肾病综合征(英文)-儿科课件-.ppt

,单击此处编辑母版标题样式,单击此处编辑母版文本样式,第二级,第三级,第四级,第五级,*,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 proteinuria,ISKDC:40mg/m,2,/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 133umol/L,Hypocomplementemia:C,3,7y,moderate edema,gross hematuria,hypertension,Complications,:,Complications(1),Infection,:URI,peritonitis,cellulitis and etc,IgG,IgA,Complement,WBC function,Lack of Zn and other trace elements,Hypercoagulable state and thrombosis,Higher concentration of,Lower level of anticoagulant substance:antithrombin，protein S,protein C,Overvigorous diuresis,Higher blood viscosity,increased platelet aggregation,Role of corticosteroids,Complications(2),Electrolyte imbalance,:hyponatrimia,hypokalemia,hypercalcemia,Salt-depleted diet,Overvigorous diuresis,Extrarenal loss,Protein-bound calcium loss from urine,Steroids induced hypocalcemia,ARF:,pre-renal,Hypovolemic shock,Others,:growth failure,Laboratory Data(1),ESR,:simple nephrosis 100mm/h,nephritic nephrosis IgM,C,3,nephritic nephrosis,IgMIgA,normal C,3,simple nephrosis,Laboratory Data(2),Renal function:usually normal,Urine protein pattern:,simple nephrosis,albumin,nephritic nephrosis,IgG,albumin and others,Ratio of U,IgG,/U,albumin,simple nephrosis,1,Diagnosis and differential diagnosis,Idiopathic or secondary,MCN or non-MCN,importance of renal biopsy,Treatment of NS,General principle,Anticoagulation,Corticosteroids,Immunosuppressive agents,Chinese traditional medicine,General principle,Low salt diet(2g/d),appropriate protein intake(2-3g/kg/d),Avoiding infection,Diuresis:,Thiazide DHCT 2mg/kg/d,Antisterone 2-4mg/kg/d,Dextran 10-20ml/kg followed by,Lasix at 2mg/kg,Anticoagulation,Dipyridamole:5mg/kg/d,Heparin:0.5-1mg/kg/d7-10d,Warfarin:initial dose:2.5mgTid3-5d,Subsequent dose:2.5-10mg/d,based on PT,Corticosteroids,Short course,:2mg/kg/d pro(-)1.5mg/kg/qod4w no taper,Course,8w,Relapse rate(1y)=,81%,Standard course,:2mg/kg/d4w,2mg/kg/qod4w,taper,Course,6m,Relapse rate(1y)=,61%,Long course,:2mg/kg/d4-6w,2mg/kg/qod4-6w,taper,Course,9-12m,Relapse rate(1y)=,31%,Steroids treatment response,high response,:4wproteinuria(-),response,:8wproteinuria(-),partial response,:8wproteinuria(+/+),steroid dependent,:responsive but require high dose,relapse,:proteinuria(-)proteinuria(+or up),frequent relapse,:relapse twice/6m or trice/1y,Side effect of steroids,Growth failure,Hypertension,posterior sublenticular cataracts,Osteoporotic bone disease,Gastric ulcerhematesis,Immunosuppressive agents(1),CTX,:2-2.5mg/kg/d8-12w,maxium single dose 0.1,maxium cumulative 200-250mg/kg,Chlorambucil,:0.2mg/kg/d8-12w,maxium single dose 6mg,maxium cumulative dose 12-16mg/kg,Cyclosporin A,:5-6mg/kg/d2-6m,keep blood concentration at 50-150ng/ml,Immunosuppressive agents(2),Vincristine,:0.075mg/kg weekly,maxium single dose2mg,6-TG(6-thioguanine),:2mg/kg/d8w,maxium single dose75mg,nitrogen mustard,:0.1mg/kg/d4d,may be repeated 1 month later,azathioprine,:1-2mg/kg/d8-12w,New immunosuppressive agents,Mycophenolate mofetil acid(MMF):Cellcept,FK506:Prograf,Tacrolimus,Rapamycin:Sirulimus,Simulect,basiliximab,daclizumab,FYT-720、Gusperimus、SDS-RAD,Pulse therapy,Methylprednisolone:15-30mg/kg3d,Indication:refractory nephrosis,Lupus,RPGN:cocktail protocol,FSGS:Mendoza protocol,MN:Ponticelli protocol,Prognosis,Generally good,especially for MCN,MsPGN:good or poor,FSGS:50%to ESRF,Male,4 years and 6 months old,Complaint of edema and oliguria,