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Focal segmental glomerulosclerosis

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Abstract

Over the last 2 decades, we have learnt that focal segmental glomerulosclerosis (FSGS) is a ubiquitous phenomenon underlying the progressive deterioration of many different types of renal diseases in both pediatric and adult populations. FSGS may also be the primary renal lesion, whether in new disease entities such as glycogen storage disease and human immunodeficiency virus infection, or in idiopathic FSGS. Although the mechanism which triggers the development of primary FSGS still remains unknown, laboratory and clinical studies have identified several key pathophysiological events leading to end-stage renal disease. While therapeutic modalities have not changed remarkably, a recent study, although uncontrolled, demonstrated an impressive efficacy of intravenous steroid pulse therapy in inducing remission. Nevertheless, it remains largely unknown whether such a forced remission decreases the overall risk of developing chronic renal failure. Studies have revealed an important pathophysiological role of angiotensin and the therapeutic efficacy of angiotensin converting enzyme inhibitors in progressive loss of renal function in diseases where glomerulosclerosis is secondary; however, it remains to be verified whether these results hold true in primary FSGS. As a result of the improvement in allograft survival rate, the benefit of renal transplant outweighs the risk of recurrence of FSGS, hence transplantation continues to be a vital therapy for FSGS patients who have reached renal failure. Thus, FSGS is not one disease, but rather a range of lesions seen in many settings. The type of lesions and the patient's unique genetic factors contribute to prognosis, and also may dictate choice of optimum therapy.

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References

  1. Rennke HG, Klein PS (1989) Pathogenesis and significance of nonprimary focal and segmental glomerulosclerosis. Am J Kidney Dis 13:443–456

    PubMed  Google Scholar 

  2. Corwin HL, Schwartz MM, Lewis EJ (1988) The importance of sample size in the interpretation of the renal biopsy. Am J Nephrol 8:85–89

    PubMed  Google Scholar 

  3. Remuzzi A, Mazarska M, Gephardt GN, Novick AC, Brenner BM, Remuzzi G (1995) Three-dimensional analysis of glomerular morphology in patients with subtotal nephrectomy. Kidney Int 48:155–162

    PubMed  Google Scholar 

  4. Fogo A, Glick AD, Horn SL, Horn RG (1995) Is focal segmental glomerulosclerosis really focal? Distribution of lesions in adults and children. Kidney Int 47:1690–1696

    PubMed  Google Scholar 

  5. Fogo A, Hawkins EP, Berry PL, Glick AD, Chiang ML Mac-Donell Jr, Ichikawa I (1990) Glomerular hypertrophy in minimal change disease predicts subsequent progression to focal glomerular sclerosis. Kidney Int 38:115–123

    PubMed  Google Scholar 

  6. Schwartz MM, Korbet SM (1993) Primary focal segmental glomerulosclerosis: pathology, histological variants, and pathogenesis. Am J Kidney Dis 22:874–883

    PubMed  Google Scholar 

  7. D'Agati V (1994) The many masks of focal segmental glomerulosclerosis. Kidney Int 46:1223–1241

    PubMed  Google Scholar 

  8. Sandison A, Newbold KM, Howie AJ (1992) Evidence for unique distribution of Kimmelstiel-Wilson nodules in glomeruli. Diabetes 41:952–955

    PubMed  Google Scholar 

  9. Cohen AH, Nast CC (1988) HIV-associated nephropathy: a unique combined glomerular, tubular, and interstitial lesion. Mod Pathol 1:87–97

    PubMed  Google Scholar 

  10. Detwiler RK, Falk RF, Hogan SL, Jennette JC (1994) Collapsing glomerulopathy: a clinically and pathologically distinct variant of focal segmental glomerulosclerosis. Kidney Int 45:1416–1424

    PubMed  Google Scholar 

  11. Yoshikawa N, Itto H, Akamatsu R, Matsuyama S, Hasegawa O, Nakahara C, Matsu T (1986) Focal segmental glomerulosclerosis with an without nephrotic syndrome in children. J Pediatr 109:65–70

    PubMed  Google Scholar 

  12. Ito H, Yoshikawa N, Aozai F, Hazikano H, Sakaguchi H, Akamatsu R, Matsuo T, Matsuyama S (1984) Twenty-seven children with focal segmental glomerulosclerosis: correlation between the segmental location of the glomerular lesions and prognosis. Clin Nephrol 22:9–14

    PubMed  Google Scholar 

  13. Howie AJ (1986) Changes at the glomerular tip: a feature of membranous nephropathy and other disorders associated with proteinuria. J Pathol 150:13–20

    PubMed  Google Scholar 

  14. Howie AJ, Brewer DB (1985) Further studies on the glomerular tip lesion: early and late stages and life table analysis. J Pathol 147:245–255

    PubMed  Google Scholar 

  15. Haas M, Spargo B, Coventry S (1995) Increasing incidence of focal-segmental glomerulosclerosis among adult nephropathies: a 20-year renal biopsy study. Am J Kidney Dis 26:740–750

    PubMed  Google Scholar 

  16. Schwartz MM, Lewis EJ (1985) Focal segmental glomerular sclerosis: the cellular lesion. Kidney Int 28:968–974

    PubMed  Google Scholar 

  17. Report of the Southwest Pediatric Nephrology Group (1983) Childhood nephrotic syndrome associated with diffuse mesangial hypercellularity. Kidney Int 24:87–94

    Google Scholar 

  18. International Study of Kidney Disease in Children (1981) Primary nephrotic syndrome in children: clinical significance of histopathologic variants of minimal change and of diffuse mesangial hypercellularity. Kidney Int 20:765–771

    Google Scholar 

  19. Hui A, Poucell S, Thorner P, Rance CP, Baumal R (1984) Clinical features and glomerular immunofluorescence of renal biopsies from children with nephrotic syndrome due to minimal change disease and two variants of mesangial proliferative glomerulonephritis. Int J Pediatr Nephrol 5:5–10

    PubMed  Google Scholar 

  20. Southwest Pediatric Nephrology Study Group (1985) A report: focal segmental glomerulosclerosis in children with idiopathic nephrotic syndrome. Kidney Int 27:442–449

    Google Scholar 

  21. Cohen AH, Border WA, Glassock R, Trysgtad C (1978) Glomerulonephritis with mesangial IgM deposits. Lab Invest 38:610–619

    PubMed  Google Scholar 

  22. Tejani A, Nicastri AD (1983) Mesangial IgM nephropathy. Nephron 35:1–5

    PubMed  Google Scholar 

  23. Saha H, Mustonen J, Pasternack A, Helin H (1989) Clinical follow-up of 54 patients with IgM-nephropathy. Am J Nephrol 9:124–128

    PubMed  Google Scholar 

  24. Border WA (1988) Distinguishing minimal-change disease from mesangial disorders. Kidney Int 34:419–434

    PubMed  Google Scholar 

  25. Kincaid-Smith P (1975) Glomerular lesions in atrophic pyelonephritis and reflux nephropathy. Kidney Int 8 [Suppl 4]: S81-S83

    Google Scholar 

  26. Rao TKS, Nicastri AD, Friedman EA (1974) Natural history of heroin-associated nephropathy. N Engl J Med 290:19–23

    PubMed  Google Scholar 

  27. D'Agati V, Suh JI, Carbone L, Cheng JT, Appel G (1989) Pathology of HIV-associated nephropathy: a detailed morphologic and comparative study. Kidney Int 35:1358–1370

