The glomerular basal lamina in hereditary nephritis

Abstract

Characteristic ultrastructural alterations of the glomerular basal lamina have been reported in hereditary nephritis. The basal lamina is irregularly thickened and the lamina densa shows replication with a “basket weave” pattern, enclosing electron-lucent lacunae which frequently contain small dense particles. However there is controversy regarding the specificity of this lesion in hereditary nephritis. To determine the specificity, 366 renal biopsies from 310 children were studied retrospectively. Twenty-four out of 27 patients with hereditary nephritis showed the characteristic changes of the basal lamina and they were widespread in 17. Two patients with recurrent haematuria but no family history of deafness or haematuria showed similar extensive changes and are regarded as new mutant cases of hereditary nephritis. Similar changes were seen in 17 of the 281 patients with other conditions but were always localized to a few capillary loops. We conclude that a widespread “basket weave” pattern appears to be confined to hereditary nephritis and is seen in the great majority of such cases.