Cytomegalovirus-related congenital nephrotic syndrome with diffuse mesangial sclerosis
The glomeruli show a constellation of findings, including a diffuse form with mesangial sclerosis and uniform thickening of capillary loops, nodular lesions recognized as KW lesions, exudative or segmental hyalinosis lesions, and the subcapsular capsular drops.
Electron microscopy findings include prominent mesangial sclerosis, GBM thickening, variable effacement of foot processes, thickening of TBMs, absence of fibrils, or electron-dense immune deposits.
Two-year-old Lucia suffers from a rare and incurable kidney condition called diffuse mesangial sclerosis
and has been treated at the RVI.
If glomerular alterations, such as crescents, fibrinoid necrosis, GBM abnormalities (eg, thickening, "spike" formation, or double contours), mesangial sclerosis or mesangial hypercellularity (>2 mesangial cells per mesangial region away from the vascular pole in a 2-[micro]m-thick section), infiltration by inflammatory cells, or endocapillary proliferation, are present, direct IF (21,22) and/or EM (23,24) should be performed using formalin-fixed, paraffin-embedded tissue, albeit with generally less sensitivity for IF microscopy and processing/preservation artifact for EM.
More advanced cases develop nodular mesangial sclerosis (or nodular glomerulosclerosis), also known as Kimmelstiel-Wilson nodules (Figure 1, B).
Lucia Castro-Quiroga, two, suffers from a condition called Diffuse Mesangial Sclerosis
, and would normally require a transplant.
A recently proposed taxonomy of podocytopathies identifies 4 patterns of glomerular injury that appear to result from these 4 pathways that injured podocytes may take: (1) minimal change nephropathy (MCN), if the number of podocytes per glomerulus is unchanged; (2) focal segmental glomerulosclerosis (FSGS), if there is podocytopenia; (3) diffuse mesangial sclerosis (DMS), where low proliferative index has been described; or (4) collapsing glomerulopathy (CG), if there is marked proliferation (Figures 1 and 2).
In fact, ectopic expression of Notch intracellular domain in embryonic podocytes of transgenic mice leads to dedifferentiation and reentry of the cell cycle (proliferation), with a resulting pattern of glomerular injury resembling diffuse mesangial sclerosis.
31) Mice null for CD2AP develop proteinuria and mesangial sclerosis and proliferation.
She suffers from an extremely rare and incurable kidney condition called Diffuse Mesangial Sclerosis
Victor Castro-Quiroga has become a champion charity fundraiser since his daughter was diagnosed with the rare and incurable kidney condition Diffuse Mesangial Sclerosis