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1
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0018188039
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International Study of Kidney Disease in Children: Nephrotic syndrome in children: Prediction of histopathology from clinical and laboratory characteristics at time of diagnosis
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ISKDC
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ISKDC. International Study of Kidney Disease in Children: Nephrotic syndrome in children: Prediction of histopathology from clinical and laboratory characteristics at time of diagnosis. Kidney Int 1978; 13:159-165.
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(1978)
Kidney Int
, vol.13
, pp. 159-165
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2
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84913632642
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Pathology of the nephrotic syndrome in children
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Churg J, Habib R, White RHR. Pathology of the nephrotic syndrome in children. Lancet 1970; 1:1299-1302.
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(1970)
Lancet
, vol.1
, pp. 1299-1302
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Churg, J.1
Habib, R.2
White, R.H.R.3
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3
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4344634421
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The changing face of childhood nephrotic syndrome. Nephrology forum
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Chesney R. The changing face of childhood nephrotic syndrome. Nephrology forum. Kidney Int 2004; 66:1294-1302.
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Kidney Int
, vol.66
, pp. 1294-1302
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Chesney, R.1
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4
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34548457364
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Boyer O, Moulder JK, Somers MJ. Focal and segmental glomerulosclerosis in children: a longitudinal assessment. Pediatr Nephrol 2007; 22:1159-1166. A similar review of the data shows increasing incidence of SRNS secondary to FSGS.
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Boyer O, Moulder JK, Somers MJ. Focal and segmental glomerulosclerosis in children: a longitudinal assessment. Pediatr Nephrol 2007; 22:1159-1166. A similar review of the data shows increasing incidence of SRNS secondary to FSGS.
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5
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34447344837
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Fernandes Borges F, Shiraichi L, Da Silva MP, et al. Is focal segmental glomerulosclerosis increasing in patients with nephrotic syndrome? Pediatr Nephrol 2007; 22:1309-1313. This reviews data regarding increasing incidence of FSGS up to the current time.
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Fernandes Borges F, Shiraichi L, Da Silva MP, et al. Is focal segmental glomerulosclerosis increasing in patients with nephrotic syndrome? Pediatr Nephrol 2007; 22:1309-1313. This reviews data regarding increasing incidence of FSGS up to the current time.
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6
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0042466540
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Eddy AA, Symons JM. Nephrotic syndrome in childhood. Lancet 2003; 362:629-639 [seminar].
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Eddy AA, Symons JM. Nephrotic syndrome in childhood. Lancet 2003; 362:629-639 [seminar].
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7
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33845687658
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Focal segmental glomerulosclerosis- epidemiology aspects in children and adults
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This article provides an overview of the epidemiology of FSGS by reviewing published surveys of renal biopsies, experiences from clinical registries of children with renal insufficiency, and data from the US Renal Data Systems
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Hogg R, Middleton J, Vehaskari VM. Focal segmental glomerulosclerosis- epidemiology aspects in children and adults. Pediatr Nephrol 2007; 22:183-186. This article provides an overview of the epidemiology of FSGS by reviewing published surveys of renal biopsies, experiences from clinical registries of children with renal insufficiency, and data from the US Renal Data Systems.
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(2007)
Pediatr Nephrol
, vol.22
, pp. 183-186
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Hogg, R.1
Middleton, J.2
Vehaskari, V.M.3
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8
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33846413899
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Trachtman H. Educational feature on focal segmental glomerulosclerosis: an introduction. Pediatr Nephrol 2007; 22:26-27. This commentary introduces a series of educational articles covering the epidemiology, pathophysiology, genetics and treatment of SRNS secondary to FSGS.
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Trachtman H. Educational feature on focal segmental glomerulosclerosis: an introduction. Pediatr Nephrol 2007; 22:26-27. This commentary introduces a series of educational articles covering the epidemiology, pathophysiology, genetics and treatment of SRNS secondary to FSGS.
