The pathology of the kidney in the Alport syndrome

The histologic picture in the kidney in Alport syndrome varies (later lesions resemble glomerulonephritis, pyelonephritis, or interstitial nephritis), and the site of the initial renal lesion is uncertain. We have observed marked characteristic ultrastructural changes in the basement membranes of glomerular capillaries, even when few abnormalities were seen in the kidney by light microscopy. Their cause is unknown. There was no significant evidence that immune complexes, fibrin, or viruses were factors in their pathogenesis.