1、肾病综合征英文肾病综合征英文 Definition of Nephrotic syndrome Etiology Pathogenesis Clinical picture Diagnostic workup Pathological picture Complication Managements proteinuria(3.5g/day),hypoalbuminemia(3,5 g/day(nephrotic-range proteinuria)The history and physical examination Systemic diseaseSerologic studies(AN
2、A),complement,hepatitis B and hepatitis C serologies and the measurement of cryoglobulins,serum or urine protein electrophoresis.Renal biopsy required to establish the diagnosis in most of times.BUN,creatinine,creatinin clearnce.bicarbonates,chloride serum albumin,serum proteins,calcium,Lipid profil
3、e,Coagulation tests AdultsRenal biopsy is mandatory for every nephrotic patient except the diabetic patient.In one study of adults with nephrotic range proteinuria,knowledge of the histology altered management 40%!Children glucocorticoid therapy is usually begun empirically and a renal biopsy is per
4、formed only for glucocorticoid-resistant disease.-Management of patients with nephrotic syndrome.Swissmedwkly 2009;139(29-30):416-422.-Knowledge of renal histology alters patient management in over 40 percent of patients.Nephrol Dial Transplant 1994;9:1255.10%of nephrotic syndrome cases in diabetes
5、are due to other renal diseases*Presence atypical features such as1-A rapidly progressive nephrotic syndrome 2-Acute renal failure3-Presence of glomerular haematuria and/or absence of associated microvascular lesions(retinopathy,neuropathy)Management of patients with nephrotic syndrome.Swissmedwkly
6、2009;139(29-30):416-422.36Histologic PatternKey Pathologic FeaturesMCDLM NormalEMFoot process fusionMNLMThickening of the GBM EMSubepithelial deposits of IgG FSGSLMFocal and segmental sclerosis of glomeruliEMFocal or diffuse foot process effacement.MPGNLMthickening of all capillary walls+cellular pr
7、oliferation EMThe double-contour or tram-track appearance represents interposition of mesangial cell with the GBM -subendothelial(type I)or intramembranous(type II)Due to loss of proteins in the urineDue to oncotic pressureImmunoglobulinsusceptibility to infectionantithrombin III and proteins C and
8、SThromboembolismvit Dbinding protein vit D deficiencyTransferrinIron deficiency anemiaHyperlipidaemiaHypovolemia Acute renal failureAnasarcarisk of cellulitis,bacterial peritonitis with ascites,large pleural effusions or pulmonary edema38Oedema Low salt dietDiureticsserial measurement of body weight
9、Proteinuria ACE inhibitors or ARBsHypoalbuminaemiaHigh protein diet not indicated0.81 g/kg/dayRef:Up to date online 17.3.Hyperlipidaemia Regular Lipid profileStatin if severe long lasting nephrotic syndromeControl other CVD risk factorstarget blood pressure 125/75Thromboembolic risk Routin Prophylac
10、tic anticoagulation not recommendHigh index of suspicion for thromboemboliInfections High index of suspicionAntipneumococcal and influenza vaccinationsRef:Up to date online 17.3.41Minimal change disease-Approximately 80%of adults with MCD respond to prednisone-Failure to respond may reflect an error
11、 in diagnosis;MCD is most commonly confused with early FSGS-Treatment with cytotoxic agents may be indicated in patients who are considered:A-steroid dependent relapse while on corticosteroid therapy or requirement for continuation of steroids to maintain remission B-steroid resistant No remission w
12、ith using of steroid C-frequent relapsers 3 relapses/Y42Membranous Nephropathy Because of the generally good outcome,treatment usually is reserved for patients with poor prognostic factors:(age 50,male gender,hypertension,reduced GFR,proteinuria 10 g/d,or marked interstitial fibrosis on renal biopsy
13、)or severe symptomatic nephrotic syndrome Treatment options include high-dose alternate-day glucocorticoids in conjunction with a cytotoxic agent(e.g.,chlorambucil or cyclophosphamide for 6-12 months and in nonresponders,cyclosporine for 12 months43Focal Segmental Glomerulosclerosis PrednisoneIt:not
14、 proven to be effective May reduce proteinuria and slow progression to ESRD.Resistant cases may respond to a combination of glucocorticoids and cytotoxic agents.44Membranoproliferative Glomerulonephropathy treatment has not been shown to improve disease-free survival the use of corticosteroids in ch
15、ildren likely stabilizes disease.HCV-associated MPGN may improve with successful antiviral therapy1-Immunosuppressive treatment for idiopathic membranous nephropathy in adults with nephrotic syndrome.Cochrane Database Syst Rev.2004 2-Interventions for minimal change disease in adults with nephrotic
16、syndrome.Cochrane Database of Systematic Reviews 2008 Remains controversial with no proven benefit Cochrane reviews on the treatment of nephrotic syndrome in adults found:-weak benefit for disease remission and proteinuria in persons with membranous nephropathy -no benefit for mortality or need for
17、dialysis with corticosteroid therapy for membranous nephropathy or minimal change disease 46 It is more clearly established that children respond well to corticosteroid treatment.Classically,minimal change disease responds better to corticosteroids than FSGS.Corticosteroid therapy for nephrotic synd
18、rome in children.Cochrane Database Syst Rev.200947Diabetic NephropathyTreatment involves aggressive glucose control and aggressive BP control with ACE inhibitors or ARBs or both Up to date Washington manual Nephrotic Syndrome in Adults:Diagnosis and Management.Am Fam Physician.2009;80(10):1129-1134
19、Management of patients with nephrotic syndrome.Swissmedwkly 2009;139(29-30):416-422Corticosteroid therapy for nephrotic syndrome in children.Cochrane Database Syst Rev.2009 Interventions for minimal change disease in adults with nephrotic syndrome.Cochrane Database of Systematic Reviews 2008 Immunosuppressive treatment for idiopathic membranous nephropathy in adults with nephrotic syndrome.Cochrane Database Syst Rev.2004 Knowledge of renal histology alters patient management in over 40 percent of patients.Nephrol Dial Transplant 1994;9:1255.
