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Idiopathic Nephrotic Syndrome in Children: Clinical Aspects

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Abstract

In children, the most common cause of nephrotic syndrome is idiopathic nephrotic syndrome (INS), also called nephrosis (1). INS is defined by the combination of a nephrotic syndrome (proteinuria, hypoalbuminemia, hyperlipidemia, and edema) and non-specific histological abnormalities of the kidney including minimal changes, focal and segmental glomerular sclerosis (FSGS), and diffuse mesangial proliferation. Glomeruli show a fusion of epithelial cell foot processes on electron microscopy and no significant deposits of immunoglobulins or complement on immunofluorescence.

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References

  1. Eddy AA, Symons JM. Nephrotic syndrome in childhood. Lancet 2003;362(9384):629–639.

    Article  PubMed  Google Scholar 

  2. Kriz W, Elger M, Nagata M, Kretzler M, Uiker S, Koeppen-Hageman I et al. The role of podocytes in the development of glomerular sclerosis. Kidney Int Suppl 1994;45:S64–S72.

    Article  PubMed  CAS  Google Scholar 

  3. Bariety J, Bruneval P, Hill G, Irinopoulou T, Mandet C, Meyrier A. Posttransplantation relapse of FSGS is characterized by glomerular epithelial cell transdifferentiation. J Am Soc Nephrol 2001;12(2):261–274.

    PubMed  CAS  Google Scholar 

  4. Barisoni L, Mokrzycki M, Sablay L, Nagata M, Yamase H, Mundel P. Podocyte cell cycle regulation and proliferation in collapsing glomerulopathies. Kidney Int 2000;58(1):137–143.

    Article  PubMed  CAS  Google Scholar 

  5. Shankland SJ, Eitner F, Hudkins KL, Goodpaster T, D’Agati V, Alpers CE. Differential expression of cyclin-dependent kinase inhibitors in human glomerular disease: role in podocyte proliferation and maturation. Kidney Int 2000;58(2):674–683.

    Article  PubMed  CAS  Google Scholar 

  6. Moudgil A, Nast CC, Bagga A, Wei L, Nurmamet A, Cohen AH et al. Association of parvovirus B19 infection with idiopathic collapsing glomerulopathy. Kidney Int 2001;59(6):2126–2133.

    PubMed  CAS  Google Scholar 

  7. Tanawattanacharoen S, Falk RJ, Jennette JC, Kopp JB. Parvovirus B19 DNA in kidney tissue of patients with focal segmental glomerulosclerosis. Am J Kidney Dis 2000;35(6):1166–1174.

    Article  PubMed  CAS  Google Scholar 

  8. Strehlau J, Schachter AD, Pavlakis M, Singh A, Tejani A, Strom TB. Activated intrarenal transcription of CTL-effectors and TGF-beta1 in children with focal segmental glomerulosclerosis. Kidney Int 2002;61(1):90–95.

    Article  PubMed  CAS  Google Scholar 

  9. Kashgarian M, Hayslett JP, Siegel NJ. Lipoid nephrosis and focal sclerosis distinct entities or spectrum of disease. Nephron 1974;13(2):105–108.

    Article  PubMed  CAS  Google Scholar 

  10. Habib R, Gubler MC. Focal sclerosing glomerulonephritis. Perspect Nephrol Hypertens 1973;1(Pt 1):263–278.

    PubMed  Google Scholar 

  11. Siegel NJ, Gur A, Krassner LS, Kashgarian M. Minimal-lesion nephrotic syndrome with early resistance to steroid therapy. J Pediatr 1975;87(3):377–380.

    Article  PubMed  CAS  Google Scholar 

  12. McAdams AJ, Valentini RP, Welch TR. The nonspecificity of focal segmental glomerulosclerosis. The defining characteristics of primary focal glomerulosclerosis, mesangial proliferation, and minimal change. Medicine (Baltimore) 1997;76(1):42–52.

    Article  CAS  Google Scholar 

  13. Nephrotic syndrome in children: prediction of histopathology from clinical and laboratory characteristics at time of diagnosis. A report of the International Study of Kidney Disease in Children. Kidney Int 1978;13(2):159–165.

    Article  Google Scholar 

  14. McKinney PA, Feltbower RG, Brocklebank JT, Fitzpatrick MM. Time trends and ethnic patterns of childhood nephrotic syndrome in Yorkshire, UK. Pediatr Nephrol 2001;16(12):1040–1044.

    Article  PubMed  CAS  Google Scholar 

  15. Schlesinger ER, Sultz HA, Mosher WE, Feldman JG. The nephrotic syndrome. Its incidence and implications for the community. Am J Dis Child 1968;116(6):623–632.