    PubMed  Google Scholar 

  28. Cohen AH, Sun NCJ, Shapshak P, Imagawa DT (1989) Demonstration of human immunodeficiency virus in renal epithelium in HIV-associated nephropathy. Mod Pathol 2:125–128

    PubMed  Google Scholar 

  29. Habib R, Courtecuisse V, Mathieu H, Royer P (1962) Un type anatomoclinique particulier d'insuffisance rénale chronique de l'infant. L'hypoplasie oligonéphronique congénitale bilatérale. J Urol Nephrol (Paris) 68:139

    Google Scholar 

  30. Fogo A, Ichikawa I (1992) Glomerular growth promoter — the common channel to glomerular sclerosis. In: Mitch WE (ed) Contemporaty issues in nephrology: the progressive nature of renal disease. 2 edn. Churchill Livingstone, New York, pp 23–54

    Google Scholar 

  31. Aschinberg LC, Koskimies O, Bernstein J Nash M, Edelmann CM Jr, Spitzer A (1978) The influence of age on the response to renal parencymal loss. Yale J Biol Med 51:341–345

    PubMed  Google Scholar 

  32. O'Donnell MP, Kasiske BL, Raij L, Keane WF (1985) Age is a determinant of the glomerular morphologic and functional responses to chronic nephron loss. J Lab Clin Med 106:308–313

    PubMed  Google Scholar 

  33. Ikoma M, Yoshioka T, Ichikawa I, Fogo A (1990) Mechanism of the unique susceptibility of deep cortical glomeruli of maturing kidneys to severe focal glomerular sclerosis. Pediatr Res 28:270–276

    PubMed  Google Scholar 

  34. Novick AC, Gephardt G, Guz B, Steinmuller D, Tubbs RR (1991) Long-term follow-up after partial removal of a solitary kidney. N Engl J Med 325:1058–1062

    PubMed  Google Scholar 

  35. Chen YT, Cohen RA, Scheinman JI, Kolbeck PC, Sidbury JB (1988) Renal disease in type I glycogen storage disease. N Engl J Med 318:7–11

    PubMed  Google Scholar 

  36. Thomson PD, Stokes CR, Barratt TM, Tuner MW, Soothill JF (1976) HLA antigens and atopic features in steroid-responsive nephrotic syndrome of childhood. Lancet II:765–768

    Google Scholar 

  37. Schnaper HW (1989) The soluble immune response suppressor pathway in nephrotic syndrome. Semin Nephrol 9:107–111

    PubMed  Google Scholar 

  38. Takeda T, Nakagawa Y, Narita I, Kimura H, Kutsuwada, T, Arakawa M (1995) Identification and characterization of specifically expressed genes in monoclear cells from patients with glomerulosclerosis (abstract). J Am Soc Nephrol 6:854

    Google Scholar 

  39. Tanaka R, Sugihara K, Tatematsu A, Fogo A (1995) Internephron heterogeneity of growth factors and sclerosis — modulation of platelet derived growth factor by angiotensin II. Kidney Int 47:131–139

    PubMed  Google Scholar 

  40. Pesce CM, Striker LJ, Peten E, Elliot SJ, Striker GE (1991) Glomerulosclerosis at both early and late stages is associated with increased cell turnover in mice transgenic for growth hormone. Lab Invest 65:601–605

    PubMed  Google Scholar 

  41. Nadasdy T, Laszik Z, Blick KE, Johnson LD, Silva FG (1994) Proliferative activity of intrinsic cell populations in the normal human kidney. J Am Soc Nephrol 4:2032–2039

    PubMed  Google Scholar 

  42. Szaboles MJ, Ward L, Buttyan R, D'Agati V (1994) Apoptosis elucidated by labeling for DNA fragmentation in human renal biopsies (abstract). Lab Invest 70:160A

    Google Scholar 

  43. Oikawa T, Kakuchi J, Fogo A (1995) Interleukin 1β converting enzyme (ICE) is increased in association with pathological apoptosis in the sclerotic kidney (abstract). J Am Soc Nephrol 6:1026

    Google Scholar 

  44. Johnson RJ (1994) The glomerular responses to injury: progression or resolution? Kidney Int 45:1769–1782

    PubMed  Google Scholar 

  45. Baker AJ, Mooney A, Hughes J, Lombardi D, Johnson RJ, Savill J (1994) Mesagial cell apoptosis: the major mechanism for resolution of glomerular hypercellularity in experimental mesangial proliferative nephritis. J Clin Invest 94:2105–2116

    PubMed  Google Scholar 

  46. Kitamura H, Shimizu A, Masuda Y, Muroga K, Ishizaki M, Sugisaki Y, Yamanaka N (1995) Capillary regeneration during progression or recovery of glomerulonephritis (GN) (abstract). J Am Soc Nephrol 6:872

    Google Scholar 

  47. Ikoma M, Kawamura T, Kakinuma, Y, Fogo A, Ichikawa I (1991) Cause of variable therapeutic efficiency of angiotensin converting enzyme inhibitor on glomerular lesions. Kidney Int 40:195–202

    PubMed  Google Scholar 

  48. Yoshida H, Mitarai T, Kawamura T, Kitajima T, Miyazaki Y, Nagasawa R, Kawaguchi Y, Kubo H, Ichikawa I, Sakai O (1985) Role of the deletion polymorphism of the angiotensin converting enzyme gene in the progression and therapeutic responsiveness of IgA nephropathy. J Clin Invest 96:2162–2169

    Google Scholar 

  49. Danoff TM, Chiang MY, Oritz A, Neilson EG (1993) Transcriptional regulation of murine RANTES (MuR) in proximal tubular cells (abstract). J Am Soc Nephrol 4:599

    Google Scholar 

  50. Jennette JC, Marquis A, Falk RJ, Bodick N (1990) Glomerulomegaly in focal segmental glomerulosclerosis (FSGS) but not minimal change glomerulopathy (MCG) (abstract). Lab Invest 62:48A

    Google Scholar 

  51. Suzuki J, Yoshikawa N, Nakamura H (1994) A quantitative analysis of the glomeruli in focal segmental glomerulosclerosis. Pediatr Nephrol 8:416–419

    PubMed  Google Scholar 

  52. Nyberg E, Bohman SO, Berg U (1994) Glomerular volume and renal function in children with different types of the nephrotic syndrome. Pediatr Nephrol 8:285–289

    PubMed  Google Scholar 

  53. Mauer SM, Steffes MW, Ellis EN, Sutherland DER, Brown DM, Goetz FC (1984) Structural-functional relationship in diabetic nephropathy. J Clin Invest 74:1143–1155

    PubMed  Google Scholar 

  54. Vats AN, Basgen JM, Steffes MW, Mauer M (1994) Mean glomerular volume (GV) in minimal change nephrotic syndrome (MCNS), focal segmental glomerulosclerosis (FSGS), normal children and adults (abstract). J Am Soc Nephrol, 5:797

    Google Scholar 

  55. Vats A, Basgen J, Steffes M, Mauer M (1994) Glomerular number in focal segmental glomerulosclerosis (FSGS) and minimal change nephrotic syndrome (MCNS) (abstract). J Am Soc Nephrol 5:797

