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Linear IgG deposition on light microscopy
2%
6/269
IgA-immune complexes in the mesangium on electron microscopy
9%
23/269
“Tram-track” appearance on light microscopy
4%
11/269
Subepithelial deposits with “spike and dome” appearance on electron microscopy
12/269
Podocyte effacement on electron microscopy
78%
211/269
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The clinical scenario describes a child who is suffering from nephrotic syndrome secondary to minimal change disease (MCD). The pathognomic histologic finding is podocyte effacement on electron microscopy. MCD is the most common cause of nephrotic syndrome in children. While the pathogenesis of minimal change disease is not fully understood, the functional deficit stems from loss of negative charge on the glomerular basement membrane that usually repels negative serum proteins such as albumin and transferrin. Because the filtration of only certain proteins is increased, MCD is considered a "selective" glomerulonephropathy. Increased excretion of protein in the urine results in decreased plasma oncotic pressure and peripheral edema. Loghman-Adham et al addresses the qualitative and quantitative evaluation of proteinuria in children. They report "a rapid but qualitative assessment of proteinuria can be made using dipstick or sulfosalicylic acid methods. More precise quantitation is obtained by measuring protein excretion in 24-hour urine samples or by calculating the protein/creatinine ratio in random urine samples." Lahdenkari et al. studied the characteristics of podocytes that permit their function as a filtration barrier for the kidneys. In MCD they found that “in proteinuric kidneys, the podocyte slit pore density was decreased by 69 to 80% and up to half of the slits were so "tight" that no visible space between foot processes was seen; thus, the filtration surface area between podocytes was dramatically reduced”. Illustration A shows an electron microscopy of podocyte effacement in a patient with MCD. Incorrect Answers: Answer 1: Linear IgG deposition is characteristic of Goodpasture's Syndrome. Answer 2: IgA-IC deposition is characteristic of Berger's disease, a nephritic syndrome. Answer 3: "Tram-track" appearance on LM is characteristic of membranoproliferative glomerulonephritis. Answer 4: Subepithelial deposits with a "spike and dome" appearance on EM is characteristic of diffuse membraneous glomerulopathy, the most common cause of adult nephrotic syndrome. Bullet Summary: Minimal change disease is characterized by podocyte effacement on electron microscopy.
3.7
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