    PubMed  CAS  Google Scholar 

  16. Wong W. Idiopathic nephrotic syndrome in New Zealand children, demographic, clinical features, initial management and outcome after twelve-month follow-up: results of a three-year national surveillance study. J Paediatr Child Health 2007;43(5):337–341.

    Article  PubMed  Google Scholar 

  17. Sharples PM, Poulton J, White RH. Steroid responsive nephrotic syndrome is more common in Asians. Arch Dis Child 1985;60(11):1014–1017.

    Article  PubMed  CAS  Google Scholar 

  18. Srivastava RN, Mayekar G, Anand R, Choudhry VP, Ghai OP, Tandon HD. Nephrotic syndrome in Indian children. Arch Dis Child 1975;50(8):626–630.

    Article  PubMed  CAS  Google Scholar 

  19. Anochie I, Eke F, Okpere A. Childhood nephrotic syndrome: change in pattern and response to steroids. J Natl Med Assoc 2006;98(12):1977–1981.

    PubMed  Google Scholar 

  20. Coovadia HM, Adhikari M, Morel-Maroger L. Clinico-pathological features of the nephrotic syndrome in South African children. Q J Med 1979;48(189):77–91.

    PubMed  CAS  Google Scholar 

  21. Doe JY, Funk M, Mengel M, Doehring E, Ehrich JH. Nephrotic syndrome in African children: lack of evidence for ‘tropical nephrotic syndrome’? Nephrol Dial Transplant 2006;21(3):672–676.

    Article  PubMed  Google Scholar 

  22. Cameron JS, Turner DR, Ogg CS, Sharpstone P, Brown CB. The nephrotic syndrome in adults with ‘minimal change’ glomerular lesions. Q J Med 1974;43(171):461–488.

    PubMed  CAS  Google Scholar 

  23. Hayslett JP, Kashgarian M, Bensch KG, Spargo BH, Freedman LR, Epstein FH. Clinicopathological correlations in the nephrotic syndrome due to primary renal disease. Medicine (Baltimore) 1973;52(2):93–120.

    Google Scholar 

  24. Meadow SR, Sarsfield JK, Scott DG, Rajah SM. Steroid-responsive nephrotic syndrome and allergy: immunological studies. Arch Dis Child 1981;56(7):517–524.

    Article  PubMed  CAS  Google Scholar 

  25. Salsano ME, Graziano L, Luongo I, Pilla P, Giordano M, Lama G. Atopy in childhood idiopathic nephrotic syndrome. Acta Paediatr 2007;96(4):561–566.

    Article  PubMed  Google Scholar 

  26. Thomson PD, Barratt TM, Stokes CR, Soothill JF, Turner MW. HLA typing and atopic features in steroid sensitive nephrotic syndrome of childhood. Monogr Allergy 1977;11:60.

    PubMed  CAS  Google Scholar 

  27. Laurent J, Rostoker G, Robeva R, Bruneau C, Lagrue G. Is adult idiopathic nephrotic syndrome food allergy? Value of oligoantigenic diets. Nephron 1987;47(1):7–11.

    Article  PubMed  CAS  Google Scholar 

  28. Audard V, Larousserie F, Grimbert P, Abtahi M, Sotto JJ, Delmer A et al. Minimal change nephrotic syndrome and classical Hodgkin’s lymphoma: report of 21 cases and review of the literature. Kidney Int 2006;69(12):2251–2260.

    Article  PubMed  CAS  Google Scholar 

  29. Eagen JW. Glomerulopathies of neoplasia. Kidney Int 1977;11(5):297–303.

    Article  PubMed  CAS  Google Scholar 

  30. Lee JC, Yamauchi H, Hopper J, Jr. The association of cancer and the nephrotic syndrome. Ann Intern Med 1966;64(1):41–51.

    PubMed  CAS  Google Scholar 

  31. Dede F, Ayli D, Atilgan KG, Yuksel C, Duranay M, Sener D et al. Focal segmental glomerulosclerosis associating Kimura disease. Ren Fail 2005;27(3):353–355.

    PubMed  Google Scholar 

  32. Nakahara C, Wada T, Kusakari J, Kanemoto K, Kinugasa H, Sibasaki M et al. Steroid-sensitive nephrotic syndrome associated with Kimura disease. Pediatr Nephrol 2000;14(6):482–485.

    Article  PubMed  CAS  Google Scholar 

  33. Rajpoot DK, Pahl M, Clark J. Nephrotic syndrome associated with Kimura disease. Pediatr Nephrol 2000;14(6):486–488.

    Article  PubMed  CAS  Google Scholar 

  34. Habib R, Kleinknecht C. The primary nephrotic syndrome of childhood. Classification and clinicopathologic study of 406 cases. Pathol Annu 1971;6:417–474.

