Congenital nephrotic syndrome in Sudan: histological and immunological features.
We report a 5-month-old Sudanese boy with a probable diagnosis of Congenital Nephrotic Syndrome. Our case had features which are neither typical of the Finnish type nor of other hereditary renal diseases. Histologically, the most striking changes were in the glomerular basement membranes which show patchy thinning, thick segments (with reduplication) and occasional low spikes. Tubules are well preserved, and no foam cells were seen. Electron microscopy showed extensive fusion of foot processes with podocyte microvilli. In parts the glomerular basement membrane shows irregular thickening and splitting, and incorporation of podocyte cytoplasm into the membrane. In other areas there is marked thinning of the basement membrane. Immunological features include a high level of IgA and IgG. To our knowledge such features have not been described before.
D001485:Basement Membrane / Q000648:ultrastructure; D006801:Humans; D007070:Immunoglobulin A / Q000032:analysis; D007074:Immunoglobulin G / Q000032:analysis; D007223:Infant; D007668:Kidney / Q000473:pathology*; D008297:Male; D008854:Microscopy, Electron; D009404:Nephrotic Syndrome / Q000151:congenital* / Q000473:pathology; D013397:Sudan
D007070:Immunoglobulin A; D007074:Immunoglobulin G