内科学-培训课件PPT
内科学-培训课件PPT,内科学,培训,课件,PPT
1Outline of Glomerular DiseaseDivision of nephrology,the first affiliated hospital of Guangzhoumedical collegePeng Yonghua2Pathogen vPrimaryvSecondary vHereditary 3Clinical Classifications of Primary Glomerular DiseasesuAcute glomerulonephritisuRapidly progressive glomerulonephritisuChronic glomerulonephritisuLatent glomerulonephritisuNephrotic syndromeAcute glomerulonephritisvFollowing infections by nephritogenic strains of Group A hemolytic streptococcivproliferation of glomerular cells&infiltration by WBCs,electron dense deposits “humps”vAbout1-3 weeks after a sore throat or skin infection.Haematuria,proteinuria,oliguria,Edema.vC3 Complement(C3):usually still to 8 weeksv95%with complete recovery.45Rapidly Progressive GN(RPGN)vRPGN is characterized by rapidly progressive deterioration in renal function associated with oliguria.vDecline in GFR occurs over weeks.vMany crescents are seen on biopsy.Chronic glomerulonephritisvPatients usually have hypertension,hematuria,proteinuria and broad casts.vSmall kidneys on ultrasound.vGenerally no specific diagnosis can be established by renal biopsy6Latent glomerulonephritisvHematuria or proteinuria:onlyvNo:edema,hypertension,or renal dysfuction7Nephrotic syndromevMassive proteinuria:Excretion of 3.5 g or more of protein in the urine per day(3.5g/d)The protein excretion is due to glomerular injuryvHypoalbuminemia 30g/L;vEdemav Hyperlipidemia,and lipiduria89Pathologic classifications of Glomerular Diseasesvminor glomerular abnormalitie vFocal segmental lesion(focal glomerulonephritis)vDiffuse glomerulonephritisvUnclassified glomerulonephritis10Diffuse glomerulonephritisMembranous glomerulonephritis,Membranous nephropathy proliferative glomerulonephritis 1.mesangial proliferative glomerulonephritis 2.Endcapillary proliferative glomerulonephritis 3.mesangiocapillary-proliferative glomerulonephritis 4.Crescentic and necrotizing glomerulonephritis Sclerosing glomerulonephritis14Pathophysiological Mechanisms of GlomerulonephritisImmunologic abnormalities1.Deposition of circulating soluble antigen-antibody complexes,often with complement fragments2.Formation of antibodies against the glomerular basement membraneStreptococcal release of neuramidase15Clinical Features of Glomerular Diseasesv Proteinuriav HematuriavEdema vHypertensionvRenal Dysfunction16 ProteinuriaGlomerular filtration of proteins present in plasmavProximal tubular reabsorption of filtered proteinvAdditional secretion of protein into urine,such as Tamm-Horsfall mucoprotein17 ProteinuriavBarriers to Filtration of ProteinSize molecules Charge Glomerular BM181920HematuriavGlomerular Dysmorphic RBCs have lost their biconcave configuration and often have multiple membrane blebs.vNon-glomerular21EdemavNephrotic Edema hypoalbuminemiavNephritic Edema unbalance of glomerulus and tubules22vHypertensionvRenal Dysfunction v AcuteChronic23QuestionvClinical classifications of Glomerular DiseasesvPathologic classifications of Glomerular Diseases24Acute glomerulonephritis25EtiologyvFollowing infections by nephritogenic strains of Group A hemolytic streptococciPharyngitis(serotypes 1,2,4 and 12)Impetigo(serotypes 47,49,57)vClose contact with potentially infected individuals occurs in child care and grade school26Pathologyvenlarged hypercellular glomeruli vproliferation of glomerular cells&infiltration by WBCsv compression of glomerular capillary lumina v granular deposits of IgG,IgM&C3 in the mesangium&along the GBM v electron dense deposits “humps”2728293031Clinical FeaturesvAbout1-3 weeks after a sore throat or skin infection.vHaematuria:30 50%patients have Cola-or tea-colored urine,Proteinuria:urine protein+.vOliguria:all most patients have variant oliguriavEdema:which star at usually face,then develop to ankles,even to trunk.32Clinical FeaturesvHypertention:30 70%patients occur still the end of oliguria;vRenal involvement ranges from asymptomatic microscopic hematuria tovImmune abnormal:antistreptolysin(ASO)(250);C3 Complement(C3):usually still to 8 weeks;ARF.Blood routine:Mild anemia,ESR ;33Diagnosis and differential diagnosisvOther Glomerulonephritis as Nephritic syndromeOther infection associated Glomerulonephritis.mesangiocapillary proliferative glomerulonephritismesangial proliferative glomerulonephritis(IgA of non-IgA)Rapidly progressive glomerulonephritisSystem disease(sle)34Treatment and PrognosisvIn bed 10 day,control diet:Na+restricted dietv10-14 day courseIf throat culture positive,not for pyodermaDoes