肾病综合征英文

Nephrotic Syndrome,The outlines,Definition of Nephrotic syndromeEtiologyPathogenesisClinical pictureDiagnostic workupPathological pictureComplicationManagements,What is Nephrotic syndrome?,proteinuria (3.5g/day), hypoalbuminemia (3,5 g/day (nephrotic-range proteinuria),2nd Goal: to seek a possible cause,The history 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 function,BUN, creatinine, creatinin clearnce.bicarbonates,chloride,4th Goal: to identify biochemical disorders related to the nephrotic state.,serum albumin, serum proteins, calcium, Lipid profile, Coagulation tests,Indication of Renal Biobsy,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 performed 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.,The indications for renal biopsy in diabetic patients,10% of nephrotic syndrome cases in diabetes 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 2009;139(29-30):416-422.,Normal Kidney biopsy,Minimal change Disease,Membranous Nephropathy,Focal Segmental Glomerulosclerosis,Membranoproliferative Glomerulonephritis,36,Renal biopsy results,COMPLICATIONS,Due to loss of proteins in the urine,Due to oncotic pressure,Immunoglobulinsusceptibility to infectionantithrombin III and proteins C and SThromboembolismvit Dbinding protein vit D deficiencyTransferrinIron deficiency anemia,HyperlipidaemiaHypovolemia Acute renal failureAnasarcarisk of cellulitis, bacterial peritonitis with ascites ,large pleural effusions or pulmonary edema,38,Treatment,Management of symptoms,Oedema Low salt dietDiureticsserial measurement of body weightProteinuria ACE inhibitors or ARBsHypoalbuminaemiaHigh protein diet not indicated0.81 g/kg/day,Ref: Up to date online 17.3.,Management of complication,Hyperlipidaemia 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 index of suspicionAntipneumococcal and influenza vaccinations,Ref: Up to date online 17.3.,41,Disease-Specific Therapy,Minimal 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 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/Y,42,Disease-Specific Therapy,Membranous 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 syndromeTreatment 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 months,43,Disease-Specific Therapy,Focal Segmental GlomerulosclerosisPrednisoneIt : not proven to be effectiveMay reduce proteinuria and slow progression to ESRD.Resistant cases may respond to a combination of glucocorticoids and cytotoxic agents.,44,Disease-Specific Therapy,Membranoproliferative Glomerulonephropathytreatment has not been shown to improve disease-free survivalthe use of corticosteroids in children likely stabilizes disease. HCV-associated MPGN may improve with successful antiviral therapy,1-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 syndrome. Cochrane Database of Systematic Reviews 2008,Corticosteroids For Adults,Remains 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 nephropathy -no benefit for mortality or need for dialysis with corticosteroid therapy for membranous nephropathy or minimal change disease,46,Corticosteroids For Children,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 syndrome in children. Cochrane Database Syst Rev. 2009,47,Disease-Specific Therapy,Diabetic NephropathyTreatment involves aggressive glucose control and aggressive BP control with ACE inhibitors or ARBs or both,References,Up to date Washington manual Nephrotic Syndrome in Adults: Dia