Heparan sulfate-proteoglycan (HS-PG) anionic sites located in the glomerular basement membrane (GBM) are thought to contribute to glomerular permselectivity in man. The number and distribution of HS-PG anionic sites in the GBM and mesangial matrix of seven normal human kidneys and three kidneys from children with congenital nephrotic syndrome (CNS) were evaluated by ex vivo perfusion of polyethyleneimine (PEI; Mr 40,000 to 60,000). In the normal kidneys lamina rara externa (LRE) anionic sites (21.8 +/- 2.4 per 1000-nm actual GBM length) were well labeled and similar to those obtained by immersion staining of fixed tissue with Mr 1200 PEI. Lamina rara interna (LRI) anionic site number (22.0 +/- 2.6 per 1000-nm GBM) and appearance were better demonstrated by PEI perfusion than by the immersion technique. PEI perfusion also demonstrated regularly arranged (60 to 120 nm apart) mesangial matrix anionic sites (20- to 30-nm diameter) at 4.09 +/- 0.59 x 10(3) sites per nm2 matrix. PEI perfusion of three kidneys from children with CNS demonstrated decreased (16.3 +/- 1.9 per 1000-nm GBM) LRE anionic sites and normal LRI anionic sites (22.0 +/- 3.5 per 1000-nm GBM). Mesangial volume was increased, and mesangial anionic sites were less frequent (3.24 +/- 0.42 x 10(3) per nm2 matrix) and irregular in size in the children with CNS.(ABSTRACT TRUNCATED AT 250 WORDS)
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