    PubMed  CAS  Google Scholar 

  35. Alwadhi RK, Mathew JL, Rath B. Clinical profile of children with nephrotic syndrome not on glucorticoid therapy, but presenting with infection. J Paediatr Child Health 2004;40(1–2):28–32.

    Article  PubMed  CAS  Google Scholar 

  36. Elises JS, Griffiths PD, Hocking MD, Taylor CM, White RH. Simplified quantification of urinary protein excretion in children. Clin Nephrol 1988;30(4):225–229.

    PubMed  CAS  Google Scholar 

  37. Joven J, Villabona C, Vilella E, Masana L, Alberti R, Valles M. Abnormalities of lipoprotein metabolism in patients with the nephrotic syndrome. N Engl J Med 1990;323(9):579–584.

    Article  PubMed  CAS  Google Scholar 

  38. Thabet MA, Salcedo JR, Chan JC. Hyperlipidemia in childhood nephrotic syndrome. Pediatr Nephrol 1993;7(5):559–566.

    Article  PubMed  CAS  Google Scholar 

  39. Vaziri ND. Molecular mechanisms of lipid disorders in nephrotic syndrome. Kidney Int 2003;63(5):1964–1976.

    Article  PubMed  Google Scholar 

  40. Sato KA, Gray RW, Lemann J, Jr. Urinary excretion of 25-hydroxyvitamin D in health and the nephrotic syndrome. J Lab Clin Med 1982;99(3):325–330.

    PubMed  CAS  Google Scholar 

  41. Freundlich M, Bourgoignie JJ, Zilleruelo G, Abitbol C, Canterbury JM, Strauss J. Calcium and vitamin D metabolism in children with nephrotic syndrome. J Pediatr 1986;108(3):383–387.

    Article  PubMed  CAS  Google Scholar 

  42. McVicar M, Exeni R, Susin M. Nephrotic syndrome and multiple tubular defects in children: an early sign of focal segmental glomerulosclerosis. J Pediatr 1980;97(6):918–922.

    Article  PubMed  CAS  Google Scholar 

  43. Rodriguez-Soriano J, Vallo A, Castillo G, Oliveros R. Defect in urinary acidification in nephrotic syndrome and its correction by furosemide. Nephron 1982;32(4):308–313.

    Article  PubMed  CAS  Google Scholar 

  44. Vaziri ND, Kaupke CJ, Barton CH, Gonzales E. Plasma concentration and urinary excretion of erythropoietin in adult nephrotic syndrome. Am J Med 1992;92(1):35–40.

    Article  PubMed  CAS  Google Scholar 

  45. Cavagnaro F, Lagomarsino E. Peritonitis as a risk factor of acute renal failure in nephrotic children. Pediatr Nephrol 2000;15(3–4):248–251.

    Article  PubMed  CAS  Google Scholar 

  46. Vande Walle J, Mauel R, Raes A, Vandekerckhove K, Donckerwolcke R. ARF in children with minimal change nephrotic syndrome may be related to functional changes of the glomerular basal membrane. Am J Kidney Dis 2004;43(3):399–404.

    Article  PubMed  Google Scholar 

  47. Bohman SO, Jaremko G, Bohlin AB, Berg U. Foot process fusion and glomerular filtration rate in minimal change nephrotic syndrome. Kidney Int 1984;25(4):696–700.

    Article  PubMed  CAS  Google Scholar 

  48. Sakarcan A, Timmons C, Seikaly MG. Reversible idiopathic acute renal failure in children with primary nephrotic syndrome. J Pediatr 1994;125(5 Pt 1):723–727.

    PubMed  CAS  Google Scholar 

  49. Fliser D, Zurbruggen I, Mutschler E, Bischoff I, Nussberger J, Franek E et al. Coadministration of albumin and furosemide in patients with the nephrotic syndrome. Kidney Int 1999;55(2):629–634.

    Article  PubMed  CAS  Google Scholar 

  50. Dorhout EJ, Roos JC, Boer P, Yoe OH, Simatupang TA. Observations on edema formation in the nephrotic syndrome in adults with minimal lesions. Am J Med 1979;67(3):378–384.

    Article  PubMed  CAS  Google Scholar 

  51. Raij L, Keane WF, Leonard A, Shapiro FL. Irreversible acute renal failure in idiopathic nephrotic syndrome. Am J Med 1976;61(2):207–214.