    Google Scholar 

  56. Diamond JR, Karnovsky MJ (1986) Focal and segmental glomerulosclerosis following a single intravenous dose of puromycin aminonucleoside. Am J Pathol 122:481–487

    PubMed  Google Scholar 

  57. Fogo A, Hawkins EP, Verani R, MacDonell RC Jr, Ichikawa I (1991) Focal segmental glomerulosclerosis (FGS) in renal transplants is associated with marked glomerular hypertrophy (abstract). J Am Soc Nephrol 2:797

    Google Scholar 

  58. Rich AR (1957) A hitherto undescribed vulnerability of the juxtamedullary glomeruli in lipoid nephrosis. Bull Johns Hopkins Hosp 100:173–186

    PubMed  Google Scholar 

  59. Schreiner GF, Rovin BH, Lefkowith JB (1989) The anti-inflammatory effects of essential fatty acid deficiency in experimental glomerulonephritis: the modulation of macrophage migration and eicosanoid metabolism. J Immunol 143:3192–3199

    PubMed  Google Scholar 

  60. Johnson RJ, Raines EW, Floege J, Yoshimura A, Pritzl P, Alpers C, Ross R, (1992) Inhibition of mesangial cell proliferation and matrix expansion in glomerulonephritis in the rat by antibody to platelet-derived growth factor. J Exp Med 175:1413–1416

    PubMed  Google Scholar 

  61. Yamamoto T, Nakamura T, Noble NA, Ruoslahti E, Border WA (1993) Expression of transforming growth factor β is elevated in human and experimental diabetic nephropathy. Proc Natl Acad Sci USA 90:1814–1818

    PubMed  Google Scholar 

  62. Ketteler M, Noble NA, Border WA (1995) Transforming growth factor-β and angiotensin II: the missing link from glomerular hyperiltration to glomerulosclerosis? Annu Rev Physiol 57:279–295

    PubMed  Google Scholar 

  63. Lewis EJ, Hunsicker LG, Bain RP, Rohde RD (1993) The effect of angiotensin-converting-enzyme inhibition on diabetic nephropathy. N Engl J Med 329:1456–1462

    PubMed  Google Scholar 

  64. Yoshida H, Kuriyama S, Atsumi Y, Tomonari Y, Mitarai T, Hamaguchi A, Kubo H, Kawaguchi Y, Kon V, Matsuoka K, Ichikawa I, and Sakai O, Angiotensin-1 converting enzyme gene polymorphism in non-insulin dependent eiabetes mellitus: Risk for progression to chronic renal failure and mortality. Kidney Int (in press)

  65. Neuringer JR, Anderson S, Brenner BM (1992) The role of systemic and intraglomerular hypertension In: Mitch WE (ed) The progressive nature of renal disease, vol 26. Churchill Livingstone, New York, pp 1–21

    Google Scholar 

  66. Yoshida Y, Fogo A, Shiraga H, Glick AD, Ichikawa I (1988) Serial micropuncture analysis of single nephron function in the rat model of subtotal renal ablation. Kidney Int 33:855–867

    PubMed  Google Scholar 

  67. Yoshida Y, Fogo A, Ichikawa I (1989) Glomerular hemodynamic changes vs. hypertrophy in experimental glomerular sclerosis. Kidney Int 35:654–660

    PubMed  Google Scholar 

  68. Daniels BS, Hostetter TH (1990) Adverse effects of growth in the glomerular microcirculation. Am J Physiol 258:F1409-F1416

    PubMed  Google Scholar 

  69. Dworkin LD, Levin RI, Bernstein JA, Parker M, Ullian ME, Kim Y, Feiner HD (1990) Effects of nifedipine and enalapril on glomerular injury in rats with deoxycorticosterone-salt hypertension. Am J Physiol 259:F598-F604

    PubMed  Google Scholar 

  70. Nyengaard JR, Rasch R (1993) The impact of experimental diabetes mellitus in rats on glomerular capillary number and sizes. Diabetologia 36:189–194

    PubMed  Google Scholar 

  71. Marcussen N, Nyengaard JR, Christensen S (1994) Compensatory growth of glomeruli is accomplished by an increased number of glomerular capillaries. Lab Invest 70:868–874

    PubMed  Google Scholar 

  72. Akaoka K, White RHR, Raafat F (1995) Glomerular morphometry in childhood reflux nephropathy, emphasizing the capillary changes. Kidney Int 47:1108–1114

    PubMed  Google Scholar 

  73. Yoshioka T, Shiraga H, Yoshida Y, Fogo A, Rennke HG, Deen WM, Hoyer JR, Ichikawa I (1988) “Intact nephrons” as the primary origin of proteinuria in chronic renal disease: a study in the rat model of subtotal nephrectomy. J Clin Invest 82:1614–1623

    PubMed  Google Scholar 

  74. Morelli E, Loon N, Meyer T, Peters W, Myers BD (1990) Effects of converting-enzyme inhibition on barrier function in diabetic glomerulopathy. Diabetes 39:76–82

    PubMed  Google Scholar 

  75. Artero ML, Sharma R, Savin VJ, Vincenti F (1994) Plasmapheresis reduces proteinuria and serum capacity to injure glomeruli in patients with recurrent focal glomerulosclerosis. Am J Kidney Dis 23:574–581

    PubMed  Google Scholar 

  76. Dantal J, Bigot E, Bogers W, Testa A, Kriaa F, Jacques Y, Hurualt de Ligny B, Niaudet P, Charpentier B, Soulillou JP (1994) Effect of plasma protein adsorption on protein excretion in kidneytransplant recipients with recurrent nephrotic syndrome. N Engl J Med 330:7–14

    PubMed  Google Scholar 

  77. Sharma M, Sharma R, Gunwar S, Vincenti F, Artero M, Savin V (1992) Partial purification and characterization of a circulating protein (HFSF) that increases glomerular albumin permeability in human focal segmental glomerular sclerosis (abstract). J Am Soc Nephrol 3:751

    Google Scholar 

  78. Benigni A, Zoja C, Remuzzi G (1995) The renal toxicity of sustained glomerular protein traffic. Lab Invest 73:461–468

    PubMed  Google Scholar 

  79. Eddy AA, Giachelli CM (1995) Renal expression of genes that promote interstitial inflammation and fibrosis in rats with proteinoverload proteinuria. Kidney Int 47:1546–1557

    PubMed  Google Scholar 

  80. Thomas ME, Schreiner GF (1993) Contribution of proteinuria to progressive renal injury: consequences of tubular uptake of fatty acid bearing albumin. Am J Nephrol 13:385–398

    PubMed  Google Scholar 

  81. Kees-Folt D, Sadow JL, Schreiner GF (1994) Tubular catabolism of albumin is associated with the release of an inflammatory lipid. Kidney Int 45:1697–1709

    PubMed  Google Scholar 

  82. Keane WF, Kasiske BL, O'Donnell MP (1988) Hyperlipidemia and the progression of renal disease. Am J Clin Nutr 7:157–160

    Google Scholar 

  83. Allon M, Pasque CB, Rodriguez M (1990) Acute effect of captopril and ibuprofen on proteinuria in patients with nephrosis. J Lab Clin Med 116:462–468

    PubMed  Google Scholar 

  84. Schainnuck LI, Striker GE, Cutler RE, Benditt EP (1970) Structural-functional correlations in renal disease. II. The correlations. Hum Pathol 1:631–641