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9
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40549116191
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North American Renal Trials and Collaborative Studies (NAPRTCS). 2006 Annual Report. Boston: NAPRTCS administrative office; 2006. This is a comprehensive database on the epidemiology and treatments for kidney failure in children in North America.
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North American Renal Trials and Collaborative Studies (NAPRTCS). 2006 Annual Report. Boston: NAPRTCS administrative office; 2006. This is a comprehensive database on the epidemiology and treatments for kidney failure in children in North America.
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10
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33750601574
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Chua AN, Alexander SR, Sarwal MM, et al. Proteinuria in pediatric renal transplant recipients during the first 60 posttransplant days. Pediatr Transplant 2006; 10:957-961. The authors refresh our memory regarding the risk for recurrence of original disease posttransplant.
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Chua AN, Alexander SR, Sarwal MM, et al. Proteinuria in pediatric renal transplant recipients during the first 60 posttransplant days. Pediatr Transplant 2006; 10:957-961. The authors refresh our memory regarding the risk for recurrence of original disease posttransplant.
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11
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34248646767
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Pathophysiology of focal segmental glomerulosclerosis
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This article reviews some of the experimental and clinical data on the pathophysiology of progression in FSGS and emphasizes the mechanisms of podocyte injury in various models
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Reidy K, Kaskel F. Pathophysiology of focal segmental glomerulosclerosis. Pediatr Nephrol 2007; 22:350-354. This article reviews some of the experimental and clinical data on the pathophysiology of progression in FSGS and emphasizes the mechanisms of podocyte injury in various models.
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(2007)
Pediatr Nephrol
, vol.22
, pp. 350-354
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Reidy, K.1
Kaskel, F.2
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12
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0037349392
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Pathologic classification of focal segmental glomerulosclerosis
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D'Agati V. Pathologic classification of focal segmental glomerulosclerosis. Semin Nephrol 2003; 23:117-134.
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(2003)
Semin Nephrol
, vol.23
, pp. 117-134
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D'Agati, V.1
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13
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0742323170
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Pathologic classification of focal segmental glomerulosclerosis: A working proposal
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D'Agati VD, Fogo AB, Brujin JA. Pathologic classification of focal segmental glomerulosclerosis: A working proposal. Am J Kidney Dis 2004; 368-382.
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(2004)
Am J Kidney Dis
, pp. 368-382
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D'Agati, V.D.1
Fogo, A.B.2
Brujin, J.A.3
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14
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33749256664
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Collapsing glomerulopathy
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This is a complete description of the histologic entity of collapsing FSGS
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Albaqumi M, Soos TJ, Barisoni L, Nelson PJ. Collapsing glomerulopathy. J Am Soc Nephrol 2006; 17:2854-2863. This is a complete description of the histologic entity of collapsing FSGS.
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(2006)
J Am Soc Nephrol
, vol.17
, pp. 2854-2863
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Albaqumi, M.1
Soos, T.J.2
Barisoni, L.3
Nelson, P.J.4
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15
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33644525974
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Clinical and pathologic characteristics of focal segmental glomerulosclerosis pathologic variants
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This is a thorough evaluation of the different histologic subtypes in FSGS, their clinical characteristics, response to therapies and outcome and progression
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Thomas DB, Franceschini N, Hogan SL, et al. Clinical and pathologic characteristics of focal segmental glomerulosclerosis pathologic variants. Kidney Int 2006; 69:920-926. This is a thorough evaluation of the different histologic subtypes in FSGS, their clinical characteristics, response to therapies and outcome and progression.
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(2006)
Kidney Int
, vol.69
, pp. 920-926
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Thomas, D.B.1
Franceschini, N.2
Hogan, S.L.3
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16
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34248641396
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Idiopathic collapsing focal segmental sclerosis in pediatric patients
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This original article from the Children's Hospital of Michigan describes their experience with both collapsing and noncollapsing FSGS. Surprisingly, only one patient with the former diagnosis progressed. Effective treatment prevented progression in this high-risk group
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El-Refaey AM, Kapur G, Jain A, et al. Idiopathic collapsing focal segmental sclerosis in pediatric patients. Pediatr Nephrol 2007; 22:396-402. This original article from the Children's Hospital of Michigan describes their experience with both collapsing and noncollapsing FSGS. Surprisingly, only one patient with the former diagnosis progressed. Effective treatment prevented progression in this high-risk group.
