The Experts below are selected from a list of 20262 Experts worldwide ranked by ideXlab platform
Gerald B. Appel - One of the best experts on this subject based on the ideXlab platform.
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renal vein thrombosis Nephrotic Syndrome and systemic lupus erythematosus
Annals of Internal Medicine, 2020Co-Authors: Gerald B. Appel, Gail S. Williams, Jay I. Meltzer, Conrad L. PiraniAbstract:Abstract We report here four cases of systemic lupus erythematosus associated with the Nephrotic Syndrome and renal vein thrombosis and review six similar cases previously noted in the literature. ...
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treatment of Nephrotic Syndrome with adrenocorticotropic hormone acth gel
Drug Design Development and Therapy, 2011Co-Authors: Andrew S Bomback, James A Tumlin, Joel Baranski, James E Bourdeau, Anatole Besarab, Alice S Appel, Jai Radhakrishnan, Gerald B. AppelAbstract:Purpose: A synthetic adrenocorticotropin (ACTH) analog has shown efficacy in Europe as primary and secondary therapy for Nephrotic Syndrome, but there is no published experience using the natural, highly purified ACTH gel formulation, available in the United States, for Nephrotic Syndrome. We therefore investigated the use of ACTH gel for Nephrotic Syndrome in the United States.
Conrad L. Pirani - One of the best experts on this subject based on the ideXlab platform.
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renal vein thrombosis Nephrotic Syndrome and systemic lupus erythematosus
Annals of Internal Medicine, 2020Co-Authors: Gerald B. Appel, Gail S. Williams, Jay I. Meltzer, Conrad L. PiraniAbstract:Abstract We report here four cases of systemic lupus erythematosus associated with the Nephrotic Syndrome and renal vein thrombosis and review six similar cases previously noted in the literature. ...
Walter Manucha - One of the best experts on this subject based on the ideXlab platform.
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follow up of steroid resistant Nephrotic Syndrome tubular proteinuria and enzymuria
Pediatric Nephrology, 2000Co-Authors: Patricia G Valles, Mirta Peralta, Iliana Principi, Liliana Carrizo, Laura Martin, Adriana Gonzalez, Walter ManuchaAbstract:The aim of this study was to examine the compromise of proximal tubule cells in steroid-resistant Nephrotic Syndrome patients with a histologic diagnosis of focal segmental glomerulosclerosis (FSGS) through assessment of the urinary levels of β2-microglobulin (β2M) and N-acetyl-β-d-glucosaminidase (NAG) during active disease and remission over a follow-up period of 3 years. We studied 34 children with Nephrotic Syndrome: 12 with steroid-resistant Nephrotic Syndrome (SRNS) and massive proteinuria, 7 with steroid-dependent Nephrotic Syndrome (SDNS) and 15 with steroid-sensitive Nephrotic Syndrome (SSNS). Of the SSNS patients, 8 children were in remission (RM) and 7 were in relapse (RL). Seven healthy children were included as controls. Urinary β2M, measured by enzyme-linked immunosorbent assay, was significantly increased in the SRNS group as compared to the SDNS group (P<0.01), SSNS in remission (P<0.01), and controls (P<0.01). There were no differences between the SRNS group and SSNS in relapse. Analysis of urinary N-acetyl-β-d-glucosaminidase (U-NAG) by colorimetric assay showed significantly higher values in the SRNS group of patients than in SDNS, SSNS, and control groups. A positive correlation between U-NAG and proteinuria was demonstrated (r=0. 73, P<0.01). The SRNS group of patients (n=12, 11 with a histologic diagnosis of FSGS and one with diffuse mesangial proliferation) was treated with the same protocol of i.v. methylprednisone and oral cyclophosphamide. Long-term follow-up showed a progressive decrease in U-β2M and U-NAG excretion to control values in the 3rd year, except in one patient who did not respond to the treatment. In the FSGS patients, evaluation of the contribution of structural interstitial histological abnormalities, including each of the histological parameters considered in interstitial scarring to the functional tubule abnormalities assessed by β2M and NAG excretion, was performed by multiple regression analysis. The r 2 values for β2M and NAG were 53.99%, P=0.19, and 57.90%, P=0.14, respectively; neither was significant. We conclude that: (1) proximal tubule cell dysfunction, partially affected by massive albuminuria, may account for the higher values of β2M and NAG excretion in the SRNS patients and (2) urine β2M and NAG levels are not helpful in identifying histological evidence of structural tubulointerstitial damage in children with steroid-resistant Nephrotic Syndrome.