    PubMed  Google Scholar 

  85. Baylis C, Brenner BM (1978) Mechanism of the glucocorticoid-induced increase in glomerular filtration rate. Am J Physiol 234:F166-F170

    PubMed  Google Scholar 

  86. Fogo A, Sakai T, Berliner KTN, Ichikawa I (1991) Insidious nature of diabetic nephropathy: glomerular sclerosis can develop with normal serum creatinine. Lab Invest 64:97A

    Google Scholar 

  87. Besbas N, Topaloglu R, Saatci O, Bakkaloglu A (1992) Long term follow-up in children with steroid-resistant nephrotic syndrome. Clin Pediatr (Phila) 31:283–288

    Google Scholar 

  88. United States Renal Data System 1991 Annual Data Report (1991) ESRD in children. Am J Kidney Dis 18 [Suppl 2]:79–88

    Google Scholar 

  89. McEnery PT, Stablein DM, Arbus G, Tejani A (1991) Renal transplantation in children. A report of the North American Pediatric Renal Transplant Cooperative Study. N Engl J Med 326:1727–1732

    Google Scholar 

  90. Bakir AA, Bazilinski NG, Rhee HL, Ainis H, Dunea G (1989) Focal segmental glomerulosclerosis. A common entity in nephrotic black adults. Arch Intern Med 149:1802–1804

    PubMed  Google Scholar 

  91. Walker R, Bailey RR, Lynn KL, Burry AF (1982) Focal glomerulosclerosis — another familial renal disease? N Z Med J 95:686–688

    PubMed  Google Scholar 

  92. Felldin M, Norden G, Svalander C, Nyberg G (1995) Focal segmental glomerulosclerosis — familial and sporadic forms (abstract). Proceedings of the Nordic Nephrology Meeting. p. 12

  93. Schulman SL, Vaniska J, Kaplan B (1995) Two populations of focal segmental glomerulosclerosis (FSGS) in children (abstract). J Am Soc Nephrol 6:432

    Google Scholar 

  94. Pesce C, Schmidt K, Fogo A, Okoye MI, Kim R, Striker LJ, Striker GE (1994) Glomerular size and the incidence of renal disease in African Americans and Caucasians. J Nephrol 7:355–358

    Google Scholar 

  95. Hunley TE, Julian BA, Phillips JA, Summar ML, Yoshida H, Horn RG, Brown NJ, Fogo A, Ichikawa I, Kon V (1996) An giotensin converting enzyme gene polymorphism: potential silencer motif and impact on progression in IgA nephropathy. Kidney Int 49:571–577

    PubMed  Google Scholar 

  96. Fogo A, Yoshida Y, Yared A, Ichikawa I (1990) Importance of angiogenic action of angiotensin II in the glomerular growth of maturing kidneys. Kidney Int 38:1068–1074

    PubMed  Google Scholar 

  97. Dileepan KN, Sharma R, Stechschulte DJ, Savin VJ (1993) Effect of superoxide exposure on albumin permeability of isolated rat glomeruli. J Lab Clin Med 121:797–804

    PubMed  Google Scholar 

  98. Diamond JR, Bonventre JV, Karnovsky MJ (1986) A role for oxygen free radicals in aminonucleoside nephrosis. Kidney Int 29:478–483

    PubMed  Google Scholar 

  99. Williams JD, Coles GA (1994) Proteinuria — a direct cause of renal morbidity? Kidney Int 45:43–50

    PubMed  Google Scholar 

  100. D'Amico G, Ferrario F, Rastaldi MP, (1995) Tubulointerstitial damage in glomerular diseases: its role in the progression of renal damage. Am J Kidney Dis 26:124–132

    PubMed  Google Scholar 

  101. Praga M, Hernandez E, Montoyo C, Andres A, Ruilope LM, Rodicio JL (1992) Long-term beneficial effects of angiotensin-converting enzyme inhibition in patients with nephrotic proteinuria. Am J Kidney Dis 20:240–248

    PubMed  Google Scholar 

  102. Primack WA, Schulman SL, Kaplan BS (1994) An analysis of the approach to management of childhood nephrotic syndrome by pediatric nephrologists. Am J Kidney Dis 23:524–527

    PubMed  Google Scholar 

  103. Cattran DC, Greenwood C, Ritchie S (1994) Long-term benefit of angiotensin-converting enzyme inhibitor therapy in patients with severe immunoglobulin A nephropathy: a comparison to patients receiving treatment with other antihypertensive agents and to patients receiving no therapy. Am J Kidney Dis 23:247–254

    PubMed  Google Scholar 

  104. Arbeitsgemeinschaft für Padiatrische Nephrologie (1988) Short versus standard prednisone therapy for initial treatment for idiopathic nephrotic syndrome in children. Lancet I:380–383

    Google Scholar 

  105. Pei Y, Cattran D, Delmore T, Katz A, Lang A, Rance P (1987) Evidence suggesting under-treatment in adults with idiopathic focal segmental glomerulosclerosis. Am J Med 82:938–944

    PubMed  Google Scholar 

  106. Brodehl J, Hoyer PF, Oemar BS, Helmchen U, Wonigeit K (1988) Cyclosporine treatment of nephrotic syndrome in children (review). Transplant Proc 20 [Suppl 4]:269–274

    Google Scholar 

  107. Cameron JS, Turner DR, Ogg CS, Chantler C, Williams DC (1978) The long-term prognosis of patients with focal segmental glomerulosclerosis. Clin Nephrol 10:211–218

    Google Scholar 

  108. White RHR, Glasgow EF, Mills RJ (1970) Clinicopathologic study of nephrotic syndrome in childhood. Lancet I:1299–1302

    Google Scholar 

  109. Habib R, Kleinknecht C (1971) The primary nephrotic syndrome of childhood. Pathol Annu 6:417–474

    PubMed  Google Scholar 

  110. Hyman LR, Burkholder PM (1974) Focal sclerosing glomerulonephropathy with hylanosis. J Pediatr 84:217–225

    PubMed  Google Scholar 

  111. Nash MA, Greifer I, Olbing H, Bennet B, Spitzer A (1976) The significance of focal sclerotic lesions of glomeruli in children. J Pediatr 88:806–813

    PubMed  Google Scholar 

  112. Morita M, White RHR, Coad NAG, Raafat F (1990) The clinical significance of the glomerular location of segmental lesion in focal segmental glomerulosclerosis. Clin Nephrol 33:211–219

    PubMed  Google Scholar 

  113. Mongeau JG, Corneille L, Robitaille PO, O'Regan S, Pelletier M (1981) Primary nephrosis in childhood associated with focal glomerular sclerosis: is long-term prognosis that severe? Kidney Int 20:743–746

    PubMed  Google Scholar 

  114. International Study of Kidney Disease in Children (1981) The rimary nephrotic syndrome in children. Identification of patients with minimal change nephrotic syndrome from initial response to prednisone. J Pediatr 98:561–564

    Google Scholar 

  115. Arbus GS, Poucell S, Bacheyie GS, Baumal R (1982) Focal segmental glomerulosclerosis with idiopathic nephrotic syndrome: three types of clinical response. J Pediatr 101:40–45

    PubMed  Google Scholar 

  116. Mowry J, Marik J, Cohen A, Hogg R, Sahney S, Ettenger R (1993) Treatment of recurrent focal segmental glomerulosclerosis with high-dose cyclosporine A and plasmapheresis. Transplant Proc 25:1345–1346

