屏障

filtrationbarrier，由有孔毛細血管內(nèi)皮、基膜和足細胞裂孔膜構(gòu)成。

濾過屏障模式圖

返回diffuselythickenedcapillary

wallsandmildendocapillaryproliferationCellularcrescents(arrows)tubularatrophyandinterstitialinflammationfragmentedredcellsTheGBMwasmarkedlythickened,impartinga“wireloop〞appearance白金耳abundantgranularimmune-complexdepositioninthemesangiumandalongtheglomerularbasementmembraneinaclassic“fullhouse〞pattern滿堂亮(anti-IgGimmunofluorescence)Immunecomplexdepositionisalsoseenalongthetubularbasementmembraneabundantelectron-densedepositsareseeninamesangial,intramembranous,subepithelial,andsubendothelialdistributionshowedstrongstainingwithIgG,IgM,IgA,C3,C1q,andkappaandlambdalightchainsinagranularpatterninthemesangiumandalongtheGBM所涉及的病理學術(shù)語的定義彌漫性病變(diffuse)：病變累及50％腎小球。局灶性病變(focal)：病變僅累及≤50％腎小球。球性病變(global)：病變累及一個腎小球的大局部毛細血管袢(>50％)。節(jié)段性病變(segmental)：病變僅累及一個腎小球的少局部毛細血管袢(≤50％)。系膜細胞增生(mesangialhypercellularity)：3μm切片中，一個系膜區(qū)超過3個細胞。毛細血管內(nèi)增生(endocapillaryproliferation)：腎小球毛細血管內(nèi)皮細胞和系膜細胞增生，單個核細胞浸潤，導致毛細血管腔狹窄。I型輕微病變性狼瘡性腎炎II型系膜增殖性狼瘡性腎炎III型局灶性狼瘡性腎炎III〔A〕：活動性病變—局灶增殖性LNIII〔A/C〕：活動和慢性化病變—局灶增殖和硬化性LNIII〔C〕：慢性非活動性病變—局灶硬化性LNIV型彌漫性狼瘡性腎炎IV-S〔A〕IV-G〔A〕IV-S〔A/C〕Ⅴ型膜性狼瘡性腎炎Ⅵ型終末硬化性狼瘡性腎炎狼瘡性腎炎的病理組織學分類〔ISN/RPS2003〕IV-G〔A/C〕IV-S〔C〕IV-G〔C〕根據(jù)活動性和硬化性病變又分為多個亞型IV-S〔A〕：活動性病變—彌漫節(jié)段增殖性LNIV-G〔A〕：活動性病變—彌漫球性增殖性LN