An 18-year-old man has persistent microscopic haematuria detected on screening. He has bilateral sensorineural hearing loss and a cone-shaped protrusion of the lens noted by the ophthalmologist. His maternal uncle required dialysis at age 30. What will electron microscopy of his kidney biopsy most likely show?
- A Diffuse effacement of epithelial podocyte foot processes
- B Irregular thickening and splitting of the glomerular basement membrane with a basket-weave appearance ✓
- C Subepithelial immune deposits with spike formation on silver stain
- D Thinning of the glomerular basement membrane to half the normal thickness without other change
Explanation
Alport syndrome results from defective type IV collagen, usually X-linked COL4A5, producing the triad of hereditary nephritis, sensorineural deafness, and ocular lesions including anterior lenticonus. Electron microscopy shows alternating thickening, thinning, and lamellation of the glomerular basement membrane described as a basket-weave pattern. Foot process effacement is the lesion of minimal change disease, spikes indicate membranous nephropathy, and isolated uniform thinning describes thin basement membrane lesion, a benign familial haematuria that lacks extrarenal features and family history of renal failure.
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.