普通型變異缺陷綜合征_第1頁
普通型變異缺陷綜合征_第2頁
普通型變異缺陷綜合征_第3頁
普通型變異缺陷綜合征_第4頁
普通型變異缺陷綜合征_第5頁
已閱讀5頁，還剩19頁未讀， 繼續(xù)免費(fèi)閱讀

下載本文檔

版權(quán)說明：本文檔由用戶提供并上傳，收益歸屬內(nèi)容提供方，若內(nèi)容存在侵權(quán)，請進(jìn)行舉報(bào)或認(rèn)領(lǐng)

文檔簡介

CVIDcommonvariableimmunodeficiencyCASESTUDY

A29-year-oldmanhadahistoryofcough,sputumandhemoptysisfor22years.Thediseaseoutbreaks3to5timeswithadurationofonemonth..Hehadattackofviralmyocarditis,pleuritis,sinusitisandbacterialpneumonia.Whenhewasadmittedintohospital,hewasinfever(T=39-41℃),cough,blood-tingedsputumwithnightsweatfor17days.CASESTUDYT=38.7℃

HeartRate=96times/minBloodPressure=136/75mmHgQuantitationofserumimmunoglobulinsshowed:IgG<1.89g/L(NR:8-15g/L)IgM<0.225g/L(NR:0.83~3.0g/L)IgA<0.183g/L(NR:0.5~2.5g/L)Bcellcountisnormal.DiagnosisLowerrespiratorytractinfectionCommonvariableimmunodeficiency(CVID)*QUESTIONS*WhatisCVID?Whydoesthepatientsufferfromfrequentinfections?Howdoseittreatedandwhatabouttheprognosis?CVID

Commonvariableimmunodeficiencyisagroupofunclarifiedimmunediseaseswithlowbloodlevelofgammaimmunoglobulinasitscharacter.Mechanism1.Geneticfactor2.Bcellabnormality3.Tcellabnormality4.AutoimmunityYES!!PatientswithCVIDhassomethingwrongwithIgproduction↓ThepatientislackofimmunoglobulinLet’scometothefunctionofIg!

ThefunctionofAb1)

Neutralization2)

Activatecomplementsystem3)Opsonization–macrophage4)

ADCC—NKADCCIgG1.Highestconcentrationinserum(75%oftotalIg)2.FunctionsofIgG:againstbacteriaandvirus,neutralizetoxinsomebelongtotheauto-antibodies:eg.longactivethyroidstimulator(LATS)

combinewiththeFcreceptor(FcγR)Now,weknowtheantibodyplaysanimportantroleinanti-infectionsofourbodies.-----ohoh!Ifwearelackofantibody,ourbodieswillbesusceptivetoinfections!!SuchasinCVID!!Treatment

Immunoglobulinandfrequentuseofantibioticareusuallyrequired.Additionalimmunoglobulinshouldbegivenduringanacuteillness.Thegamma-immunoglobulinisthefirstchoice(0.35-0.5g/Kgeverymonth)tokeepthelevelofserumIgGat3-4g/L.Ifplasmaisused,thedosageis100mg/Kgeverymonth.PrognosisEventhoughthepatientsmaylivealonglife(70)withpropertreatmentmentionedabove,theprognosiscannotbeconsideredsatisfiedenough.Becausemanypatientsmayinducedintochronicinfections,especiallythechronicrespiratoryinfections;partofthepatientscomplicatedwithautoimmunitydiseaseandcancer.ThereisstillalongjourneybeforewecuretheCVID!Let’sstart,NOW!!

THANKYOU!!1GeneticFactor

Eventhoughmostcasesarescattered,somecaseshavefamilyhistory,especiallyoccuralongwithselectiveIgA

abscentdisease.Fromthetruthabove,wecaninducethatbothdiseasesduetosomegenemutation.MHCⅢcomplementgenemutationisthemostsuspicious.

Bcellabnormality(1)AbnormalBcellproduction(2)DisorderofBcelldifferentiation(3)DisfuntionofBcell(4)InhibitoryfactorofBcellsTcellabnormality(1)DisfunctionofTh2cell(2)DisfunctionofTregcellNormalTh2helpBcellactivationTreg

cellTregcellisakindofCD4+TcellwithCD25expressedonitanditcanproduceakindofproteincalledfoxp3.Tregcellplaysanimportantroleinregulationofimmuneresponse.ItsuppressesBcellresponsebysecretingcytokinessuchasTGF-betaandIL-10.4Autoimmunity

Insomecases,serologicaltestshowedpositiveanti-Bcellantibodyoranti-Tcellantibody,thefindingin

溫馨提示

  • 1. 本站所有資源如無特殊說明，都需要本地電腦安裝OFFICE2007和PDF閱讀器。圖紙軟件為CAD,CAXA,PROE,UG,SolidWorks等.壓縮文件請下載最新的WinRAR軟件解壓。
  • 2. 本站的文檔不包含任何第三方提供的附件圖紙等，如果需要附件，請聯(lián)系上傳者。文件的所有權(quán)益歸上傳用戶所有。
  • 3. 本站RAR壓縮包中若帶圖紙，網(wǎng)頁內(nèi)容里面會有圖紙預(yù)覽，若沒有圖紙預(yù)覽就沒有圖紙。
  • 4. 未經(jīng)權(quán)益所有人同意不得將文件中的內(nèi)容挪作商業(yè)或盈利用途。
  • 5. 人人文庫網(wǎng)僅提供信息存儲空間，僅對用戶上傳內(nèi)容的表現(xiàn)方式做保護(hù)處理，對用戶上傳分享的文檔內(nèi)容本身不做任何修改或編輯，并不能對任何下載內(nèi)容負(fù)責(zé)。
  • 6. 下載文件中如有侵權(quán)或不適當(dāng)內(nèi)容，請與我們聯(lián)系，我們立即糾正。
  • 7. 本站不保證下載資源的準(zhǔn)確性、安全性和完整性, 同時也不承擔(dān)用戶因使用這些下載資源對自己和他人造成任何形式的傷害或損失。

最新文檔

評論

0/150

提交評論