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(2007)
Pediatr Nephrol
, vol.22
, pp. 396-402
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El-Refaey, A.M.1
Kapur, G.2
Jain, A.3
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34548863393
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Silverstein DM, Craver R. Presenting features and short-term outcome according to pathologic variant in childhood primary focal segmental glomerulosclerosis. Clin J Am Soc Nephrol 2007; 2:700-707. An original article from the Children's Hospital, New Orleans, describes their experience with the variants of FSGS and outcome, demonstrating that there was no difference between African-Americans and Caucasians.
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Silverstein DM, Craver R. Presenting features and short-term outcome according to pathologic variant in childhood primary focal segmental glomerulosclerosis. Clin J Am Soc Nephrol 2007; 2:700-707. An original article from the Children's Hospital, New Orleans, describes their experience with the variants of FSGS and outcome, demonstrating that there was no difference between African-Americans and Caucasians.
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18
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33244498085
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Differential risk of remission and ESRD in childhood FSGS
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This is a review of their experience with FSGS based upon response to therapies. Remission of proteinuria had best outcome
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Gipson DS, Chin H, Presler T, et al. Differential risk of remission and ESRD in childhood FSGS. Pediatr Nephrol 2006; 21:344-349. This is a review of their experience with FSGS based upon response to therapies. Remission of proteinuria had best outcome.
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(2006)
Pediatr Nephrol
, vol.21
, pp. 344-349
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Gipson, D.S.1
Chin, H.2
Presler, T.3
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19
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33746997398
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Urine proteomic profiling of pediatric nephrotic syndrome
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This is an original article from the Children's Hospital, Boston
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Khurana M, Traum AZ, Aivado A, et al. Urine proteomic profiling of pediatric nephrotic syndrome. Pediatr Nephrol 2006; 21:1257-1265. This is an original article from the Children's Hospital, Boston.
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(2006)
Pediatr Nephrol
, vol.21
, pp. 1257-1265
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Khurana, M.1
Traum, A.Z.2
Aivado, A.3
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20
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36249032609
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Urinary cytokines and steroid responsiveness in idiopathic nephrotic syndrome of childhood
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An original report evaluates the urinary cytokine profile in relation to response to treatment in INS
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Woroniecki RP, Shatat IF, Supe K, et al. Urinary cytokines and steroid responsiveness in idiopathic nephrotic syndrome of childhood. Am J Nephrol 2008; 28:83-90. An original report evaluates the urinary cytokine profile in relation to response to treatment in INS.
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(2008)
Am J Nephrol
, vol.28
, pp. 83-90
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Woroniecki, R.P.1
Shatat, I.F.2
Supe, K.3
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21
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33645403170
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Hereditary proteinuria syndromes and mechanisms of proteinuria
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This is an excellent review on the normal glomerular filtration barrier and mechanisms of disease
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Tryggvason K, Patrakka J, Wartiovaara J. Hereditary proteinuria syndromes and mechanisms of proteinuria. N Engl J Med 2006; 354:1387-1401. This is an excellent review on the normal glomerular filtration barrier and mechanisms of disease.
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(2006)
N Engl J Med
, vol.354
, pp. 1387-1401
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Tryggvason, K.1
Patrakka, J.2
Wartiovaara, J.3
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22
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Barisoni L, Schnaper HW, Kopp JB. A proposed taxonomy for the podocytopathies: A reassessment of the primary nephrotic diseases. In depth review. Clin J Am Soc Nephrol 2007; 2:529-542. This discusses a proposed new and interesting taxonomy based on the histopathology and etiology of glomerular diseases associated with podocyte injury.
