医学课件 IgA肾病业务学习.ppt

GlobulardepositsofIgA(alongwithC3andIgG)asdemonstratedbyImmunofluorescencemicroscopyinthemesangiumandglomerularcapillarywall.Electronmicroscopyshowingelectrondensedepositsofmesangium.Mesangialelectrondensedepositswith↑mesangialmatrixcellularityinIgANClinicalPresentationClinicalfeature：1.predilectioninyoungmen：predominate:13～15yr2.manyptswithforerunnerinfectionbeforeepisodeorrelapse3.hematuriaisverycommon一、hematuria：infectionortired40to50%presentwithrecurrentgrosshematuria.30to50%withmicroscopichematuriaandprotienuria.20to25%progresstogrosshematuria.二、proteinuriawithorwithoutmicroscopichematuriathecommonestclinicalfeature(50%)三、nephroticsyndromeabout3~4%，areportinChina16.7％四、HypertensionAdultIgA50%,children5%。about6yrsbeforerenalfailure。somepatientsmalignanthypertension五、Chronicrenalfailure10～20yrsafterdignosis，someptsatthefirstvisit。六、Acuterenalfailure（10％）1.ATN:RBCblockkidneytubules2.Acutenephriticsyndrome，transientRF，pathologyissameasAGN3.Rapidlyprogressiveglomerulonephritis,RPGN，progressiverenalfunctiondeteriorationFiveClinicalsyndromes1.Grosshematuria2.Asymptomaticmicroscopichematuriawith/withoutproteinuria----62%3.Acutenephritiswithhypertensionand/orrenalinsufficiency4.Nephroticsyndrome5.Amixednephritic-nephroticsyndromeLaboratoryexaminationUA:hematuria,proteinuriaserumIgA↑(30～70%，China10～30％)IgG，IgMarenormal，C3，CH50arenormalorslightly↑IgA-FN↑，IgA-IC↑someptswithHBsAgpositiveDiagnosisimmunohistochemicalfindingsofmesangialdepositionofIgAlightmicroscopy:mesangialcellproliferationisthecommonestfeatureDifferentialdiagnosis

*O-糖基半乳糖（GM）缺陷的异常糖基化IgA1分子，此种缺陷的IgA1不能被脱唾液酸糖蛋白受体识别，从而肝细胞无法将其清除。Case24-year-old,maleMaincomplaintsRepeatedgrosshema