    PubMed  Google Scholar 

  117. Habib R (1973) Focal glomerularsclerosis. Kidney Int 4:355–361

    PubMed  Google Scholar 

  118. Ellis D, Kapur S, Antonovych TT, Salcedo JR, Yunis EJ (1978) Focal glomerulosclerosis in children: correlation of histology with prognosis. J Pediatr 93:762–768

    PubMed  Google Scholar 

  119. Beaufils H, Alphonse JC, Guedon J, Legrain M (1978) Focal glomerulosclerosis: natural history and treatment. A report of 70 cases. Nephron 21:75–85

    PubMed  Google Scholar 

  120. Ellis D, Buffone GJ (1981) Protein clearances and selectivity determinations in childhood nephrosis: a reappraisal. Clin Chem 27:1397–1400

    PubMed  Google Scholar 

  121. Guasch A, Hashimoto H, Sibley RK, Deen WM, Myers BD (1991) Glomerular dysfunction in nephrotic humans with minimal changes or focal glomerulosclerosis. Am J Physiol 260:F728-F737

    PubMed  Google Scholar 

  122. Laurent J, Philippon C, Lagrue G, Laurent G, Weil B, Rostoker G (1993) Proteinuria selectivity index — prognostic value in lipoid nephrosis and related disease. Nephron 65:185–189

    PubMed  Google Scholar 

  123. Kouhat EC, Edwards GA, Hill LL, Rosenberg HS, Hartley MW (1981) Focal glomerulosclerosis. Extent of involvement related to steroid resistance. Am J Clin Pathol 75:181–185

    PubMed  Google Scholar 

  124. Querfeld U, Waldherr R, Scharer K (1985) The significance of focal global sclerosis in idiopathic nephrotic syndrome: long-term clinical observations. Acta Paediatr Scand 74:913–919

    PubMed  Google Scholar 

  125. Schulman SL, Kaiser BA, Polinsky MS, Srinivasan R, Baluarter HJ (1988) Predicting the response to cytotoxic therapy for childhood nephrotic syndrome: superiority of response to corticosteroid therapy over histopathologic patterns. J Pediatr 113:996–1001

    PubMed  Google Scholar 

  126. Siegel NJ, Gaudio KM, Krassner LS, McDonald BM, Anderson FP, Kashgarian M (1981) Steroid-dependent nephrotic syndrome in children: histopathology and relapses after cyclophosphamide treatment. Kidney Int 19:454–459

    PubMed  Google Scholar 

  127. Holdsworth SR, Bellomo R (1984) Differential effects of steroids on leukocyte-mediated glomerulonephritis in the rabbit. Kidney Int 26:162–169

    PubMed  Google Scholar 

  128. Schreiber AD, Parson J, McDermott P, Cooper RA (1975) Effects of corticosteroids on human monocyte IgG and complement receptors. J Clin Invest 56:1189–1197

    PubMed  Google Scholar 

  129. Werb Z (1978) Biochemical actions of glucocorticoids on macrophages in culture. Specific inhibition of elastase, collagenase and plasminogen activator secretion and effects on other metabolic functions. J Exp Med 147:1685–1712

    Google Scholar 

  130. Fujiwara Y (1984) An ultrastructural study of the effect of steroid in puromycin aminonucleoside nephrosis rats. Virchow Arch [A] 405:11–24

    Google Scholar 

  131. Kawamura T, Yoshioka T, Bills T, Fogo A, Ichikawa I (1991) Glucocorticoid activates glomerular antioxidant enzymes and protects glomeruli from oxidant injuries. Kidney Int 40:291–301

    PubMed  Google Scholar 

  132. Ritz E (1994) Pathogenesis of “idiopathic” nephrotic syndrome. N Engl J Med 330:61–62

    PubMed  Google Scholar 

  133. Yoshioka T, Kawamura T, Meyrick BO, Beckman JK, Hoover RL, Yoshida H, Ichikawa I (1994) Induction of manganese superoxide dismutase by glucocorticoids in glomerular cells. Kidney Int 45:211–219

    PubMed  Google Scholar 

  134. Macconi D, Zanoli AF, Orisio S, Longaretti L, Magrini L, Rota S, Radice A, Pozzi C, Remuzzi G (1993) Methylprednisolone normalizes superoxide anion production by polymorphs from patients with ANCA-positive vasculitides. Kidney Int 44:215–220

    PubMed  Google Scholar 

  135. Tsunoda Y, Iwamoto N, Shiraga H, Kawaguchi H, Ito K, Nomura K, Shimizu S, Yoshioka T (1995) Down regulation of glucocorticoid receptor expressions in young patients with steroid-resistant nephrotic syndrome (abstract). J Am Soc Nephrol 6:433

    Google Scholar 

  136. Brodehl J (1986) Nephrotic syndrome in children. Diagnosis and treatment. World Pediatr Child Care 1:9–18

    Google Scholar 

  137. Mendoza SA, Reznik VM, Griswold WR, Krensky AM, Yorgin PD, Tune BM (1990) Treatment of steroid-resistant focal segmental glomerulosclerosis with pulse methylprednisolone and alkylating agents. Pediatr Nephrol 4:303–307

    PubMed  Google Scholar 

  138. International Study of Kidney Disease in Children (1982) Cyclophosphamide therapy in focal segmental glomerular sclerosis: a controlled clinical trial. Pediatr Res 16:320A

    Google Scholar 

  139. Arbeitsgemeinschaft für Pädiatrische Nephrologie (1982) Effect of cytotoxic drugs in frequently relapsing nephrotic syndrome with and without steroid dependence. N Engl J Med 306:451–454

    Google Scholar 

  140. Travis LB, Chesney R, McEnery P, Moel D, Pennisi A, Potter D, Talwalkar YB, Wolff E (1978) Growth and glucocorticoids in children with kidney disease. Kidney Int 14:365–368

    PubMed  Google Scholar 

  141. Lettgen B, Jeken C, Reiners C (1994) Influence of steroid medication on bone mineral density in children with nephrotic syndrome. Pediatr Nephrol 8:667–670

    PubMed  Google Scholar 

  142. Padilla R, Brem AS (1989) Linear growth of children with nephrotic syndrome: effect of alkylating agents. Pediatrics 84:495–499

    PubMed  Google Scholar 

  143. Korbet SM, Schwartz MM, Lewis EJ (1994) Primary focal segmental glomerulosclerosis: clinical course and response to therapy. Am J Kidney Dis 23:773–783

    PubMed  Google Scholar 

  144. Lentz RD, Bergstein J, Steffes MW, Brown DR, Prem K, Michael AF, Vernier RL (1977) Postpubertal evaluation of gonadal function following cyclophosphamide therapy before and during puberty. J Pediatr 91:385–394

    PubMed  Google Scholar 

  145. Watson AR, Taylor J, Rance CP, Bain J (1986) Gonadal function in women treated with cyclophosphamide for childhood nephrotic syndrome: a long-term follow-up study. Fertil Steril 46:331–333

    PubMed  Google Scholar 

  146. Bogdanovic R, Banicevic M, Cvoric A (1990) Pituitary-gonadal function in women following cyclophosphamide treatment for childhood nephrotic syndrome. Pediatr Nephrol 4:455–458

