1. Nephrotic Syndrome Dr. Raid Jastania

2. Causes Minimal Change disease (lipoid nephrosis) Membranous glomerulonephritis Focal segmental glomerulosclerosis Membranoproliferative glomerulonephritis

3. Minimal Change GN Focal Segmental GN Membranous GN Membrano- proliferative GN Good Prognosis Bad Prognosis

4. Minimal Change Disease Clinical presentation

5. Minimal Change Disease Most common cause of nephrotic syndrome in children 2-3 years

6. Minimal Change Disease Pathogenesis

7. Minimal Change Disease Pathogenesis: Disorder of T-cells Suspected mediators of epithelial cell injury: –IL-8, TNF Nephrin gene mutation

8. Minimal Change Disease Light Microscopy

9. Minimal Change Disease Light Microscopy –Normal –Lipoid Nephrosis

10. Minimal Change Disease Immuno Fluorescence:

11. Minimal Change Disease Immuno Fluorescence: –Negative

12. Minimal Change Disease Electron Microscopy:

13. Minimal Change Disease Electron Microscopy: –Loss of Foot Processes of epithelial cells –Uniform and Diffuse

14. Minimal Change Disease Prognosis

15. Minimal Change Disease The changes are reversible 90% respond to steroid May recur 5% progress to CRF

16. Membranous Glomerulonephritis Clinical Presentation

17. Membranous Glomerulonephritis Nephrotic syndrome Non-selective proteinuria 30-50 years of age

18. Membranous Glomerulonephritis Primary or Secondary

19. Membranous Glomerulonephritis Primary or Secondary –85%15% »1. Infections (HepB, malaria) »2. Cancer (Lung ca, colon ca) »3. SLE »4. Gold, mercury »5. Drugs (penicillamine, capropril, NSAID’s)

20. Membranous Glomerulonephritis Pathogenesis

21. Membranous Glomerulonephritis Pathogenesis Immune complex –Mostly: In-situ –Few: Circulating Complement activation (MAC)

22. Membranous Glomerulonephritis Light microscopy:

23. Membranous Glomerulonephritis Light microscopy: Thick GBM (diffuse) Spikes

24. Membranous Glomerulonephritis Electron microscopy:

25. Membranous Glomerulonephritis Electron microscopy: Sub-epithelial deposits

26. Membranous Glomerulonephritis Immuno Fluorescence:

27. Membranous Glomerulonephritis Immuno Fluorescence: Ig, comp. deposits

28. Membranous Glomerulonephritis Prognosis

29. Membranous Glomerulonephritis Less response to steroid 40% progress to CRF

30. Minimal Change GN Focal Segmental GN Membranous GN Membrano- proliferative GN Good Prognosis Bad Prognosis

31. Focal Segmental Glomerulosclerosis Primary or Secondary

32. Focal Segmental Glomerulosclerosis Primary or Secondary »1. Infections (HIV) »2. Drugs/Toxins (Heroin) »3. Inherited »4. Progress from other GN

33. Focal Segmental Glomerulosclerosis Pathogenesis

34. Focal Segmental Glomerulosclerosis Pathogenesis: Relation to Minimal change GN Progression from other GN Ablation nephropathy

35. Focal Segmental Glomerulosclerosis Light microscopy

36. Focal Segmental Glomerulosclerosis Light microscopy: Hyalinosis/Sclerosis

37. Focal Segmental Glomerulosclerosis Immuno Fluorescence:

38. Focal Segmental Glomerulosclerosis Immuno Fluorescence: IgM, Comp.

39. Focal Segmental Glomerulosclerosis Electron Microscopy:

40. Focal Segmental Glomerulosclerosis Electron Microscopy: Not specific Deposits in areas of hyalinosis Epithelial cell detachment

41. Focal Segmental Glomerulosclerosis Prognosis

42. Focal Segmental Glomerulosclerosis 50% progress to CRF Recurrence in transplant Collapsing FSGS

43. Membranoproliferative Glomerulonephritis Clinical presentation

44. Membranoproliferative Glomerulonephritis Nephrotic, Nephritic-Nephrotic

45. Membranoproliferative Glomerulonephritis Light Microscopy:

46. Membranoproliferative Glomerulonephritis Light Microscopy: Thick GBM Tram-Track Large Glomeruli Mesangial proliferation

47. Membranoproliferative Glomerulonephritis Electron Microscopy:

48. Membranoproliferative Glomerulonephritis Electron Microscopy: Type I: sub-endothelial deposits Type II: dense-deposit disease (within GBM)

49. Membranoproliferative Glomerulonephritis Immuno Fluorescence:

50. Membranoproliferative Glomerulonephritis Immuno Fluorescence: Type I: Ig, Comp. Type II: C3 only, may be Ig

51. Membranoproliferative Glomerulonephritis Prognosis:

52. Membranoproliferative Glomerulonephritis Prognosis: Disease persists or progress to CRF

53. Minimal Change GN Focal Segmental GN Membranous GN Membrano- proliferative GN Good Prognosis Bad Prognosis