not affect course or risk of GNDoes limit the spread of nephritogenic organismsvAnti Hypertensive MedsCa+channel blockersVasodilatorsACE inhibitorsvManagement of ARF complicationsvDialysis vChinese herb35Treatment and Prognosisv95%with complete recovery.vAcute Phase resolves anywhere from 1-4weeks after onset.Hematuria(gross or microscopic)can continue for up to1/2-1 yearC3 Complement(C3):usually still to 8 weeksvRecurrence is rare.vOnly rare chage to chronic36Rapidly Progressive GN(RPGN)37Rapidly Progressive GN(RPGN)vRPGN is characterized by rapidly progressive deterioration in renal function associated with oliguria.vDecline in GFR occurs over weeks.vMany crescents are seen on biopsy.38RPGN is classified as followsvType I AntiGBM ds/Goodpasture syndromevType II Immune complex mediated like MPGN,Lupus nephritis,Ig A nephropathyvType III Pauci-immune ANCA associated vasculitis like Wegners granulomatosismicroscopic polyangiitisvType=Type I+ANCA(+);vType=Type III+ANCA(-)39clinical featuresPatients present with acute nephritic syndrome,occasionally have the nephrotic syndrome ANCA,anti-GBM&antinuclear Abs are helpful in Dx Rx consists of steroids,cytotoxic drugs&plasmapheresis40Type I RPGN AntiGBM ds/Goodpasture syndromevType I RPGN is caused by antibodies directed against antigens(type IV collagen)in the glomerular and pulmonary alveolar basement membranes.vManifests as GN with rapidly deteriorating renal function and associated pneumonitis with hemoptysis and cough.vPeak incidence in young males.vAnti-GBM antibodies are present in some patients41Type II RPGNvImmune Complex mediatedLupus NephritisCryoglobulinemicMPGNPost-infectious GN42Type III RPGNvPauci immune proliferative GN lack of immune deposits on IF and EMWegners granulomatosisMicroscopic polyangiitisChurg-Strauss Disease43Type III RPGNvProdrome intermittent fever,arthralgia,weight loss,dyspnea,middle ear effusionvWegners sinusitis,epistaxis,hemoptysis.Urine with RBC casts.vPeak incidence is in fifth through sixth decade.vRenal biopsy is needed to confirm the diagnosis.44TreatmentvEarly diagnosis is essential for successful treatment.vPlasmapheresis is recommended to remove anti-GBM antibodies.vSteroids and cyclophosphamide are used to stop further production of antibodies.vRenal replace treatment45Chronic glomerulonephritis46clinical featuresvPatients usually have hypertension,hematuria,proteinuria and broad casts.vSmall kidneys on ultrasound.vGenerally no specific diagnosis can be established by renal biopsy47TreatmentvControl of BPvUse of ACE inhibitorvReduce intake of protein and phosphate vControl of hyperlipidemiavCortisone vAspirin and persantin vAvoidance of other nephrotoxic substancesvCessation of smoking48Ig A Nephropathy49IgA Nephropathy(Berger Disease)vIgA nephropathy is the most common primary GN worldwidevUsually present with hematuriavEpisodes of gross hematuria are precipitated by flu like illness,exercisevUrinary protein excretion usually non-nephroticvAssociated with chronic liver ds,psoriasis,IBD and HIV disease.50pathogenesisAbnormality of immune regulation v mucosal IgA Synthesis in response to respiratory or GI exposure to environmental antigens IgA1&IgA1 immune complexes are then entrapped in the mesangiumvactivation of alternative complement pathway vglomerular injury51secondary IgA nephropathyLiver disease Celiac disease Henoch-Schnlein purpura52PathologyvCharacterized by presence of prominent deposits of IgA in the mesangium vMorphologyConsiderable variation on light microscopy:glomeruli may appear normal,may exhibit mesangioproliferative GN,focal proliferative GN or rarely crescentic GN IF reveals mesangial deposition of IgA,often with C3,early complement components are absent53clinical courseaffects children&young adults present with hematuria often after a respiratory infection All kind nephritis and nephrotic syndrom ARF10%,Only 30%of patients with IgA nephropathy has progressive diseaseslow progression to CRF in 15-40%of cases in 20 years54TreatmentvTreatment is depended on type of nephritis and nephrotic syndrom vIn progressive disease,use of ACEI of fish oil may be beneficial,may be not enough.vImmunosuppressive therapy in patients with Ig A nephropathy has not consistently shown to be of benefit55QuestionvClinical classifications of Glomerular DiseasesvPathologic classifications of Glomerular DiseasesvDefinition of Rapidly Progressive GNvWhat have we learned?56vThank you for your attention!
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