腎病綜合征英文課件

1、腎病綜合征英文The outlinesDefinition of Nephrotic syndromeEtiologyPathogenesisClinical pictureDiagnostic workupPathological pictureComplicationManagementsWhat is Nephrotic syndrome?proteinuria (3.5g/day), hypoalbuminemia (3,5 g/day (nephrotic-range proteinuria)2nd Goal: to seek a possible causeThe history

2、and physical examination Systemic diseaseSerologic studies (ANA), 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.3rd Goal: To assess renal functionBUN, c

3、reatinine, creatinin clearnce.bicarbonates,chloride 4th Goal: to identify biochemical disorders related to the nephrotic state. serum albumin, serum proteins, calcium, Lipid profile, Coagulation testsIndication of Renal BiobsyAdultsRenal biopsy is mandatory for every nephrotic patient except the dia

4、betic 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 performed only for glucocorticoid-resistant disease.-Management of patients with nephrotic synd

5、rome. 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.The indications for renal biopsy in diabetic patients10% of nephrotic syndrome cases in diabetes are due to other renal diseases *P

6、resence 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 2009;139(29-30):416-422.Normal

7、 Kidney biopsyMinimal change DiseaseMembranous NephropathyFocal Segmental Glomerulosclerosis Membranoproliferative Glomerulonephritis 36Renal biopsy resultsHistologic PatternKey Pathologic FeaturesMCDLM NormalEMFoot process fusionMNLMThickening of the GBMEMSubepithelial deposits of IgG FSGSLMFocal a

8、nd segmental sclerosis of glomeruliEMFocal or diffuse foot process effacement.MPGNLMthickening of all capillary walls +cellular proliferation EMThe double-contour or tram-track appearance represents interposition of mesangial cell with the GBM -subendothelial (type I) or intramembranous (type II) CO

9、MPLICATIONSDue to loss of proteins in the urineDue to oncotic pressureImmunoglobulinsusceptibility to infectionantithrombin III and proteins C and SThromboembolismvit Dbinding protein vit D deficiencyTransferrinIron deficiency anemiaHyperlipidaemiaHypovolemia Acute renal failureAnasarcarisk of cellu

10、litis, bacterial peritonitis with ascites ,large pleural effusions or pulmonary edema38TreatmentManagement of symptomsOedema Low salt dietDiureticsserial measurement of body weightProteinuria ACE inhibitors or ARBsHypoalbuminaemiaHigh protein diet not indicated0.81 g/kg/dayRef: Up to date online 17.

11、3.Management of complicationHyperlipidaemia Regular Lipid profileStatin if severe long lasting nephrotic syndromeControl other CVD risk factorstarget blood pressure 125/75Thromboembolic risk Routin Prophylactic anticoagulation not recommendHigh index of suspicion for thromboemboliInfections High ind

12、ex of suspicionAntipneumococcal and influenza vaccinationsRef: Up to date online 17.3.41Disease-Specific TherapyMinimal change diseaseApproximately 80% of adults with MCD respond to prednisoneFailure to respond may reflect an error in diagnosis; MCD is most commonly confused with early FSGSTreatment

13、 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 with using of steroid C-frequent relapsers 3 relapses/Y42Disease-Spe

14、cific TherapyMembranous NephropathyBecause 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) or severe symptomatic nephrotic sy

15、ndromeTreatment 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 months43Disease-Specific TherapyFocal Segmental GlomerulosclerosisPrednisoneIt : not proven

16、to be effectiveMay reduce proteinuria and slow progression to ESRD.Resistant cases may respond to a combination of glucocorticoids and cytotoxic agents.44Disease-Specific TherapyMembranoproliferative Glomerulonephropathytreatment has not been shown to improve disease-free survivalthe use of corticos

17、teroids in children 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

18、 with nephrotic syndrome. Cochrane Database of Systematic Reviews 2008Corticosteroids For AdultsRemains controversial with no proven benefitCochrane reviews on the treatment of nephrotic syndrome in adults found: -weak benefit for disease remission and proteinuria in persons with membranous nephropa

19、thy -no benefit for mortality or need for dialysis with corticosteroid therapy for membranous nephropathy or minimal change disease 46Corticosteroids For ChildrenIt is more clearly established that children respond well to corticosteroid treatment. Classically, minimal change disease responds better

20、 to corticosteroids than FSGS.Corticosteroid therapy for nephrotic syndrome in children. Cochrane Database Syst Rev. 200947Disease-Specific TherapyDiabetic NephropathyTreatment involves aggressive glucose control and aggressive BP control with ACE inhibitors or ARBs or bothReferences Up to date Washington