腎小球疾病概述(英文版)課件

1、1Outline of Glomerular Disease1Outline of Glomerular Disease2Pathogen PrimarySecondary Hereditary 2Pathogen Primary3Clinical Classifications of Primary Glomerular DiseasesAcute glomerulonephritisRapidly progressive glomerulonephritisChronic glomerulonephritisLatent glomerulonephritisNephrotic syndro

2、me3Clinical Classifications of PAcute glomerulonephritisFollowing infections by nephritogenic strains of Group A hemolytic streptococciproliferation of glomerular cells & infiltration by WBCs, electron dense deposits “humps”About1- 3 weeks after a sore throat or skin infection. Haematuria, proteinur

3、ia, oliguria , Edema.C3 Complement (C3) : usually still to 8 weeks95% with complete recovery.4Acute glomerulonephritisFollow5Rapidly Progressive GN (RPGN)RPGN is characterized by rapidly progressive deterioration in renal function associated with oliguria. Decline in GFR occurs over weeks.Many cresc

4、ents are seen on biopsy.5Rapidly Progressive GN (RPGN)Chronic glomerulonephritisPatients usually have hypertension, hematuria, proteinuria and broad casts.Small kidneys on ultrasound.Generally no specific diagnosis can be established by renal biopsy6Chronic glomerulonephritisPatiLatent glomeruloneph

5、ritisHematuria or proteinuria: onlyNo : edema, hypertension, or renal dysfuction7Latent glomerulonephritisHematNephrotic syndromeMassive 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 injuryHypoalbuminemia 30g/L;Edema Hyper

6、lipidemia, and lipiduria8Nephrotic syndromeMassive prot9Pathologic classifications of Glomerular Diseasesminor glomerular abnormalitie Focal segmental lesion (focal glomerulonephritis)Diffuse glomerulonephritisUnclassified glomerulonephritis9Pathologic classifications of10Diffuse glomerulonephritisM

7、embranous glomerulonephritis, Membranous nephropathy proliferative glomerulonephritis 1.mesangial proliferative glomerulonephritis 2.Endcapillary proliferative glomerulonephritis 3.mesangiocapillary- proliferative glomerulonephritis 4.Crescentic and necrotizing glomerulonephritis Sclerosing glomerul

8、onephritis10Diffuse glomerulonephritisMe腎小球疾病概述(英文版)課件腎小球疾病概述(英文版)課件腎小球疾病概述(英文版)課件14Pathophysiological Mechanisms of GlomerulonephritisImmunologic abnormalities1.Deposition of circulating soluble antigen-antibody complexes, often with complement fragments2.Formation of antibodies against the glomeru

9、lar basement membraneStreptococcal release of neuramidase14Pathophysiological Mechanis15Clinical Features of Glomerular Diseases Proteinuria HematuriaEdema HypertensionRenal Dysfunction15Clinical Features of Glomer16 ProteinuriaGlomerular filtration of proteins present in plasmaProximal tubular reab

10、sorption of filtered proteinAdditional secretion of protein into urine ,such as Tamm-Horsfall mucoprotein16 ProteinuriaGlomerular filtr17 ProteinuriaBarriers to Filtration of ProteinSize molecules Charge Glomerular BM17 ProteinuriaBarriers to Filt1818191920HematuriaGlomerular Dysmorphic RBCs have lo

11、st their biconcave configuration and often have multiple membrane blebs.Non-glomerular20HematuriaGlomerular21EdemaNephrotic Edema hypoalbuminemiaNephritic Edema unbalance of glomerulus and tubules21EdemaNephrotic Edema22HypertensionRenal Dysfunction AcuteChronic22Hypertension23QuestionClinical class

12、ifications of Glomerular DiseasesPathologic classifications of Glomerular Diseases23QuestionClinical classificat24Acute glomerulonephritis24Acute glomerulonephritis25EtiologyFollowing infections by nephritogenic strains of Group A hemolytic streptococciPharyngitis (serotypes 1, 2, 4 and 12)Impetigo

13、(serotypes 47,49, 57)Close contact with potentially infected individuals occurs in child care and grade school25EtiologyFollowing infections26Pathologyenlarged hypercellular glomeruli proliferation of glomerular cells & infiltration by WBCs compression of glomerular capillary lumina granular deposit

14、s of IgG, IgM & C3 in the mesangium & along the GBM electron dense deposits “humps”26Pathologyenlarged hypercellu272728282929303031Clinical FeaturesAbout1- 3 weeks after a sore throat or skin infection.Haematuria :30 50% patients have Cola-or tea-colored urine ,Proteinuria: urine protein + + .Oligur

15、ia:all most patients have variant oliguriaEdema : which star at usually face, then develop to ankles ,even to trunk.31Clinical FeaturesAbout1- 3 w32Clinical FeaturesHypertention :30 70% patients occur still the end of oliguria;Renal involvement ranges from asymptomatic microscopic hematuria toImmune