    Article  PubMed  CAS  Google Scholar 

  52. Focal segmental glomerulosclerosis in children with idiopathic nephrotic syndrome. A report of the Southwest Pediatric Nephrology Study Group. Kidney Int 1985;27(2):442–449.

    Article  Google Scholar 

  53. Paik KH, Lee BH, Cho HY, Kang HG, Ha IS, Cheong HI et al. Primary focal segmental glomerular sclerosis in children: clinical course and prognosis. Pediatr Nephrol 2007;22(3):389–395.

    Article  PubMed  Google Scholar 

  54. Ingulli E, Tejani A. Racial differences in the incidence and renal outcome of idiopathic focal segmental glomerulosclerosis in children. Pediatr Nephrol 1991;5(4):393–397.

    Article  PubMed  CAS  Google Scholar 

  55. Feinstein EI, Kaptein EM, Nicoloff JT, Massry SG. Thyroid function in patients with nephrotic syndrome and normal renal function. Am J Nephrol 1982;2(2):70–76.

    Article  PubMed  CAS  Google Scholar 

  56. Garin EH, Grant MB, Silverstein JH. Insulinlike growth factors in patients with active nephrotic syndrome. Am J Dis Child 1989;143(7):865–867.

    PubMed  CAS  Google Scholar 

  57. Krensky AM, Ingelfinger JR, Grupe WE. Peritonitis in childhood nephrotic syndrome: 1970–1980. Am J Dis Child 1982;136(8):732–736.

    PubMed  CAS  Google Scholar 

  58. Andrew M, Brooker LA. Hemostatic complications in renal disorders of the young. Pediatr Nephrol 1996;10(1):88–99.

    PubMed  CAS  Google Scholar 

  59. Lilova MI, Velkovski IG, Topalov IB. Thromboembolic complications in children with nephrotic syndrome in Bulgaria (1974–1996). Pediatr Nephrol 2000;15(1–2):74–78.

    Article  PubMed  CAS  Google Scholar 

  60. Hoyer PF, Gonda S, Barthels M, Krohn HP, Brodehl J. Thromboembolic complications in children with nephrotic syndrome. Risk and incidence. Acta Paediatr Scand 1986;75(5):804–810.

    Article  PubMed  CAS  Google Scholar 

  61. Churg J, Habib R, White RH. Pathology of the nephrotic syndrome in children: a report for the International Study of Kidney Disease in Children. Lancet 1970;760(1):1299–1302.

    Article  PubMed  CAS  Google Scholar 

  62. Powell HR. Relationship between proteinuria and epithelial cell changes in minimal lesion glomerulopathy. Nephron 1976;16(4):310–317.

    Article  PubMed  CAS  Google Scholar 

  63. Kawano K, Wenzl J, McCoy J, Porch J, Kimmelstiel P. Lipoid nephrosis. A multifold blind study, including quantitation. Lab Invest 1971;24(6):499–503.

    PubMed  CAS  Google Scholar 

  64. Waldherr R, Gubler MC, Levy M, Broyer M, Habib R. The significance of pure diffuse mesangial proliferation in idiopathic nephrotic syndrome. Clin Nephrol 1978;10(5):171–179.

    PubMed  CAS  Google Scholar 

  65. White RH, Glasgow EF, Mills RJ. Clinicopathological study of nephrotic syndrome in childhood. Lancet 1970;1(7661):1353–1359.

    Article  PubMed  CAS  Google Scholar 

  66. Yoshikawa N, Ito H, Akamatsu R, Matsuyama S, Hasegawa O, Nakahara C et al. Focal segmental glomerulosclerosis with and without nephrotic syndrome in children. J Pediatr 1986;109(1):65–70.

    Article  PubMed  CAS  Google Scholar 

  67. Morita M, White RH, Coad NA, Raafat F. The clinical significance of the glomerular location of segmental lesions in focal segmental glomerulosclerosis. Clin Nephrol 1990;33(5):211–219.

    PubMed  CAS  Google Scholar 

  68. Howie AJ, Brewer DB. The glomerular tip lesion: a previously undescribed type of segmental glomerular abnormality. J Pathol 1984;142(3):205–220.

    Article  PubMed  CAS  Google Scholar 

  69. Ito H, Yoshikawa N, Aozai F, Hazikano H, Sakaguchi H, Akamatsu R et al. Twenty-seven children with focal segmental glomerulosclerosis: correlation between the segmental location of the glomerular lesions and prognosis. Clin Nephrol 1984;22(1):9–14.

