Sera from patients with collapsing focal segmental glomerulosclerosis increase albumin permeability of isolated glomeruli
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Glomerulosclerosis
Serum Albumin
Albuminuria
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Biopsies were done for 27 nephrotic children whose presentations were compatible with minimal lesion nephrotic syndrome. Percentages of glomeruli involved by focal glomerular sclerosis and its subtypes were calculated. The presence or absence of interstitial disease was noted. These histologic findings were correlated with steroid responsiveness. All patients who had greater than 5% of glomeruli involved by focal glomerular sclerosis resistant to steroids. Interstitial disease was present in ten of the 27 patients studied; eight of the ten were resistant to steroids. Steroid resistance was found to be associated not with the mere presence of focal glomerular sclerosis but rather with its extent and the presence of interstitial disease.
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Minimal change disease
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Glomerulosclerosis
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Background Glomerulosclerosis represents the final stage of glomerular injury during the course of kidney disease and can result from a primary disturbance in disorders like focal segmental glomerulosclerosis or a secondary response to tubulointerstitial disease. Overall, primary focal glomerulosclerosis (FSGS), the focus of this review, accounts for 10–20% of patients of all ages who progress to end stage kidney disease. There are no FDA approved therapeutic options that effectively prevent or delay the onset of kidney failure.Areas covered Current immunosuppressive therapy and conservative management including inhibitors of the renin-angiotensin-aldosterone axis and sodium-glucose cotransporter are reviewed. FSGS is now recognized to represent a heterogeneous entity with multiple underlying disease mechanisms. Therefore, novel approaches targeting the podocyte cytoskeleton, immunological, inflammatory, hemodynamic and metabolic pathways are highlightedExpert opinion A number of factors are driving the development of drugs to treat focal segmental glomerulosclerosis in particular and glomerulosclerosis in general including growing awareness of the burden of chronic kidney disease, improved scientific understanding of the mechanism of injury, and the development of noninvasive profiles to identify subgroups of patients with discrete mechanisms of glomerular injury.
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Focal segmental glomerulosclerosis (FSGS) is the leading cause of nephrotic syndrome in an adult worldwide. The prevalence of FSGS is estimated as being 20-30% in adults over the age of 15 years and slightly higher (30-35%) in the elderly (age > 60 years). The diagnosis solely relies on pathologic findings, which sclerosis involves some, but not all glomeruli (focal), and sclerosis affects a portion, but not the entire, glomerular tuft (segmental). The pathogenesis remains inconclusive but podocyte injury has been postulated. Even though steroid is the mainstay treatment, only 20-40% of patients are complete respond.
Glomerulosclerosis
Histopathology
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<b><i>Background:</i></b> Focal segmental glomerulosclerosis (FSGS) is a histologic pattern characterized by focal glomerular scarring, which often progresses to systemic and diffuse glomerulosclerosis. Previous studies have emphasized that the initiation of classic FSGS occurs in podocytes. The dysfunction and loss of podocytes have been associated with the development of proteinuria and the progression of various diseases. In addition, primary, secondary, and genetic FSGS are caused by different mechanisms of podocyte injury. <b><i>Summary:</i></b> The potential sources and mechanism of podocyte supplementation are the focus of our current research. Increasing attention has been paid to the role played by parietal epithelial cells (PECs) during the progression of FSGS. PECs are not only the primary influencing factors in glomerulosclerosis lesions but also have repair abilities, which remain a focus of debate. Notably, other resident glomerular cells also play significant roles in the progression of this disease. <b><i>Key Message:</i></b> In this review, we focus on the mechanism of scarring in FSGS and discuss current and potential therapeutic strategies.
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Membranous Nephropathy
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This biopsy study exploits the unique opportunity offered by long-surviving human renal allografts to analyze the effects of nephronopenia-induced glomerular hypertrophy on the association of focal glomerulosclerosis. Methods include glomerular morphometry and analysis of variance. Only allografts with focal glomerulosclerosis detected after 3 yr posttransplantation (Group TxFGS > 2 yr) have a group mean glomerular diameter (203.1 microns +/- SE (mean) 10.36) significantly larger (P < 0.05) than that of allografts biopsied beyond 2 yr posttransplantation but without focal glomerulosclerosis (Group No TxFGS > 2 yr; 158.3 microns +/- SE (mean) 8.32), allografts biopsied within 2 yr of transplantation with (Group TxFGS < 2 yr; 165.3 microns +/- SE (mean) 5.35) or without (Group NoTxFGS < 2 yr; 155.8 microns +/- SE (mean) 5.53) focal glomerulosclerosis, or allograft diameters at transplantation (Group CTx; 148.3 microns +/- SE (mean) 5.66). However, the increase is approximately 37% over that at transplantation, significantly less than the increase of approximately 70% attained by congenitally nephronopenic native kidneys (P < 0.50) with focal glomerulosclerosis (Group CNP; 264.4 microns +/- SE (mean) 11.01) over their controls (Group CN; 154.8 microns +/- SE (mean) 4.46). This observation suggests that the nephronopenic allograft has a curtailed ability for glomerular hypertrophy when compared with congenitally nephronopenic native kidneys and may explain the association of focal glomerulosclerosis in long-surviving renal homografts at a significantly smaller glomerular diameter. Although smaller, this appears to be the maximal glomerular dimension attained by long-surviving nephronopenic homografts in the absence of intrinsic glomerular disease (excluding focal glomerulosclerosis). Thus, these results are in accord with and offer further support for the general hypothesis that focal glomerulosclerosis develops in maximally hypertrophied glomeruli.
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Focal segmental glomerulosclerosis (FSGS) is a glomerular histopathological finding characterized by segmental (affecting part of the flocculus) and focal (affecting only certain glomeruli) sclerosis. It is severe kidney disease with a complex pathophysiology.
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