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Nephrotic Syndrome

Nephrotic Syndrome. Dr C arol Inward Consultant Paediatric Nephrologist NPPG Conference Bristol 2011. What Is Nephrotic Syndrome?. Causes. Primary Minimal Change Disease Focal Segmental Glomerulosclerosis Mesangioproliferative glomerulonephritis IgA Nephropathy. Secondary

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Nephrotic Syndrome

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  1. Nephrotic Syndrome Dr Carol Inward Consultant Paediatric Nephrologist NPPG Conference Bristol 2011

  2. What Is Nephrotic Syndrome?

  3. Causes • Primary • Minimal Change Disease • Focal Segmental Glomerulosclerosis • Mesangioproliferative glomerulonephritis • IgA Nephropathy • Secondary • HenochSchonleinPupura • SLE • Hepatitis B&C • HIV • Genetic • Mutations in genes coding for podocyte proteins

  4. Epidemiology • Incidence 2-7 per 100 000 population <16 yrs • Most commonly presents in pre-school children • MCNS 75% of cases overall & 93% who respond to steroids ( but only 20-30% of adolescents) • >60% will have a relapsing course • 44% will be relapse free 1 year from diagnosis • 69% relapse free after 5 years • 84% relapse free after 10 years

  5. Georgia 2001 1st presentation with NS age 3yrs Prednisolone 60mg/m2 for 4 weeks 40 mg/m2 alt days for 4 weeks Relapses on withdrawal of steroid & then on low dose alternate day prednisolone Referred for specialist opinion

  6. Georgia – 2nd line • Cyclophoshamide 3mg/kg over 8 weeks • Weekly monitoring of FBC • Continued relapses→ high dose oral prednisolone

  7. Georgia – 3rd line • Feb 2002 Cyclosporin → seizures • IV antibiotics, ganciclovir & phenobarbitone

  8. What are we treating? • Minimal Change Disease

  9. Further Strategies • Low dose alternate day prednisolone and levamisole • Chlorambucil • Mycofenolate

  10. Progress • Prolonged remission allowing withdrawal of prednisolone • Mycophenolate withdrawn in 2009 • Has had one relapse since then • Discharged from hospital follow up this year as relapse free off treatment for 12 months

  11. What causes Minimal Change Nephrotic Syndrome? • Disorder of the immune system? • Response to immunosuppressives • Remission during infection with measles virus • Response to plasma exchange • Experimental induction of nephrotic syndrome in animals by infusion of plasma from patients in relapse.

  12. Why use all these toxic medicines? • Uncontrolled oedema→ respiratory compromise, skin breakdown, cellulitis • Circulatory compromise → AKI • Prothrombotic state → risk of thromboembolism • Loss of immune mediators →sepsis • Untreated mortality 50% - treated2-5%

  13. Summary • Minimal Change Nephrotic Syndrome • Good prognosis but potentially life threatening • Immunosuppressive strategies are effective but may lead to significant complications

  14. Welcome to Bristol

  15. Nephrotic Syndrome Dr Carol Inward Consultant Paediatric Nephrologist NPPG Conference Bristol 2011

  16. What Is Nephrotic Syndrome?

  17. Causes • Primary • Minimal Change Disease • Focal Segmental Glomerulosclerosis • Mesangioproliferative glomerulonephritis • IgA Nephropathy • Secondary • HenochSchonleinPupura • SLE • Hepatitis B&C • HIV • Genetic • Mutations in genes coding for podocyte proteins

  18. Epidemiology • Incidence 2-7 per 100 000 population <16 yrs • Most commonly presents in pre-school children • MCNS 75% of cases overall & 93% who respond to steroids ( but only 20-30% of adolescents) • >60% will have a relapsing course • 44% will be relapse free 1 year from diagnosis • 69% relapse free after 5 years • 84% relapse free after 10 years

  19. Georgia 2001 1st presentation with NS age 3yrs Prednisolone 60mg/m2 for 4 weeks 40 mg/m2 alt days for 4 weeks Relapses on withdrawal of steroid & then on low dose alternate day prednisolone Referred for specialist opinion

  20. Georgia – 2nd line • Cyclophoshamide 3mg/kg over 8 weeks • Weekly monitoring of FBC • Continued relapses→ high dose oral prednisolone

  21. Georgia – 3rd line • Feb 2002 Cyclosporin → seizures • IV antibiotics, ganciclovir & phenobarbitone

  22. What are we treating? • Minimal Change Disease

  23. Further Strategies • Low dose alternate day prednisolone and levamisole • Chlorambucil • Mycofenolate

  24. Progress • Prolonged remission allowing withdrawal of prednisolone • Mycophenolate withdrawn in 2009 • Has had one relapse since then • Discharged from hospital follow up this year as relapse free off treatment for 12 months

  25. What causes Minimal Change Nephrotic Syndrome? • Disorder of the immune system? • Response to immunosuppressives • Remission during infection with measles virus • Response to plasma exchange • Experimental induction of nephrotic syndrome in animals by infusion of plasma from patients in relapse.

  26. Why use all these toxic medicines? • Uncontrolled oedema→ respiratory compromise, skin breakdown, cellulitis • Circulatory compromise → AKI • Prothrombotic state → risk of thromboembolism • Loss of immune mediators →sepsis • Untreated mortality 50% - treated2-5%

  27. Summary • Minimal Change Nephrotic Syndrome • Good prognosis but potentially life threatening • Immunosuppressive strategies are effective but may lead to significant complications

  28. Welcome to Bristol

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