    PubMed  Google Scholar 

  147. Callis L, Nieto J, Vila A, Rende J (1980) Chlorambucil treatment in minimal lesion nephrotic syndrome. a reappraisal of its gonadal toxicity. J Pediatr 97:653–656

    PubMed  Google Scholar 

  148. Guesry P, Lenoir G, Broyer M (1978) Gonadal effects of chlorambucil given to prepubertal and pubertal boys for nephrotic syndrome. J Pediatr 92:299–303

    PubMed  Google Scholar 

  149. Lewis EJ (1988) Management of the nephrotic syndrome in adults. In: Cameron JS, Glassock RJ (eds) The nephrotic syndrome. Marcel Dekker, New York, pp 461–521

    Google Scholar 

  150. Baluarte HJ, Gruskin AB (1978) Chlorambucil dosage in frequently relapsing nephrotic syndrome: a controlled clinical trial. J Pediatr 92:295–298

    PubMed  Google Scholar 

  151. Almeida MP, Almeida HA, Rosa FC (1994) Vincristine in steroid-resistant nephrotic syndrome. Pediatr Nephrol 8:79–80

    PubMed  Google Scholar 

  152. Griswold WR, Tune BM, Reznik VM, Vazquez M, Prime DJ, Brock P, Mendoza SA (1987) Treatment of childhood prednisone-resistant nephrotic syndrome and focal segmental glomerulosclerosis with intravenous methylprednisolone and oral alkylating agents. Nephron 46:73–77

    PubMed  Google Scholar 

  153. Mendoza SA, Tune BM (1992) Treatment of childhood nephrotic syndrome. J Am Soc Nephrol 3:889–894

    PubMed  Google Scholar 

  154. Tejani A, Butt K, Trachtman H, Suthanthiran M, Rosenthal CJ, Khawar MR (1987) Cyclosporine-induced remission of relapsing nephrotic syndrome in children. J Pediatr 111:1056–1062

    PubMed  Google Scholar 

  155. Niaudet P (1994) Treatment of childhood steroid-resistant idiopathic nephrosis with a combination of cyclosporine and prednisone. French Society of Pediatric Nephrology. J Pediatr 125:981–986

    PubMed  Google Scholar 

  156. Melocoton TL, Kamil ES, Cohen AH, Fine RN (1991) Long-term cyclosporine A treatment of steroid-resistant and steroid-dependent nephrotic syndrome. Am J Kidney Dis 18:583–588

    PubMed  Google Scholar 

  157. Glassock RJ (1994) Role of cyclosporine in glomerular disease (review). Cleve Clin J Med 61:363–369

    PubMed  Google Scholar 

  158. Walker RG, Kincaid-Smith P (1990) The effect of treatment of corticosteroid-resistant idiopathic (primary) focal and segmental hyalinosis and sclerosis (focal glomulosclerosis) with ciclosporin. Nephron 54:117–121

    PubMed  Google Scholar 

  159. Windom HH, Fisher M, Neale TJ (1988) Cyclosporin therapy for steroid resistant nephrotic focal glomerulosclerosis. N Z Med J 103:125–126

    Google Scholar 

  160. Hymes LC (1995) Steroid-resistant, cyclosporine-responsive, relapsing nephrotic syndrome. Pediatr Nephrol 9:137–139

    PubMed  Google Scholar 

  161. Brodehl J (1991) Conventional therapy for idiopathic nephrotic syndrome in children. Cyclophosphamide for 12 weeks for those who are steroid-dependent. Clin Nephrol 35 [Suppl 1]:S8-S15

    PubMed  Google Scholar 

  162. Cattran D, Greenwood C, Hebert L, Pohl M, Hunsicker L, Maxwell D, Kunis C (1995) Follow up results from a randomized controlled trial (RCT) of cyclosporine (T) vs placebo (C) in adults with steroid resistant idiopathic focal segmental glomerulosclerosis (SR-FSGS) (abstract). J Am Soc Nephrol 6:414

    Google Scholar 

  163. Cattran D, Greenwood C, Bernstein K, Hebert L, Pohl M, Maxwell D, Appel G, Hoy W, Faber M, Hegeman R for the North American Nephrotic Syndrome Study Group (1995) Results of a 6 month randomized controlled trial of cyclosporine (T) vs placebo (C) in adults with steroid resistant idiopathic focal segmental glomerulosclerosis (SR-FSGS) (abstract). J Am Soc Nephrol 6:415

    Google Scholar 

  164. Tirelli AS, Paterlini G, Ghio L, Edefonti A, Assael BM, Bettinelli A, Cavanna G, Sereni F (1993) Renal effects of cyclosporin A in children treated for idiopathic nephrotic syndrome. Acta Paediatr 82:463–468

    PubMed  Google Scholar 

  165. Webb Kl, Sargent P, Burke JR, (1993) Cyclosporine therapy in steroid-dependent nephrotic syndrome. J Paediatr Child Health 29:188–191

    PubMed  Google Scholar 

  166. Niaudet P, Broyer M (1989) Cyclosporine in the therapy of idiopathic nephrotic syndrome in children. In: Andreucci VE, Fine LG, Hatano M, Kjellstrand CM (eds) International yearbook of nephrology. Kluwer, Boston, pp 55–168

    Google Scholar 

  167. Burdmann EA, Young B, Andoh TF, Evans A, Alpers CE, Lindsley J, Johnson RJ, Couser W (1994) Mechanisms of cyclosporine-induced interstitial fibrosis. Transplant Proc 26:2588–2589

    PubMed  Google Scholar 

  168. Hunley TE, Fogo A, Iwasaki S, Kon V (1995) Endothelin A receptor mediates functional but not structural damage in chronic cyclosporine nephrotoxicity. J Am Soc Nephrol 5:1718–1723

    PubMed  Google Scholar 

  169. Kon V, Hunley TE, Fogo A (1995) Combined antagonism of endothelin A/B receptors links endothelin to vasoconstriction whereas angiotensin II effects fibrosis. Transplantation 60:89–95

    PubMed  Google Scholar 

  170. Tufro-McReddie A, Alvarez E, Arrizurieta E, Repetto H (1992) Focal glomerulosclerosis in children: an Argentinian experience. Pediatr Nephrol 62:158–161

    Google Scholar 

  171. Praga M, Borstein B, Andres A, Ruilope LM, Rodicio JL (1991) Nephrotic proteinuria without hypoalbuminemia: clinical characteristics and response to angiotensin-converting enzyme inhibition. Am J Kidney Dis 17:330–338

    PubMed  Google Scholar 

  172. Cochat P, Kassir A, Colon S, Glastre C, Tourniaire B, Parchoux B, Martin X, David L (1993) Recurrent nephrotic syndrome after transplantation: early treatment with plasmaphaeresis and cyclophosphamide. Pediatr Nephrol 7:50–54

    PubMed  Google Scholar 

  173. McCauley J, Shapiro R, Ellis D, Igdal H, Tzakis A, Starzl TE (1993) Pilot trial of FK506 in the management of steroid-resistant nephrotic syndrome. Nephrol Dial Transplant 8: 1286–1290

    PubMed  Google Scholar 

  174. Guidi E, Magni M, Belgiojoso GB di, Minetti L, Bianchi G (1984) Blood pressure in patients with four different primary glomerulopathies. Clin Exp Hypertens [A] 6:1357–1366