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Follow-up of steroid-resistant Nephrotic Syndrome: tubular proteinuria and enzymuria.
Pediatric nephrology (Berlin Germany), 2000Co-Authors: P Vallés, Mirta Peralta, Iliana Principi, Liliana Carrizo, Laura Martin, Adriana Gonzalez, Walter ManuchaAbstract:The aim of this study was to examine the compromise of proximal tubule cells in steroid-resistant Nephrotic Syndrome patients with a histologic diagnosis of focal segmental glomerulosclerosis (FSGS) through assessment of the urinary levels of beta 2-microglobulin (beta 2M) and N-acetyl-beta-D-glucosaminidase (NAG) during active disease and remission over a follow-up period of 3 years. We studied 34 children with Nephrotic Syndrome: 12 with steroid-resistant Nephrotic Syndrome (SRNS) and massive proteinuria, 7 with steroid-dependent Nephrotic Syndrome (SDNS) and 15 with steroid-sensitive Nephrotic Syndrome (SSNS). Of the SSNS patients, 8 children were in remission (RM) and 7 were in relapse (RL). Seven healthy children were included as controls. Urinary beta 2M, measured by enzyme-linked immunosorbent assay, was significantly increased in the SRNS group as compared to the SDNS group (P < 0.01), SSNS in remission (P < 0.01), and controls (P < 0.01). There were no differences between the SRNS group and SSNS in relapse. Analysis of urinary N-acetyl-beta-D-glucosaminidase (U-NAG) by colorimetric assay showed significantly higher values in the SRNS group of patients than in SDNS, SSNS, and control groups. A positive correlation between U-NAG and proteinuria was demonstrated (r = 0.73, P < 0.01). The SRNS group of patients (n = 12, 11 with a histologic diagnosis of FSGS and one with diffuse mesangial proliferation) was treated with the same protocol of i.v. methylprednisone and oral cyclophosphamide. Long-term follow-up showed a progressive decrease in U-beta 2M and U-NAG excretion to control values in the 3rd year, except in one patient who did not respond to the treatment. In the FSGS patients, evaluation of the contribution of structural interstitial histological abnormalities, including each of the histological parameters considered in interstitial scarring to the functional tubule abnormalities assessed by beta 2M and NAG excretion, was performed by multiple regression analysis. The r2 values for beta 2M and NAG were 53.99%, P = 0.19, and 57.90%, P = 0.14, respectively; neither was significant. We conclude that: (1) proximal tubule cell dysfunction, partially affected by massive albuminuria, may account for the higher values of beta 2M and NAG excretion in the SRNS patients and (2) urine beta 2M and NAG levels are not helpful in identifying histological evidence of structural tubulointerstitial damage in children with steroid-resistant Nephrotic Syndrome.
Jai Radhakrishnan - One of the best experts on this subject based on the ideXlab platform.
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treatment of Nephrotic Syndrome with adrenocorticotropic hormone acth
Discovery Medicine, 2011Co-Authors: Andrew S Bomback, Jai RadhakrishnanAbstract:Over the last two decades, adrenocorticotropic hormone (ACTH) has re-emerged as a potentially effective therapy for Nephrotic Syndrome, particularly for patients who have failed more conventional immunosuppressive therapies. The initial experience in Europe using synthetic ACTH in membranous nephropathy led to a randomized trial in which ACTH performed comparably to a combined regimen of steroids and alkylating agents. Observational data from American patients treated with natural ACTH gel for resistant Nephrotic Syndrome have also been promising. While we await larger clinical trials of ACTH in the Nephrotic patient population, we still also await a more precise understanding of how the therapy achieves remission of proteinuria. We discuss a number of possible mechanisms for ACTH's beneficial effects on the inflammation and injury that occurs in Nephrotic Syndrome.