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Barisoni L, Schnaper HW, Kopp JB. A proposed taxonomy for the podocytopathies: A reassessment of the primary nephrotic diseases. In depth review. Clin J Am Soc Nephrol 2007; 2:529-542. This discusses a proposed new and interesting taxonomy based on the histopathology and etiology of glomerular diseases associated with podocyte injury.
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40549096251
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Podocyte and hereditary nephrotic syndromes
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The authors describe the role of podocyte pathophysiology in genetic disorders in nephrotic syndrome
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Gubler MC. Podocyte and hereditary nephrotic syndromes. World J Pediatrics 2007; 3:8-16. The authors describe the role of podocyte pathophysiology in genetic disorders in nephrotic syndrome.
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(2007)
World J Pediatrics
, vol.3
, pp. 8-16
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Gubler, M.C.1
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24
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34247869624
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Genetics of focal segmental glomerulosclerosis
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This reviews recent advances in newly discovered genes and proteins affecting the glomerular filtration barrier and FSGS with indications for future therapies
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Woroniecki RP, Kopp JB. Genetics of focal segmental glomerulosclerosis. Pediatr Nephrol 2007; 22:638-644. This reviews recent advances in newly discovered genes and proteins affecting the glomerular filtration barrier and FSGS with indications for future therapies.
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(2007)
Pediatr Nephrol
, vol.22
, pp. 638-644
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Woroniecki, R.P.1
Kopp, J.B.2
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Microarray analysis of focal segmental glomerulosclerosis
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Schwab K, Witte DP, Aronow BJ, Devarajan P, et al. Microarray analysis of focal segmental glomerulosclerosis. Am J Nephrol 2004; 24:438-447.
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(2004)
Am J Nephrol
, vol.24
, pp. 438-447
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Schwab, K.1
Witte, D.P.2
Aronow, B.J.3
Devarajan, P.4
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26
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34247391838
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Association of steroid and cyclosporine resistance in focal segmental glomerulosclerosis
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An original report describes the association of steroid resistance to subsequent CsA resistance and the need to screen for potential genetic mutations in SRNS patients before administering additional immunosuppression
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Shatat IF, Schoeneman M, Flynn JT, Woroniecki RP. Association of steroid and cyclosporine resistance in focal segmental glomerulosclerosis. Pediatr Nephrol 2007; 22:834-839. An original report describes the association of steroid resistance to subsequent CsA resistance and the need to screen for potential genetic mutations in SRNS patients before administering additional immunosuppression.
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(2007)
Pediatr Nephrol
, vol.22
, pp. 834-839
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Shatat, I.F.1
Schoeneman, M.2
Flynn, J.T.3
Woroniecki, R.P.4
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27
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33846459973
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Therapeutic approach to FSGS in children
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This educational article reviews the current therapeutic approach including non-immunological, conventional and experimental treatments in children with primary FSGS. Excellent review
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Gipson DS, Gibson K, Gipson PE, et al. Therapeutic approach to FSGS in children. Pediatr Nephrol 2007; 22:28-36. This educational article reviews the current therapeutic approach including non-immunological, conventional and experimental treatments in children with primary FSGS. Excellent review.
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(2007)
Pediatr Nephrol
, vol.22
, pp. 28-36
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Gipson, D.S.1
Gibson, K.2
Gipson, P.E.3
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28
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33751117477
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New approaches to the treatment of glomerular diseases
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This concise article reviews newer selective immunomodulating and immunosuppressive regimens, including MMF, rituximab, sirolimus, calcineurin inhibitors, and eculizumab, in adults with different etiologies for SRNS
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Appel GB, Waldman M, Radhakrishnan J. New approaches to the treatment of glomerular diseases. Kidney Int 2006; 70:545-550. This concise article reviews newer selective immunomodulating and immunosuppressive regimens, including MMF, rituximab, sirolimus, calcineurin inhibitors, and eculizumab, in adults with different etiologies for SRNS.
