An 18-year-old man has persistent microscopic hematuria detected on routine testing. He has bilateral sensorineural hearing loss and anterior lenticonus. His maternal uncle had kidney failure at age 30. Electron microscopy of kidney tissue would most likely show:
- A Diffuse effacement of epithelial foot processes
- B Subendothelial immune deposits with tram-track appearance
- C Splitting and lamellation of the glomerular basement membrane ✓
- D Thinning of the glomerular basement membrane without lamellation
Correct answer: C. Splitting and lamellation of the glomerular basement membrane
Explanation
Alport syndrome results from mutations affecting the collagen IV alpha-3, alpha-4, or alpha-5 chains, classically X-linked COL4A5. The characteristic ultrastructural lesion is irregular thickening with splitting and lamellation of the lamina densa, described as a basket-weave pattern. Isolated thinning without lamellation defines thin basement membrane lesion, a benign condition that lacks extrarenal features. Foot process effacement alone is seen in minimal change disease.
Reference: Robbins and Cotran Pathologic Basis of Disease, 10th ed.
High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP
Written and medically reviewed by the StethoPrep medical team.