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treatment of Nephrotic Syndrome with adrenocorticotropic hormone acth gel
Drug Design Development and Therapy, 2011Co-Authors: Andrew S Bomback, James A Tumlin, Joel Baranski, James E Bourdeau, Anatole Besarab, Alice S Appel, Jai Radhakrishnan, Gerald B. AppelAbstract:Purpose: A synthetic adrenocorticotropin (ACTH) analog has shown efficacy in Europe as primary and secondary therapy for Nephrotic Syndrome, but there is no published experience using the natural, highly purified ACTH gel formulation, available in the United States, for Nephrotic Syndrome. We therefore investigated the use of ACTH gel for Nephrotic Syndrome in the United States.
Natalia Polanco - One of the best experts on this subject based on the ideXlab platform.
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spontaneous remission of Nephrotic Syndrome in membranous nephropathy with chronic renal impairment
Nephrology Dialysis Transplantation, 2012Co-Authors: Natalia Polanco, Elena Gutierrez, J Baltar, Francisco Rivera, Ines Castellanos, Dolores Lorenzo, Manuel PragaAbstract:Background Spontaneous remission (SR) of Nephrotic Syndrome, in the absence of immunosuppressive treatment, is relatively common among patients with idiopathic membranous nephropathy (IMN) and normal renal function. However, it has not been reported in patients with chronic renal impairment. Methods All patients with IMN who had developed SR in the presence of chronic renal insufficiency were identified among the nephrology departments that belong to the Spanish Group for the Study of Glomerular Diseases (GLOSEN). Their characteristics and outcome after SR were studied. Results Eleven patients were identified. All of them showed renal insufficiency and Nephrotic Syndrome at the time of renal biopsy. Serum creatinine (Scr) continued to increase in the following months, reaching a peak value of 2.6 ± 1.5 mg/dL (range 1.7-6.5). Angiotensin converting enzyme inhibitors or spironolactone were prescribed in 10/11 patients at renal biopsy or shortly after it. Nephrotic proteinuria persisted during the first months of follow-up, but it started to spontaneously decrease 12 ± 7 months (2-30 months) after renal biopsy. Finally, complete (nine patients) or partial (two patients) remission of Nephrotic Syndrome was observed. Coinciding with proteinuria remission, renal function tended to improve. Nephrotic Syndrome relapsed in two patients, accompanied by a rapid deterioration of renal function. In the remaining nine patients, remission persisted throughout a follow-up of 146 ± 64 months. Mean Scr at the last visit was 1.9 ± 0.9 mg/dL and proteinuria 0.2 g/24 h. Conclusion SR of Nephrotic Syndrome can also be observed in membranous nephropathy patients exhibiting chronic renal impairment.
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spontaneous remission of Nephrotic Syndrome in idiopathic membranous nephropathy
Journal of The American Society of Nephrology, 2010Co-Authors: Natalia Polanco, Elena Gutierrez, Adelardo Covarsi, Francisco Ariza, A Carreno, Ana Vigil, J Baltar, Gema Fernandezfresnedo, Carmen Martin, Salvador PonsAbstract:Spontaneous remission is a well known characteristic of idiopathic membranous nephropathy, but contemporary studies describing predictors of remission and long-term outcomes are lacking. We conducted a retrospective, multicenter cohort study of 328 patients with Nephrotic Syndrome resulting from idiopathic membranous nephropathy that initially received conservative therapy. Spontaneous remission occurred in 104 (32%) patients: proteinuria progressively declined after diagnosis until remission of disease at 14.7 ± 11.4 months. Although spontaneous remission was more frequent with lower levels of baseline proteinuria, it also frequently occurred in patients with massive proteinuria: 26% among those with baseline proteinuria 8 to 12 g/24 h and 22% among those with proteinuria >12 g/24 h. Baseline serum creatinine and proteinuria, treatment with angiotensin-converting enzyme inhibitors or angiotensin receptor antagonists, and a >50% decline of proteinuria from baseline during the first year of follow-up were significant independent predictors for spontaneous remission. Only six patients (5.7%) experienced a relapse of Nephrotic Syndrome. The incidence of death and ESRD were significantly lower among patients with spontaneous remission. In conclusion, spontaneous remission is common among patients with Nephrotic Syndrome resulting from membranous nephropathy and carries a favorable long-term outcome with a low incidence of relapse. A decrease in proteinuria >50% from baseline during the first year predicts spontaneous remission.