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(2006)
Kidney Int
, vol.70
, pp. 545-550
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Appel, G.B.1
Waldman, M.2
Radhakrishnan, J.3
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33749065865
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Clin J Am Soc Nephrol, This is an excellent review of the experimental and clinical literature important in understanding the physiologic rationale for interference with the renin-angiotensin system in proteinuric diseases
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Griffin KA, Bidani AK. Progression of renal disease: renoprotective specificity of renin-angiotensin system blockade. Clin J Am Soc Nephrol 2006; 1:1054-1065. This is an excellent review of the experimental and clinical literature important in understanding the physiologic rationale for interference with the renin-angiotensin system in proteinuric diseases.
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(2006)
Progression of renal disease: Renoprotective specificity of renin-angiotensin system blockade
, vol.1
, pp. 1054-1065
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Griffin, K.A.1
Bidani, A.K.2
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3242805216
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Antihypertensive and antiproteinuric efficacy of ramipril in children with chronic renal failure
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Wühl E, Mehls O, Schaefer F. Antihypertensive and antiproteinuric efficacy of ramipril in children with chronic renal failure. Kidney Int 2004; 66:768-776.
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(2004)
Kidney Int
, vol.66
, pp. 768-776
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Wühl, E.1
Mehls, O.2
Schaefer, F.3
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32
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0028817502
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Intravenous methylprednisolone and oral alkylating agent therapy of prednisone-resistant pediatric focal segmental glomerulosclerosis: A long-term follow-up
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Tune BM, Kirpekar R, Sibley RK, et al. Intravenous methylprednisolone and oral alkylating agent therapy of prednisone-resistant pediatric focal segmental glomerulosclerosis: a long-term follow-up. Clin Nephrol 1995; 43:84-88.
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(1995)
Clin Nephrol
, vol.43
, pp. 84-88
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Tune, B.M.1
Kirpekar, R.2
Sibley, R.K.3
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This is a review of their experience with intravenous pulse methylprednisolone and cyclophosphamide and oral prednisone in SRNS. A good response was found in MCD patients although increased risk for infections was observed
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Nammalwar BR, Vijayakumar M, Prahlad N, Jain DV. Steroid resistant nephrotic syndrome: Is sustained remission attainable? Indian Pediatr 2006; 43:39-43. This is a review of their experience with intravenous pulse methylprednisolone and cyclophosphamide and oral prednisone in SRNS. A good response was found in MCD patients although increased risk for infections was observed.
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(2006)
Steroid resistant nephrotic syndrome: Is sustained remission attainable? Indian Pediatr
, vol.43
, pp. 39-43
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Nammalwar, B.R.1
Vijayakumar, M.2
Prahlad, N.3
Jain, D.V.4
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34
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34848815017
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Steroid-resistant nephrotic syndrome: Long-termevolution after sequential therapy
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A single-center report on their positive experience with sequential therapy in MCD and FSGS adds support for the use of a modified Mendoza/Tune protocol in SRNS
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Peňa A, Bravo J, Melgosa M, et al. Steroid-resistant nephrotic syndrome: long-termevolution after sequential therapy. Pediatr Nephrol 2007; 22:1875-1880. A single-center report on their positive experience with sequential therapy in MCD and FSGS adds support for the use of a modified Mendoza/Tune protocol in SRNS.
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(2007)
Pediatr Nephrol
, vol.22
, pp. 1875-1880
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Peňa, A.1
Bravo, J.2
Melgosa, M.3
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Low-dose methylprednisolone pulse therapy in Chinese children with steroid resistant focal segmental glomerulosclerosis
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This is a single-center report on the efficacy of low-dose pulse steroids in SRNS secondary to FSGS, again adding renewed interest in the original reports by Mendoza/Tune
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Chang JW, Yang LY, Wang HH. Low-dose methylprednisolone pulse therapy in Chinese children with steroid resistant focal segmental glomerulosclerosis. Pediatr Int 2007; 49:349-354. This is a single-center report on the efficacy of low-dose pulse steroids in SRNS secondary to FSGS, again adding renewed interest in the original reports by Mendoza/Tune.
