• Differential diagnosis of nephrotic syndrome in the US:
  
  
  • Idiopathic causes
    • Minimal change disease - most common cause in children, the cause in 10-15% of adults, though more common in those with autoimmune disease
    • Focal segmental glomerulosclerosis - most common in African-Americans
    • Membranous nephropathy - most common in Caucasians
  • Secondary causes
    • Diabetes - most common cause of nephrotic syndrome in the United States
    • SLE - can cause MPGN
    • Collagen diseases
    • Infection-related causes - HIV, HBV, HCV, malaria
    • Medications - NSAIDS most common
    • Amyloidosis/dysproteinemias
    • Thrombotic microangiopathies
  
  

  Posted 06/30/16 09:15:26 AM by Anna Krigel

• Check for compliments!! Only four causes of nephrotic syndrome that result in hypocomplimentemia:
  1. Cryoglobulinemia
  2. SLE
  3. Post-infectious
  4. Idiopathic (C3 nephropathy)
  
  

  Posted 06/30/16 09:17:53 AM by Anna Krigel

• Classic signs and symptoms of nephrotic syndrome:
  • Proteinuria
  • Hypoalbuminemia
  • Edema
  • Hyperlipidemia
  
  

  Posted 06/30/16 09:23:39 AM by Anna Krigel

• Amyloidosis - accounts for 4-17% of cases of seemingly idiopathic nephrotic syndrome
  • Two major types
    • AL or primary amyloid - monoclonal light chains form the amyloid fibrils
    • AA or secondary amyloid - acute phase reactant serum amyloid A forms the amyloid fibrils
      • associated with chronic inflammatory diseases such as RA/juvenile RA, spondyloarthropathies/psoriasis, IBD, FMF, or idiopathic causes
  • Detected on biopsy using congo red dye for staining - will see apple-green birefringence
  
  

  Posted 06/30/16 10:33:36 AM by Anna Krigel

• For further reading...

  Natural History and Outcome in AA  Amyloidosis
  Molecular Mechanisms of Amyloidosis
  Amyloidosis in Inflammatory Bowel Disease
  
  

  Posted 06/30/16 10:57:42 AM by Anna Krigel

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