    Google Scholar 

  175. Tanaka R, Kon V, Yoshioka T, Ichikawa I, Fogo A (1994) Angiotensin converting enzyme inhibitor modulates glomerular function and structure by distinct mechanisms. Kidney Int 45:537–543

    PubMed  Google Scholar 

  176. Kon V, Fogo A, Ichikawa I (1993) Bradykinin causes selective efferent arteriolar dilation during angiotensin I converting enzyme inhibition. Kidney Int 44:545–550

    PubMed  Google Scholar 

  177. Moorhead JF (1991) Lipids and progressive kidney disease. Kidney Int 39:S35-S40

    Google Scholar 

  178. Keane WF, Mulcahy WS, Kasiske BL, Kim Y, O'Donnell MP (1991) Hyperlipidemia and progressive renal disease. Kidney Int 39:S41-S48

    Google Scholar 

  179. Golper TA, Illingworth R, Morris CD, Bennet WM (1989) Lovastatin in the treatment of multifactorial hyperlipidemia associated with proteinuria. Am J Kidney Dis 13:312–320

    PubMed  Google Scholar 

  180. Shibasaki T, Nakano H, Matsuda H, Ohno I, Matsumonot H, Misawa T, Ishimoto F, Sakai O (1993) Pravastatin administration to hyperlipidemia in patients with nephrotic syndrome. J Jpn Soc Nephrol 35:1243–1248

    Google Scholar 

  181. Larsen ML, Illingworth DR (1994) Drug treatment of dyslipoproteinemia (review). Med Clin North Am 78:225–245

    PubMed  Google Scholar 

  182. Alkjaersig N, Fletcher AP, Narayanan M, Robson AM (1987) Course and resolution of the coagulopathy in nephrotic children. Kidney Int 31:772–780

    PubMed  Google Scholar 

  183. De Mattia D, Penza R, Giordano P, Di Bitonto G Altomare M, Del Vecchio GC, Schettini F (1991) Thromboembolic risk in children with nephrotic syndrome. Haemostasis. 21:300–304

    PubMed  Google Scholar 

  184. Purkerson ML, Hoffsten PE, Klahr S (1976) Pathogenesis of the glomerulopathy associated with renal infarction in rats. Kidney Int 9:407–417

    PubMed  Google Scholar 

  185. Homma T, Butt K, Trachtman H, Suthanthiran M, Rosenthal CJ, Khawar MR (1987) Cyclosporine-induced remission of relapsing nephrotic syndrome in children. J Pediatr 111:1056–1062

    PubMed  Google Scholar 

  186. Lines DR, Coleman, M, Gallus A (1989) Focal glomerulosclerosis treated with heparin. Arch Dis Child 64:855–857

    PubMed  Google Scholar 

  187. Tojo S, Marita M, Koyama A, Sano M, Suzuki H, Tsuchiya T, Tsuchida H, Yamamoto S, Shishido H (1978) Dipyridamole therapy in the nephrotic syndrome. Contrib Nephrol 9:111–127

    PubMed  Google Scholar 

  188. Zimmerman SW (1985) Plasmapheresis and dipyridamole for recurrent focal glomerulosclerosis. Nephron 40:241–245

    PubMed  Google Scholar 

  189. Donker AJ, Brentjens JR, Hem GK van der, Arisz L (1978) Treatment of the nephrotic syndrome with indomethacin. Nephron 22:374–381

    PubMed  Google Scholar 

  190. Alavi N, Lianos EA, Venuto RC, Mookerjee BK, Bentzel CJ (1986) Reduction of proteinuria by indomethacin in patients with nephrotic syndrome. Am J Kidney Dis 8:397–403

    PubMed  Google Scholar 

  191. Heeg JE, Jong PE de, Vriesendorp R, Zeeuw D de (1990) Additive antiproteinuric effect of the NSAID indomethacin and the ACE inhibitor lisinopril. Am J Nephrol 10 [Suppl 1]: 94–97

    PubMed  Google Scholar 

  192. Golbetz H, Black V, Shemesh O, Meyers BD (1989) Mechanism of the antiproteinuric effect of indomethacin in nephrotic humans. Am J Physiol 256:F44-F51

    PubMed  Google Scholar 

  193. Mathew TH (1988) Recurrence of disease following renal transplantation. Am J Kidney Dis 12:85–96

    PubMed  Google Scholar 

  194. Striegel JE, Sibley RK, Fryd DS, Mauer SM (1986) Recurrence of focal segmental sclerosis in children following renal transplantation. Kidney Int 30:S44-S50

    Google Scholar 

  195. Dantal J, Baatard R, Hourmant M, Cantarovich D, Buzelin F, Soulillou JP (1991) Recurrent nephrotic syndrome following renal transplantation in patients with focal glomerulosclerosis. A one-center study of plasma exchange effects. Fransplantation 52:827–831

    Google Scholar 

  196. Malekzadeh MH, Ettinger RB, Uittenbogaart CH, Fine RN (1979) Focal glomerulosclerosis and renal transplantation. J Pediatr 95:249–254

    PubMed  Google Scholar 

  197. Cameron JS (1982) Glomerulonephritis in renal transplants. Transplantation 34:237–245

    PubMed  Google Scholar 

  198. Mathew TH (1988) Nephrotic syndrome in patients with transplanted kidneys. In: Cameron JS, Glassock RJ (eds) The nephrotic syndrome. Marcel Dekker, New York, pp 805–848

    Google Scholar 

  199. Artero M, Biava C, Amend W, Tomlanovich S, Vincenti F (1992) Recurrent focal glomerulosclerosis: natural history and response to therapy. Am J Med 92:375–383

    PubMed  Google Scholar 

  200. Tejani A, Stablein DH (1992) Recurrence of focal segmental glomerulosclerosis posttransplantation: a special report of the North American Pediatric Renal Transplant Cooperative Study. J Am Soc Nephrol 2 [Suppl 3]:S258–263

    Google Scholar 

  201. Cheigh JS, Mauradian J, Solithan M, Tapia L, Riggio RR, Stenzel KH, Rubin, AL (1983) Focal segmental glomerulosclerosis in renal transplants. Am J Kidney Dis 4:449–455

    Google Scholar 

  202. Hosenpud J, Piering WF, Garancis JC, Kauffman HM (1985) Successful second kidney transplantation in a patient with focal glomerulosclerosis. A case report (review). Am J Nephrol 5:299–304

    PubMed  Google Scholar 

  203. Ramos EL, Tisher CC (1994) Recurrent disease in the kidney transplant. Am J Kidney Dis 24:142–154

    PubMed  Google Scholar 

  204. Ramos EL (1991) Recurrent diseases in the renal allograft. J Am Soc Nephrol 2:109–121

    PubMed  Google Scholar 

  205. Kashtan CE, McEnery PT, Tejani A, Stablein DM (1995) Renal allograft survival according to primary diagnosis: a report of the North American Pediatric Renal Transplant Cooperative Study. Pediatr Nephrol 9:679–684

    PubMed  Google Scholar 

  206. Hayes JM, Steinmuller DR, Streem SB, Novick AC (1991) The development of proteinuria and focal-segmental glomerulosclerosis in recipients of pediatric donor kidneys. Transplantation 52:813–817

