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Snapshot
  • A 67-year-old man with a history of type 2 diabetes and hypertension presents with worsening pedal edema. His diabetes has been poorly controlled and his last HbA1C was measured at 9.7 about 3 months ago. He reports no other symptoms. Physical exam reveals bilateral 2+ pitting edema to the knees bilaterally, and fundoscopy reveals cotton wool spots. A urinalysis shows 3+ protein, and serum albumin is 2.5. He is prescribed an ACE-inhibitor by his physician. (Diabetic Nephropathy) 

 

Introduction
  • Secondary nephrotic syndrome is a multisystem disease 
  • Primary nephrotic syndrome refers to disease limited to kidney
Presentation
  • Hypoalbuminemia (serum albumin of <3.0 g/dl)
  • Proteinuria > 3.5 g/day
  • Generalized edema (anasarca)
    • caused by loss of albumin / decreased oncotic pressure
  • Hyperlipidemia with lipiduria
    • low albumin stimulates liver to produce cholesterol
  • Hypercoaguable state 
 
Differential
 
Nephrotic Syndrome
Nephritic Syndrome
Chronic Disease
Proteinuria
+ + +
+ / -
+ / -
Hematuria
+ / -
+ + +
+ / -
Cells
+ / -
+ RBCs / + WBCs
+ / -
Casts
Fatty Casts
RBC and granular casts
Waxy/pigmented/granular casts
Lipids
Free fat droplets
Oval fat bodies
   

 

Evaluation
  • Labs show
    • hypoalbuminemia (serum albumin of <3.0 g/dL) 
    • proteinuria > 3.5 g/day
    • hyperlipidemia with lipiduria
    • low albumin stimulates liver to produce cholesterol
  • Definitive diagnosis made by renal biopsy


Generalized Treatment
  • Management of primary disease process causing nephrotic syndrome
     
  • Protein restriction
  • Salt restriction
  • Diuretic therapy for edema
  • HMG-CoA reductase inhibitor for hyperlipidemia
Diabetic Glomerulonephropathies
  • Most common cause of end stage renal disease in USA
  • Early manifestation is microalbuminuria
  • Biopsy shows pathognomnic Kimmelstiel-Wilson nodules
  • Treatment
    • can slow progression with
      • ace inhibitors
      • strict glycemic and hypertension control
    • transplant is only treatment after disease has progressed
HIV Glomerulonephropathies
  • Usually seen in HIV acquired through IV drug use
  • Presents with focal segmental glomerulonephritis
  • Treat HIV
Lupus Glomerulonephritis
  • Type I
    • no renal involvement
  • Type II
    • mesangial disease with focal segmental glomerular pattern
  • Type III
    • focal proliferative disease
    • treat with aggressive prednisone +/- cyclophosphamide
  • Type IV
    • most severe form
    • diffuse proliferative disease
    • presents as combination of nephritic and nephrotic disease
    • classic wire loop abnormality (image above)
    • treat with prednisone, cyclophosphamide, transplant
  • Type V
    • membranous disease
    • indistinguishable from other primary membraneous glomerulonephropathies
    • Treatment: consider prednisone
 

 

 

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