《肾病综合征（英文）儿科课件》由会员分享，可在线阅读，更多相关《肾病综合征（英文）儿科课件（43页珍藏版）》请在金锄头文库上搜索。
1、Nephrotic Syndrome,Department of Prdiatrics, Tongji Hospital,Nephrotic Syndrome,Definition Etiology Pathology Pathophysiology Clinical Manifestation Laboratory Data Diagnosis Therapy and Prognosis,Male 4 years and 6 months old Complaint of edema and oliguria,Definition: Nephrotic Criteria,Massive pr
2、oteinuria ISKDC: 40mg/m2/hr CAN: + trice/2w or 50-100mg/kg/24hr Mendoza: Urine Protein/Cr 2.0 Hypoalbuminemia: 5.72mmol/L Edema,Definition: Nephritic Criteria,Hematuria: RBC +( 10 /HP), trice/2w Hypertension: 130/90 mmHg in children over 7y 120/80 mm Hg in 3-6y children 110/70 mm Hg in 6.4mmol/L, Cr
3、 133umol/L Hypocomplementemia: C3 50% Secondary NS: dependent on causes, APSGN, MN, FSGS, MsPGN or MPGN,Minimal Change Nephropathy (MCN ),Little or no microscopic abnormality Absence of immune complexes Effacement of epithelial foot process Occasionally mesangial hypercellurity,MCN: normal in LM,MCN
4、: effacement of epithelial foot process in EM,Mesangial proliferative glomerulonephritis (MsPGN),Mesangial proliferation and expansionIgG,C3 and sometimes IgA, IgM deposits in mesangial stalkElectron-dense deposits in mesangial or paramesangial areas,MsPGN: Mesangial proliferation and expansionIgG a
5、nd C3 deposits in mesangial,Mesangial proliferative glomerulonephritis (MsPGN),Mesangial proliferation and expansionIgG,C3 and sometimes IgA, IgM deposits in mesangial stalkElectron-dense deposits in mesangial or paramesangial areas,Focal segmental glomerulosclerosis (FSGS),Focal and segmental capil
6、lary collapse and mesangial sclerosis Deposits of IgM or C3 in the glomeruli Loss of visceral epithelial cell podocytes, duplication of the basal GBM lamina,separation of epithelial cell from GBM,Membranous Nephropathy (MN),Diffuse GBM thickening, characteristic GBM spikes Subepithelial deposit of I
7、gG and C3,Membrane proliferative glomerulonephritis (MPGN),Mesangial proliferation and expansion Subendothelial mesangial interposition, tram track apperance Mesangial and subendothelial deposits of IgG and C3,Pathophysiology of nephrotic syndrome,MCN: Involvement of immune system,No Ig or complemen
8、t deposit Association between allegy and idiopathic NS Abnormalities of humural and cellular immunity: IgG, IgA, CD4/CD8 Relapse of NS triggered by a variety of minor infections Autologous remission after measles Induction of remission by corticosteroids and akylating agents,MCN: pathogenesis of pro
9、teinuria,Lymphacyte 29kd peptide glomerular polyanion proteinuria Con A lymphacytes 60-160kd GPF proteinuria lymphacytes 13-18kd SIRS proteinuriaGPF: glomerular permeability factorSIRS: soluble immune response suppressor,MCN: pathogenesis of edema,FFNa, CH2O Edema Proteinuria Na reabsorption in dist
10、al renal tubules Na and water retention Edema Hypoalbuminemia intravascular oncotic pressure (25mmHg6-8mmHg) Fluid extravasation hypovolemia ADH and aldosterone Water and salt retension Edema,MCN: pathogenesis of hypoalbuminemia,Loss of protein from ultrafiltrationIncreased catabolism of protein in
11、renal tubules Loss from intestine proved by 51Chromium-albumin tracing,MCN: pathogenesis of hyperlipidemia,Hypoalbuminemia hypatic synthesis of lipidhyperlipidemia,Clinical Manifestation,Simple nephrosis: 2-7y, massive edema in face and paraorbital areas, ascites, pleural effusion, loss of appetite,
12、 nausea and vomiting, inertia and lethargy Nephritic nephrosis: 7y, moderate edema, gross hematuria, hypertension Complications:,Complications (1),Infection: URI, peritonitis, cellulitis and etcIgG, IgA, Complement WBC function Lack of Zn and other trace elements Hypercoagulable state and thrombosisHigher concentration of ,Lower level of anticoagulant substance: antithrombin，protein S,protein COvervigorous diuresisHigher blood viscosity, increased platelet aggregationRole of corticosteroids,
