The Experts below are selected from a list of 309 Experts worldwide ranked by ideXlab platform
Richard A Sidner - One of the best experts on this subject based on the ideXlab platform.
-
resolution of recurrent Focal Segmental Glomerulosclerosis proteinuria after rituximab treatment
The New England Journal of Medicine, 2006Co-Authors: Mark D Pescovitz, B K Book, Richard A SidnerAbstract:To the Editor: Focal Segmental Glomerulosclerosis recurs in about 30 percent of patients who undergo kidney transplantation for this condition and leads to the nephrotic syndrome and accelerated graft loss.1 Cyclosporine, cyclophosphamide, plasmapheresis, protein A immunoabsorption, and mycophenolate mofetil have been variably effective.1,2 We report the case of a seven-year-old boy who presented with immediate recurrence of Focal Segmental Glomerulosclerosis after transplantation that subsequently resolved only after rituximab treatment of a transplantation-related lymphoma that occurred five months after the surgery. This child originally had biopsy-proven primary Focal Segmental Glomerulosclerosis with the nephrotic syndrome. After 4.5 years, he progressed to . . .
-
Resolution of recurrent Focal Segmental Glomerulosclerosis proteinuria after rituximab treatment.
The New England journal of medicine, 2006Co-Authors: Mark D Pescovitz, B K Book, Richard A SidnerAbstract:To the Editor: Focal Segmental Glomerulosclerosis recurs in about 30 percent of patients who undergo kidney transplantation for this condition and leads to the nephrotic syndrome and accelerated gr...
Claudio Ponticelli - One of the best experts on this subject based on the ideXlab platform.
-
Autoimmunity in Focal Segmental Glomerulosclerosis : A Long-Standing Yet Elusive Association
Frontiers in medicine, 2020Co-Authors: Manuel Alfredo Podestà, Claudio PonticelliAbstract:Focal Segmental Glomerulosclerosis (FSGS) is a histological term that describes a pathologic renal entity affecting both adults and children, with a wide array of possible underlying etiologies. Podocyte damage with scarring, the hallmark of this condition, leads to altered permeability of the glomerular barrier, which may result in massive proteinuria and relentless renal function deterioration. A definite cause of Focal Segmental Glomerulosclerosis can be confirmed in a minority of cases, while most forms have been traditionally labeled as primary or idiopathic. Despite this definition, increasing evidence indicates that primary forms are a heterogenous group rather than a single disease entity: several circulating factors that may affect glomerular permeability have been proposed as potential culprits, and both humoral and cellular immunity have been implicated in the pathogenesis of the disease. Consistently, immunosuppressive drugs are considered as the cornerstone of treatment for primary Focal Segmental Glomerulosclerosis, but response to these agents and long-term outcomes are highly variable. In this review we provide a summary of historical and recent advances on the pathogenesis of primary Focal Segmental Glomerulosclerosis, focusing on implications for its differential diagnosis and treatment.
-
Treatment of Focal Segmental Glomerulosclerosis.
Kidney international, 2010Co-Authors: Claudio Ponticelli, Richard J. GlassockAbstract:To the Editor: In his excellent review, Meyrier1 outlined the current ignorance about the pathogenesis of Focal Segmental Glomerulosclerosis (FSGS) and hoped for new treatments aimed at counteracting the unknown factor(s) responsible for FSGS rather than pursuing trials with agents interfering with the immune system (or alternatively, acting directly on the dysfunctional podocyte).
-
Treatment of Focal Segmental Glomerulosclerosis.
Current opinion in nephrology and hypertension, 2001Co-Authors: Patrizia Passerini, Claudio PonticelliAbstract:The prognosis of untreated patients with Focal Segmental Glomerulosclerosis is poor, as the disease progress to end-stage renal disease in approximately 50-70% of nephrotic patients. Although Focal Segmental Glomerulosclerosis was initially considered to be a steroid-resistant disease, several studi
Agnes B. Fogo - One of the best experts on this subject based on the ideXlab platform.
-
Minimal Change Disease and Focal Segmental Glomerulosclerosis
Nephrology Dialysis Transplantation, 2001Co-Authors: Agnes B. FogoAbstract:Minimal change disease (MCD) and Focal Segmental Glomerulosclerosis (FSGS) are both common causes of the nephrotic syndrome. Minimal change disease accounts for greater than 90% of cases of nephrotic syndrome in children, vs. 10% to 15% of adults with nephrotic syndrome (1). Focal Segmental Glomerulosclerosis has been increasing in incidence in the United States in both African Americans and in Hispanics, in both adult and pediatric populations (2–4). It is now the most common cause of nephrotic syndrome in adults in the U.S. Patients with FSGS may have hypertension and hematuria. Serologic studies, including complement levels, are typically within normal limits in both MCD and FSGS.