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(2007)
Pediatr Int
, vol.49
, pp. 349-354
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Chang, J.W.1
Yang, L.Y.2
Wang, H.H.3
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33847354337
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Fifteen-year remission of a steroid-resistant nephrotic syndrome sustained by cyclosporine-A
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A case report demonstrates the efficacy of CsA to induce a remission long after steroids and cyclophosphamide failed
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Drube J, Geerlings C, Taylor R, et al. Fifteen-year remission of a steroid-resistant nephrotic syndrome sustained by cyclosporine-A. Pediatr Nephrol 2007; 22:600-602. A case report demonstrates the efficacy of CsA to induce a remission long after steroids and cyclophosphamide failed.
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(2007)
Pediatr Nephrol
, vol.22
, pp. 600-602
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Drube, J.1
Geerlings, C.2
Taylor, R.3
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0026708832
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Effects of cyclosporine A on glomerular barrier function in the nephrotic syndrome
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Zietse R, Wenting GJ, Kramer P, et al. Effects of cyclosporine A on glomerular barrier function in the nephrotic syndrome. Clin Sci 1992; 82:641-650.
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(1992)
Clin Sci
, vol.82
, pp. 641-650
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Zietse, R.1
Wenting, G.J.2
Kramer, P.3
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0027731266
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Cyclosporin versus cyclophosphamide for patients with steroid-dependent and frequently relapsing idiopathic nephrotic syndrome: A multicenter randomized controlled trial
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Ponticelli C, Edefonti A, Ghio L, et al. Cyclosporin versus cyclophosphamide for patients with steroid-dependent and frequently relapsing idiopathic nephrotic syndrome: a multicenter randomized controlled trial. Nephrol Dial Transplant 1993; 8:1326-1332.
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(1993)
Nephrol Dial Transplant
, vol.8
, pp. 1326-1332
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Ponticelli, C.1
Edefonti, A.2
Ghio, L.3
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0030060612
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A randomized double-blind placebo-controlled trial of cyclosporine in steroid-resistant idiopathic focal segmental glomerulosclerosis in childen
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Lieberman KV, Tejani A. A randomized double-blind placebo-controlled trial of cyclosporine in steroid-resistant idiopathic focal segmental glomerulosclerosis in childen. J Am Soc Nephrol 1996; 7:56-63.
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(1996)
J Am Soc Nephrol
, vol.7
, pp. 56-63
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Lieberman, K.V.1
Tejani, A.2
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27644545854
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Long-term effects of cyclosporine in children with idiopathic nephrotic syndrome: A single-center experience
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El-Husseini A, El-Basuony F, Mahmoud I, et al. Long-term effects of cyclosporine in children with idiopathic nephrotic syndrome: a single-center experience. Nephrol Dial Transplant 2005; 20:2433-2438.
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(2005)
Nephrol Dial Transplant
, vol.20
, pp. 2433-2438
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El-Husseini, A.1
El-Basuony, F.2
Mahmoud, I.3
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42
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32644432824
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Cyclosporine in steroid dependent and resistant nephrotic syndrome
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The authors evaluated CsA monotherapy in SRNS and dependent children. There was a good response rate but many became CsA-dependent, especially if younger
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Iyengar A, Karthik S, Kumar A, et al. Cyclosporine in steroid dependent and resistant nephrotic syndrome. Indian Pediatr 2006; 43:14-19. The authors evaluated CsA monotherapy in SRNS and dependent children. There was a good response rate but many became CsA-dependent, especially if younger.
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(2006)
Indian Pediatr
, vol.43
, pp. 14-19
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Iyengar, A.1
Karthik, S.2
Kumar, A.3
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43
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32644451375
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Steroid resistant nephrotic syndrome: Recent Developments
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This reviews advances in the field from Indian investigations and makes a case for randomized controlled clinical trials
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Bagga A. Steroid resistant nephrotic syndrome: Recent Developments. Indian Pediatr 2006; 43:9-13. This reviews advances in the field from Indian investigations and makes a case for randomized controlled clinical trials.