    PubMed  Google Scholar 

  207. Pinto J, Lacerda G, Cameron JS, Turner DR, Bewick M, Ogg CS, and the Physicians, Pediatricians, and, Pathologists of the South East Thames Renal Units (1981) Recurrence of focal segmental glomerulosclerosis in renal allografts. Transplantation 32:83–89

    PubMed  Google Scholar 

  208. Maizel SE, Sibley RK, Horstman JP, Kjellstrand CM, Simmons RL (1981) Incidence and significance of recurrent focal segmental glomerulosclerosis in renal allograft recipients. Transplantation 32:512–516

    PubMed  Google Scholar 

  209. Axelsen RA, Seymour AE, Mathew TH, Fisher G, Canny A, Pascoe V (1984) Recurrent focal glomerulosclerosis in renal transplants. Clin Nephrol 21:110–114

    PubMed  Google Scholar 

  210. Ingulli E, Tejani A (1991) Incidence, treatment, and outcome of recurrent focal segmental glomerulosclerosis posttransplantation in 42 allografts in children — a single-center experience Transplantation 51:401–405

    PubMed  Google Scholar 

  211. Kershaw DB, Sedman AB, Kelsch RC, Bunchman TE (1994) Recurrent focal segmental glomerulosclerosis in pediatric renal transplant recipients: successful treatment with oral cyclophosphamide. Clin Transplant 8:546–549

    PubMed  Google Scholar 

  212. Habib R, Antignac C, Hinglais N (1987) Glomerular lesions in the transplanted kidney in children. Am J Kidney Dis 10:198–207

    PubMed  Google Scholar 

  213. Voets AJ, Hoitsma AJ, Koene RAP (1986) Recurrence of nephrotic syndrome during cyclosporin treatment after renal transplantation (letter). Lancet 1:266–267

    Google Scholar 

  214. Pirson Y, Squifflet JP, Marbaix E, Arenas J, Oliet A, Montoyo C, Ruilope LM, Rodicio JL (1986) Recurrence of focal glomerulosclerosis despite cyclosporine treatment after renal transplantation (letter). B M J 292:1336

    Google Scholar 

  215. Motojima M, Nishijima F, Ikoma M, Kawamura T, Yoshioka T, Fogo A, Sakai T, Ichikawa I (1991) Role for “urmic toxin” in the progressive loss of intact nephrons in chronic renal failure. Kidney Int 40:461–469

    PubMed  Google Scholar 

  216. Munoz J, Sanchez M, Perez-Garcia R, Anaya F, Valderrábano F (1985) Recurrent focal glomerulosclerosis in renal transplants, proteinuria relapsing following plasma exchange. Clin Nephrol 24:213–214

    Google Scholar 

  217. Newman WJ, Tisher CC, McCoy RC, Gunnells JC, Krueger RP, Clapp JR, Robinson R (1976) Focal glomerular sclerosis: contrasting clinical patterns in children and adults. Medicine (Baltimore). 55:67–87

    Google Scholar 

  218. Feld S, Figueroa P, Cha D, Savin V, Sharma R, Sharma M, Nast C, Hirschberg R, LaPage J, Adler SG (1995) Do circulating factors contribute to proteinuria (Up) and matrix (ECM) synthesis in the native kidneys of patients with idiopathic focal sclerosis (FSGS) (abstract)? J Am Soc Nephrol 6:417

    Google Scholar 

  219. Savin VS (1993) Mechanisms of proteinuria in noninflammatory glomerular diseases. Am J Kidney Dis 21:347–362

    PubMed  Google Scholar 

  220. Koyama A, Fujisaki M, Kobayashi M, Igarashi M, Narita M (1991) A glomerular permeability factor produced by human T cell hybridomas. Kidney Int 40:453–460

    PubMed  Google Scholar 

  221. Halevy J, Hayslett JP (1986) Clinical features and course of focal glomerulosclerosis. In: Mitch WE, Brenner BM, Stein JH (eds) The progressive nature of renal disease. Churchill Livingstone, New York, pp 189–201

    Google Scholar 

  222. Broyer M, Gagnadoux MF, Guest G, Beurton D, Niaudet P, Habib R, Busson M (1987) Kidney transplantation in children: results of 383 grafts performed at Enfants Malades Hospital from 1973 to 1984. Adv Nephrol 16:307–333

    Google Scholar 

  223. Tejani A, Nicastri AD, Sen D, Chen CK, Phadke K, Adamson O, Butt KMH (1983) Long-term evaluation of children with nephrotic syndrome and focal segmental glomerular sclerosis. Nephron 35:225–231

    PubMed  Google Scholar 

  224. Takeuchi A, Yoshizawa N, Kubota T, Niwa H (1989) A clinicopathologic study of focal segmental glomerulosclerosis: comparison between nephrotic and non-nephrotic focal segmental glomerulosclerosis. Jpn J Med 28:577–584

    PubMed  Google Scholar 

  225. Korbet SM, Schwartz MM, Lewis EJ (1986) The prognosis of focal segmental glomerulosclerosis of adulthood. Medicine (Baltimore) 65:304–311

    Google Scholar 

  226. Ingulli E, Tejani A (1991) Racial differences in the incidence and renal outcome of idiopathic focal segmental glomerulosclerosis in children. Pediatr Nephrol 5:393–397

    PubMed  Google Scholar 

  227. Wakai S, Magil AB (1992) Focal glomerulosclerosis in idiopathic membranous glomerulonephritis. Kidney Int 41:428–434

    PubMed  Google Scholar 

  228. Claris-Appiani A, Galato R, Marra G, Assael BM, Seveso M (1986) Prediction of the progression of renal failure in adult and in pediatric patients with malignant focal glomerulosclerosis. Clin Nephrol 26:87–90

    PubMed  Google Scholar 

  229. Imbasciati E, Ponticelli C, Case N, Altieri P, Bolasco F, Mihatsch MJ, Zollinger HU (1981) Acute renal failure in idiopathic nephrotic syndrome. Nephron 28:186–191

    PubMed  Google Scholar 

  230. Wehrmann M, Böhle A, Held H, Schumm G, Kendziora H, Pressler H (1990) Long-term prognosis of focal sclerosing glomerulonephritis: an analysis of 250 cases with particular regard to tubulointerstitial changes. Clin Nephrol 33:115–122

    PubMed  Google Scholar 

  231. Taube D, Chapman S, Brown Z, Williams DG (1981) Depression of normal lymphocyte transformation by sera of patients with minimal change nephropathy and other forms of nephrotic syndrome. Clin Nephrol 15:286–290

    PubMed  Google Scholar 

  232. Waldo FB, Benfield MR, Kohaut EC (1992) Methylprednisolone treatment of patients with steroid-resistant nephrotic syndrome. Pediatr Nephrol 6:503–505

    PubMed  Google Scholar 

  233. Sa GA, Luis JP, Mendonca E, Almeida M, Rosa FC (1996) Treatment of childhood steroid-resistant nephrotic syndrome with pulse methylprednisolone and cyclophosphamide. Pediatr Nephrol 10:250.

    PubMed  Google Scholar 

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  1. Division of Pediatric Nephrology, Vanderbilt University Medical Center, 21st & Garland Avenue, 37232, Nashville, TN, USA

    Iekuni Ichikawa & Agnes Fogo

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Ichikawa, I., Fogo, A. Focal segmental glomerulosclerosis. Pediatr Nephrol 10, 374–391 (1996). https://doi.org/10.1007/BF00866790

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