-
Is Focal Segmental Glomerulosclerosis really Focal? Distribution of lesions in adults and children.
Kidney International, 1995Co-Authors: Agnes B. Fogo, Alan D. Glick, Sarah L. Horn, Robert G. HornAbstract:Is Focal Segmental Glomerulosclerosis really Focal? Distribution of lesions in adults and children. The distribution of lesions of Glomerulosclerosis, whether Focal or diffuse, has important implications for pathogenesis and potential therapeutic response. Determination of Focal or diffuse nature of lesions from a single section, may, however, be misleading. We therefore evaluated the distribution of Segmental Glomerulosclerosis in patients with nephrotic syndrome and idiopathic Focal Segmental Glomerulosclerosis (FSGS) by three-dimensional analysis. From our files, we identified all such biopsies with a diagnosis established by immunofluorescence, electron microscopy, and light microscopy that had >10 glomeruli and serum creatinine
Mark D Pescovitz - One of the best experts on this subject based on the ideXlab platform.
-
resolution of recurrent Focal Segmental Glomerulosclerosis proteinuria after rituximab treatment
The New England Journal of Medicine, 2006Co-Authors: Mark D Pescovitz, B K Book, Richard A SidnerAbstract:To the Editor: Focal Segmental Glomerulosclerosis recurs in about 30 percent of patients who undergo kidney transplantation for this condition and leads to the nephrotic syndrome and accelerated graft loss.1 Cyclosporine, cyclophosphamide, plasmapheresis, protein A immunoabsorption, and mycophenolate mofetil have been variably effective.1,2 We report the case of a seven-year-old boy who presented with immediate recurrence of Focal Segmental Glomerulosclerosis after transplantation that subsequently resolved only after rituximab treatment of a transplantation-related lymphoma that occurred five months after the surgery. This child originally had biopsy-proven primary Focal Segmental Glomerulosclerosis with the nephrotic syndrome. After 4.5 years, he progressed to . . .
-
Resolution of recurrent Focal Segmental Glomerulosclerosis proteinuria after rituximab treatment.
The New England journal of medicine, 2006Co-Authors: Mark D Pescovitz, B K Book, Richard A SidnerAbstract:To the Editor: Focal Segmental Glomerulosclerosis recurs in about 30 percent of patients who undergo kidney transplantation for this condition and leads to the nephrotic syndrome and accelerated gr...
A Kanatbayeva - One of the best experts on this subject based on the ideXlab platform.
-
Focal Segmental Glomerulosclerosis variants in children with nephrotic syndrome
OA Nephrology, 2013Co-Authors: Ay Naushabayeva, Ba Abeuova, G Chingayeva, K Kabulbayev, A Nurbekova, A Nugmanova, A Smagulova, A KanatbayevaAbstract:Introduction The aim of this study was to determine pathological variants of childhood Focal Segmental Glomerulosclerosis and assess efficacy of prednisolone + methylprednisolone + cyclosporin A treatment. Materials and methods This retrospective cohort study included 134 native Kazakh children (3 months–17 years) with nephrotic syndrome hospitalised in the Nephrology Department of the Republic Children’s Clinical Hospital from 2004 to 2011. Kidney biopsy and pathological investigations were performed in 38 nephrotic syndrome patients with proven steroid resistance. Results Focal Segmental Glomerulosclerosis was confirmed in 38 (28.4%) patients (treatment group). The main Focal Segmental Glomerulosclerosis variants were glomerular tip lesions and not otherwise specified types. Historical (did not undergo kidney biopsy) controls were treated with cyclosporin A or alkylating agents. Prednisolone + methylprednisolone + cyclosporin A immunosuppressive therapy was highly effective, allowing complete remission in 88.9% patients with minimum side effects. Patients with the not otherwise specified variant (NPHS2 and WT1 mutations) did not achieve remission. Combination cyclosporin A treatment was significantly more effective than alkylating agent treatment in steroid-resistant nephrotic syndrome patients without genetic mutations. Conclusion Tip lesions are predominant in childhood steroid-resistant nephrotic syndrome. Prednisolone + methylprednisolone + cyclosporin A therapy is safe and effective for childhood Focal Segmental Glomerulosclerosis. Establishing a podocyte mutation profile is important to predict treatment outcome.