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NEPHROTIC SYNDROME

NEPHROTIC SYNDROME. Nephrotic range proteinuria, ( >40mg/m 2 /hour), (> 50mg/kg/day), urine to protein creat ratio (>2mg/mg), +3-4 on dipstick Hypoalbumenia (<2.5g/dl) Hyperlipidemia edema Prevelance 2/100000 M:F 2:1, 80% <6 years. ETIOLOGY.

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NEPHROTIC SYNDROME

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  1. NEPHROTIC SYNDROME • Nephrotic range proteinuria, (>40mg/m2/hour), (> 50mg/kg/day), urine to protein creat ratio (>2mg/mg), +3-4 on dipstick • Hypoalbumenia (<2.5g/dl) • Hyperlipidemia • edema • Prevelance 2/100000 • M:F 2:1, 80% <6 years

  2. ETIOLOGY • Primary or idiopathic(MCD,FSGS,Membranous,MPGN,Mesangial proliferation) • Congenital nephrotic syndrome (first 3months) due TORCH,Finish type • Secondary to infections,systemic diseases (HSP,SLE)

  3. Pathogenesis • Defect in GBM,slit diaphram,genetic mutations in nephrin,podocin • Circulating factor,cytokine VPF explains early recurrence • Immunological abnormality,T cell dysfunction,ass with Hodgkin lymphoma • Pathophysiolgy • Decrease Intravascular volume,urine Na <20 • Increased intravascular volume

  4. Podocytes and the slit diaphragm

  5. Histopathology • MCD:normal LM,neg IF,EM effacement of foot processes of podocytes • Mesangial proliferative,IgM nephropathy:positive IgM,C3 on IF • FSGS:juxtamedullary glomeruli • Most common is MCD. • 80% of MCD were ≤6y,compared to 50% of FSGS

  6. NORMAL

  7. Moderate FSGS Moderate FGS

  8. History and examination • Periorbital swelling mistaken as allergy • Increase in weight,abdominal distention ascitis • Scrotal and sacral edema,pleural effusion • Abdominal pain due to hypovolemia,peritonitis • Decrease in urine output • Symptoms preceded by URTI • Blood pressure normal or high

  9. Laboratory investigation • Electrolytes: low Na,low albumin and calcium • ANA,C3,C4,hepatitis B,C • Hemoglobin high,platlet high • Urine Na less than 10 • Urine analysis: proteinuria,microscopic hematuria • Urine protein/creatinine more than 2 mg/mg • Elevated cholesterol and triglycerides

  10. Course and outcome • 93 % of MCD respond to steriods by 4 weeks. • 30% single episode,70% relapse • 35-50% frequent relapses • Long term:80 % have complete remission by 8 years. • SRNS can progress to CKD

  11. When a renal biobsy is done • Age less than 1. • SRNS • ARF • Gross or persistent microscopic hematuria • Biobsy sent for LM,IF,EM

  12. TREATMENT • First episode :Steriods 2mg/kg/day,60 mg/m2/day single daily dose for 4 weeks,followed by alternate dose for 3-6 months. • Response in 10 to 14 days • Recent Cockrane metaanalysis found that treatment for 3m compared to 2m reduces risk of relapse by 30% at 12-24m • No significant diff in risk of side effects or cumulative steriod dose • With each 1 m over 2m,RR of relapse falls by 11%

  13. Treatment of relapses:2mg/kg/day till remission for 3 days,then alternate days for 2-3m. • Treatment of FR or SD SSNS: 1.Long alternate steriods for 12-18 m 2.Cyclophosphamide 3.cyclosporine 4.Levimazole 5.Mycophenolate acetate

  14. Side effects • Steriods:growth,osteopenia,cataract,cushingoid,glaucoma • Cyclosporine:hirsuitism,gum hypertrophy,HTN,reduced GFR • CPM:infertility,alopecia,leucopenia,hemmrhagic cystitis. • MMF:abdominal pain,anemia,leucopenia

  15. COMPLICATIONS • 1-Infections:losses of IgG in urine,abn T cell function,low factor B (C3 proactivator),steriod use,impaired opsonization • Encapsulated bact streptococcus pneumonia,staph,Ecoli • Primary bacterial peritonitis • Immunization against pneumococcus,varicella

  16. 2-Thromboembolism:inc clotting factors,fibrinogen,low AT3,plat aggreg,hyperviscosioty Venous,RVT,sagital sinus,veins of legs 3.ARF

  17. Supportive treatment • Salt restriction • Albumin 25% 1-2g/kg/day over 4 hours with frusemide • ACEI decrease proteinuria in SRNS

  18. CNS Finish Type • AR,Mutation in nephrin gene,codes for nephrin part of slit diaphragm • Two mutations (Finmaj,Finminor) • Born premature,large placenta • Edema at birth,severe proteinuria, very low albumin • High amnoitic AFP • GFR normal first 6-12m

  19. Diffuse Mesangial Sclerosis • AD,isolated or Denys-Drash (wilms tumor,male pseudohermaphrodite) • Proteinuria in first 3m or later in infancy,less severe than CNS • ESRD months after presentation • Mesangial sclerosis,interstitial fibrosis • Mutations of WT1

  20. Treatment • No role for steriods • Albumin infusions • Nutrition:130kcal/kg,4g/kg protein • Hypothyroidism • ACEI • Indomethacin • diuertics • Anticoagulants,prophylactic pencillin • Nephrectomy,dialysis,tranplantation

  21. Hereditary nephrotic syndrome

  22. SRNS • Mostly FSGS,genetic forms,immune mediated recur after Tx • Cyclosporine better than cyclophospamide • ACEI,diuertic to control edema. • rituximab

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