Mutations in sphingosine-1-phosphate lyase cause nephrosis ...
Nephrotic Syndrome Dr. Raid Jastania. Causes Minimal Change disease (lipoid nephrosis) Membranous...
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Transcript of Nephrotic Syndrome Dr. Raid Jastania. Causes Minimal Change disease (lipoid nephrosis) Membranous...
Causes
• Minimal Change disease (lipoid nephrosis)
• Membranous glomerulonephritis
• Focal segmental glomerulosclerosis
• Membranoproliferative glomerulonephritis
Minimal Change GN
Focal Segmental
GN
Membranous GN
Membrano-proliferative GN
Good Prognosis
Bad Prognosis
Minimal Change Disease
• Pathogenesis:
• Disorder of T-cells
• Suspected mediators of epithelial cell injury:– IL-8, TNF
• Nephrin gene mutation
Minimal Change Disease
• Electron Microscopy:
–Loss of Foot Processes of epithelial cells
–Uniform and Diffuse
Minimal Change Disease
• The changes are reversible
• 90% respond to steroid
• May recur
• 5% progress to CRF
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)
Membranous Glomerulonephritis
• Pathogenesis
• Immune complex
–Mostly: In-situ
–Few: Circulating
• Complement activation (MAC)
Minimal Change GN
Focal Segmental
GN
Membranous GN
Membrano-proliferative GN
Good Prognosis
Bad Prognosis
Focal Segmental Glomerulosclerosis
• Primary or Secondary
»1. Infections (HIV)
»2. Drugs/Toxins (Heroin)
»3. Inherited
»4. Progress from other GN
Focal Segmental Glomerulosclerosis
• Pathogenesis:
• Relation to Minimal change GN
• Progression from other GN
• Ablation nephropathy
Focal Segmental Glomerulosclerosis
• Electron Microscopy:
• Not specific
• Deposits in areas of hyalinosis
• Epithelial cell detachment
Focal Segmental Glomerulosclerosis
• 50% progress to CRF
• Recurrence in transplant
• Collapsing FSGS
Membranoproliferative Glomerulonephritis
• Light Microscopy:
• Thick GBM
• Tram-Track
• Large Glomeruli
• Mesangial proliferation
Membranoproliferative Glomerulonephritis
• Electron Microscopy:
• Type I: sub-endothelial deposits
• Type II: dense-deposit disease (within GBM)
Membranoproliferative Glomerulonephritis
• Immuno Fluorescence:
• Type I: Ig, Comp.
• Type II: C3 only, may be Ig