Kidney biopsy findings in Alport syndrome

What are the characteristic kidney biopsy findings in Alport syndrome?

The pathognomonic kidney biopsy finding in Alport syndrome is diffuse thickening of the GBM with splitting of the lamina densa, giving a basket-weave appearance on electron microscopy. In young patients, both thickening and thinning of the GBM may be present, causing difficulty in distinguishing the early stages of Alport syndrome from TBMN.

Light microscopy shows segmental or global glomerulosclerosis, interstitial fibrosis, and tubular atrophy.

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