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Split glomerular basement membrane
69%
72/104
Fusion of the podocytes
12%
12/104
Birbeck granules
4%
4/104
Subepitheial deposits
7%
7/104
Subendothelial deposits
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The boy in this vignette most likely has Alport’s syndrome, as demonstrated by his sensorineural deafness in conjunction with hematuria. The classic finding of Alport syndrome on EM is alternating areas of thinned and thickened capillary loops with a split glomerular basement membrane. Alport syndrome is the most common form of hereditary nephritis. Patients with this condition eventually develop glomerulonephritis that progresses to renal failure. Some, but not all, patients develop hearing loss. A minority of patients (approximately 10%) have eye abnormalities, such as cataracts. Lenz and Avraham discuss the genetics of hereditary hearing loss, of which there are hundreds of genes encoding a large variety of proteins. Various mutations in these genes result in syndromic and non-syndromic losses, categorized by whether or not there are abnormalities outside of auditory system. These conditions vary from mild to profound losses. Haas discusses Alport syndrome and thin glomerular basement membrane nephropathy (TBMN) which are two different diseases of glomerular basement membrane. The initial presentation of both of these conditions is typically hematuria. TBMN is usually benign, while Alport syndrome, particularly the X-linked form with mutations in the alpha(5) chain of type IV collagen [alpha(5)(IV)], usually progresses to end-stage renal disease. Electron microscopy and immunohistochemistry are essential for the diagnosis of TBMN and Alport syndrome on renal biopsy. Illustration A displays a glomerular electron micrograph of a normal kidney compared to one with Alport syndrome. Incorrect Answers: Answer 2: Fusion of the podocytes on EM is characteristic of minimal change disease (MCD). Answer 3: Birbeck granules are characteristic of Langerhans cells and are seen in histiocytosis conditions. Answer 4: Subepithelial deposits are characteristic of acute post-streptococcal glomerulonephritis. Answer 5: Subendothelial deposits are characteristic of diffuse proliferative glomerulonephritis.
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