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(2006)
Indian Pediatr
, vol.43
, pp. 9-13
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Bagga, A.1
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Cattran DC, Alexopoulos E, Heering P, et al. Cyclosporin in idiopathic glomerular disease associated with the nephrotic syndrome: Workshop recommendations. Kidney Int 2007; 26 Sept [Epub ahead of print]. This review provides the evidence for the use of CsA in inducing remission in MCD and SRNS secondary to FSGS and provides algorithms for evaluation and treatment in adult and pediatric patients. It offers a useful guide to cyclosporine in renal disease.
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Cattran DC, Alexopoulos E, Heering P, et al. Cyclosporin in idiopathic glomerular disease associated with the nephrotic syndrome: Workshop recommendations. Kidney Int 2007; 26 Sept [Epub ahead of print]. This review provides the evidence for the use of CsA in inducing remission in MCD and SRNS secondary to FSGS and provides algorithms for evaluation and treatment in adult and pediatric patients. It offers a useful guide to cyclosporine in renal disease.
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Colquitt JL, Kirby J, Green C, et al. The clinical effectiveness and cost-effectiveness of treatments for children with idiopathic steroid-resistant nephrotic syndrome: a systematic review. Health Technol Assess 2007; 11:1-93. This review assessed the clinical and cost-effectiveness of treatments for children with idiopathic SRNS. Eleven trials were examined and evidence supported the efficacy of CsA in inducing remissions in the non-FSGS group and for the effectiveness of cyclophosphamide on time for remission. It emphasizes the need for a modeling framework for a well designed RCT comparing CsA with other treatments in children with SRNS without genetic mutations.
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Colquitt JL, Kirby J, Green C, et al. The clinical effectiveness and cost-effectiveness of treatments for children with idiopathic steroid-resistant nephrotic syndrome: a systematic review. Health Technol Assess 2007; 11:1-93. This review assessed the clinical and cost-effectiveness of treatments for children with idiopathic SRNS. Eleven trials were examined and evidence supported the efficacy of CsA in inducing remissions in the non-FSGS group and for the effectiveness of cyclophosphamide on time for remission. It emphasizes the need for a modeling framework for a well designed RCT comparing CsA with other treatments in children with SRNS without genetic mutations.
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Hodson EM, Habashy D, Craig JC. Interventions for idiopathic steroid-resistant nephrotic syndrome in children. Cochrane Database Syst Rev 2007; (2):CD003594. This review evaluates the benefits and harm of 11 different randomized and quasi-randomized controlled trials used in 213 children with idiopathic nephrotic syndrome who did not achieve remission following four weeks or more of daily corticosteroid therapy. It supports the efficacy of CsA in inducing remission and the use of ACEI in reducing proteinuria but emphasizes the need for well designed RCTs to confirm the efficacy of CsA and to evaluate other regimens for idiopathic SRNS.
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Tumlin JA, Miller D, Near M, et al. A prospective, open-label trial of Sirolimus in the treatment of focal segmental glomerulosclerosis. Clin J Am Soc Nephrol 2006; 1:109-116. This report describes the reduction in proteinuria and glomerular pore size and increase in glomerular ultrafiltration coefficient by Sirolimus in patients with SRNS secondary to FSGS.
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Smith GC. Is there a role for rituximab in the treatment of idiopathic childhood nephrotic syndrome? Pediatr Nephrol 2007; 22:892-898. This reviews the current field of knowledge regarding this new therapy and sets the template for future randomized controlled studies.
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Middleton J, Thomas DB, Kaskel FJ, et al. Focal segmental glomerulosclerosis: the need for improved treatment options and the basis for the ongoing NIH clinical trial. Nephrology Rounds 2007; 5:1-6. The authors review the histology, genetics, and current treatments for FSGS and the design of the NIH FSGS-CT that will evaluate possible treatment modalities.
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