Proposal of podocytic infolding XXXX as a new disease entity: a review of 25 cases from nationwide research in Japan.

@entity1 @entity644 @entity8 @entity1988 @entity244 @entity1332 @entity631 @entity1413
BACKGROUND: A rare and peculiar @entity8 has begun to be recognized in Japan. The Japanese Society of Nephrology has established a research working group and has collected cases from all over Japan in an attempt to understand the complete spectrum of this @entity8 . METHOD: The diagnostic criterion, which was needed to collect the cases, was proposed as a @entity8 showing microspheres or microtubular structures or both associated with podocytic infolding into the glomerular basement membrane (GBM) on electron microscopy. The lesion shows a non-argentaffin hole in the GBM with periodic acid @entity1332 staining and is similar to @entity631 . RESULTS: Twenty-five cases were collected from 17 institutions. @entity1 were 20-69 years old (19 @entity1 , 6 @entity1 ). Seventeen @entity1 also had collagen diseases such as @entity1988 and @entity244 . All @entity1 had @entity644 . @entity644 showed a remission in 15 of 23 @entity1 within 12 months, but @entity644 remained higher than 1.0 g/day in five @entity1 despite different types of therapy. Podocytic infolding including microspheres showed either positive or negative staining for immunoglobulins. Cluster formation of microspheres was found in 4 of 17 @entity1 with @entity1413 , and in five out eight @entity1 without @entity1413 . Electron-dense deposits in the GBM were also found in 6 of 17 @entity1 with @entity1413 but were not found in eight @entity1 without @entity1413 . CONCLUSION: Some @entity1 might have a subtype of @entity1988 , class V, or @entity631 . However, we propose a new disease entity, podocytic infolding @entity8 , as a common basis of all 25 @entity1 , because we suspect that microspheres or microtubular structures or both can be derived from podocytic infolding.