16、 abnormal: antistreptolysin(ASO) (250); C3 Complement (C3) : usually still to 8 weeks; ARF.Blood routine: Mild anemia , ESR ;32Clinical FeaturesHypertentio33Diagnosis and differential diagnosisOther Glomerulonephritis as Nephritic syndromeOther infection associated Glomerulonephritis.mesangiocapilla

17、ry proliferative glomerulonephritismesangial proliferative glomerulonephritis (IgA of non-IgA)Rapidly progressive glomerulonephritisSystem disease (sle)33Diagnosis and differential d34Treatment and PrognosisIn bed 10 day ,control diet: Na+ restricted diet10-14 day courseIf throat culture positive, n

18、ot for pyodermaDoes not affect course or risk of GNDoes limit the spread of nephritogenic organismsAnti Hypertensive MedsCa + channel blockersVasodilatorsACE inhibitorsManagement of ARF complicationsDialysis Chinese herb34Treatment and PrognosisIn be35Treatment and Prognosis95% with complete recover

19、y.Acute 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 weeksRecurrence is rare.Only rare chage to chronic35Treatment and Prognosis95% w36Rapidly Progressive GN (RPGN)36Rapidly Progressive GN (

20、RPGN37Rapidly Progressive GN (RPGN)RPGN is characterized by rapidly progressive deterioration in renal function associated with oliguria. Decline in GFR occurs over weeks.Many crescents are seen on biopsy.37Rapidly Progressive GN (RPGN38RPGN is classified as followsType I AntiGBM ds/Goodpasture synd

21、romeType II Immune complex mediated like MPGN, Lupus nephritis, Ig A nephropathyType III Pauci-immune ANCA associated vasculitis like Wegners granulomatosismicroscopic polyangiitisType = Type I + ANCA(+) ;Type = Type III + ANCA(-)38RPGN is classified as follow39clinical featuresPatients present with

22、 acute nephritic syndrome, occasionally have the nephrotic syndrome ANCA, anti-GBM & antinuclear Abs are helpful in Dx Rx consists of steroids, cytotoxic drugs & plasmapheresis39clinical features40Type I RPGN AntiGBM ds / Goodpasture syndromeType I RPGN is caused by antibodies directed against antig

23、ens (type IV collagen) in the glomerular and pulmonary alveolar basement membranes.Manifests as GN with rapidly deteriorating renal function and associated pneumonitis with hemoptysis and cough.Peak incidence in young males.Anti-GBM antibodies are present in some patients40Type I RPGN AntiGBM ds / G

24、41Type II RPGNImmune Complex mediatedLupus NephritisCryoglobulinemicMPGNPost-infectious GN41Type II RPGNImmune Complex m42Type III RPGNPauci immune proliferative GN lack of immune deposits on IF and EMWegners granulomatosisMicroscopic polyangiitisChurg-Strauss Disease42Type III RPGNPauci immune pr43

25、Type III RPGNProdrome intermittent fever, arthralgia, weight loss, dyspnea, middle ear effusionWegners sinusitis, epistaxis, hemoptysis. Urine with RBC casts.Peak incidence is in fifth through sixth decade.Renal biopsy is needed to confirm the diagnosis.43Type III RPGNProdrome inte44TreatmentEarly d

26、iagnosis is essential for successful treatment.Plasmapheresis is recommended to remove anti-GBM antibodies.Steroids and cyclophosphamide are used to stop further production of antibodies.Renal replace treatment44TreatmentEarly diagnosis is 45Chronic glomerulonephritis45Chronic glomerulonephritis46cl

27、inical featuresPatients usually have hypertension, hematuria, proteinuria and broad casts.Small kidneys on ultrasound.Generally no specific diagnosis can be established by renal biopsy46clinical featuresPatients us47TreatmentControl of BPUse of ACE inhibitorReduce intake of protein and phosphate Con

28、trol of hyperlipidemiaCortisone Aspirin and persantin Avoidance of other nephrotoxic substancesCessation of smoking47TreatmentControl of BP48Ig A Nephropathy48Ig A Nephropathy49IgA Nephropathy (Berger Disease)IgA nephropathy is the most common primary GN worldwideUsually present with hematuriaEpisod

29、es of gross hematuria are precipitated by flu like illness, exerciseUrinary protein excretion usually non-nephroticAssociated with chronic liver ds, psoriasis, IBD and HIV disease.49IgA Nephropathy (Berger Dise50pathogenesisAbnormality of immune regulation mucosal IgA Synthesis in response to respir

30、atory or GI exposure to environmental antigens IgA1 & IgA1 immune complexes are then entrapped in the mesangiumactivation of alternative complement pathway glomerular injury50pathogenesisAbnormality of i51secondary IgA nephropathyLiver disease Celiac disease Henoch-Schnlein purpura51secondary IgA nephropathyLiv52PathologyCharacterized by presence of prominent deposits of IgA in the mesangium MorphologyConsiderable variation on light microscopy: glomeruli may