26-03-2024 | Alport Syndrome | Nephrology Zebra
An unusual macroscopic aspect of a kidney biopsy: a nephrology quiz
Published in: Journal of Nephrology
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A 24-year-old woman of Iraqi descent was referred to a nephrologist because of the incidental finding of chronic kidney disease stage 3 (estimated glomerular filtration rate 58 ml/min/1.73 m2, CKD-EPI) with microscopic hematuria and proteinuria (urinary protein-creatinine ratio of 4440 mg/g), without nephrotic syndrome. Her past medical history included bilateral sensorineural hearing loss since childhood and a family history of distant consanguinity. A kidney biopsy was performed (Fig. 1).-
The macroscopic aspect of the biopsy is provided in Fig. 1A. What is the most probable cause of this distinct variegated appearance?
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Which kidney symptom reflects this macroscopic aspect of the biopsy?
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What diseases are associated with this finding?
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Besides foam cells, histopathological examination showed two glomeruli out of four with focal segmental glomerulosclerosis, while the other two were unremarkable (Fig. 1D). Vessels were normal. Immunofluorescence study showed no specific deposits. Electron microscopy was not available. What diagnosis should be ruled out here?
Fig. 1
Interstitial foam cells in a kidney biopsy of Alport syndrome. A Two yellowish flecks are macroscopically seen in the kidney core biopsy. B Within the kidney parenchyma, two foci of interstitial foamy macrophages are observed, exactly matching the macroscopic yellowish flecks. CD68 immunostaining confirmed and highlighted the histiocytic nature of the infiltrate (original magnification × 10). C Dense interstitial infiltrate by foam cells is present (Masson’s trichrome, original magnification × 200). D One lesion of focal segmental glomerulosclerosis is seen (yellow arrow), together with nearby interstitial foam cells (blue arrow, Masson’s trichrome, original magnification × 400)
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