    PubMed  CAS  Google Scholar 

  70. Huppes W, Hene RJ, Kooiker CJ. The glomerular tip lesion: a distinct entity or not? J Pathol 1988;154(2):187–190.

    Article  PubMed  CAS  Google Scholar 

  71. Deegens JK, Steenbergen EJ, Borm GF, Wetzels JF. Pathological variants of focal segmental glomerulosclerosis in an adult Dutch population – epidemiology and outcome. Nephrol Dial Transplant 2008;23(1):186–192.

    Article  PubMed  Google Scholar 

  72. Thomas DB, Franceschini N, Hogan SL, Ten Holder S, Jennette CE, Falk RJ et al. Clinical and pathologic characteristics of focal segmental glomerulosclerosis pathologic variants. Kidney Int 2006;69(5):920–926.

    Article  PubMed  CAS  Google Scholar 

  73. Fogo A, Hawkins EP, Berry PL, Glick AD, Chiang ML, MacDonell RC, Jr. et al. Glomerular hypertrophy in minimal change disease predicts subsequent progression to focal glomerular sclerosis. Kidney Int 1990;38(1):115–123.

    Article  PubMed  CAS  Google Scholar 

  74. Muda AO, Feriozzi S, Cinotti GA, Faraggiana T. Glomerular hypertrophy and chronic renal failure in focal segmental glomerulosclerosis. Am J Kidney Dis 1994;23(2):237–241.

    PubMed  CAS  Google Scholar 

  75. Jenis EH, Teichman S, Briggs WA, Sandler P, Hollerman CE, Calcagno PL et al. Focal segmental glomerulosclerosis. Am J Med 1974;57(5):695–705.

    Article  PubMed  CAS  Google Scholar 

  76. Erkan E, Garcia CD, Patterson LT, Mishra J, Mitsnefes MM, Kaskel FJ et al. Induction of renal tubular cell apoptosis in focal segmental glomerulosclerosis: roles of proteinuria and Fas-dependent pathways. J Am Soc Nephrol 2005;16(2):398–407.

    Article  PubMed  CAS  Google Scholar 

  77. Hyman LR, Burkholder PM. Focal sclerosing glomerulonephropathy with segmental hyalinosis. A clinicopathologic analysis. Lab Invest 1973;28(5):533–544.

    PubMed  CAS  Google Scholar 

  78. Nagi AH, Alexander F, Lannigan R. Light and electron microscopical studies of focal glomerular sclerosis. J Clin Pathol 1971;24(9):846–850.

    Article  PubMed  CAS  Google Scholar 

  79. Grishman E, Churg J. Focal glomerular sclerosis in nephrotic patients: an electron microscopic study of glomerular podocytes. Kidney Int 1975;7(2):111–122.

    Article  PubMed  CAS  Google Scholar 

  80. D’Agati VD, Fogo AB, Bruijn JA, Jennette JC. Pathologic classification of focal segmental glomerulosclerosis: a working proposal. Am J Kidney Dis 2004;43(2):368–382.

    Article  PubMed  Google Scholar 

  81. Abeyagunawardena AS, Sebire NJ, Risdon RA, Dillon MJ, Rees L, Van’t Hoff W et al. Predictors of long-term outcome of children with idiopathic focal segmental glomerulosclerosis. Pediatr Nephrol 2007;22(2):215–221.

    Article  PubMed  Google Scholar 

  82. Weiss MA, Daquioag E, Margolin EG, Pollak VE. Nephrotic syndrome, progressive irreversible renal failure, and glomerular “collapse”: a new clinicopathologic entity? Am J Kidney Dis 1986;7(1):20–28.

    PubMed  CAS  Google Scholar 

  83. Detwiler RK, Falk RJ, Hogan SL, Jennette JC. Collapsing glomerulopathy: a clinically and pathologically distinct variant of focal segmental glomerulosclerosis. Kidney Int 1994;45(5):1416–1424.

    Article  PubMed  CAS  Google Scholar 

  84. Nagata M, Hattori M, Hamano Y, Ito K, Saitoh K, Watanabe T. Origin and phenotypic features of hyperplastic epithelial cells in collapsing glomerulopathy. Am J Kidney Dis 1998;32(6):962–969.

    Article  PubMed  CAS  Google Scholar 

  85. Toth CM, Pascual M, Williams WW, Jr., Delmonico FL, Cosimi AB, Colvin RB et al. Recurrent collapsing glomerulopathy. Transplantation 1998;65(7):1009–1010.

    Article  PubMed  CAS  Google Scholar 

  86. Valeri A, Barisoni L, Appel GB, Seigle R, D’Agati V. Idiopathic collapsing focal segmental glomerulosclerosis: a clinicopathologic study. Kidney Int 1996;50(5):1734–1746.

    Article  PubMed  CAS  Google Scholar 

  87. Nash MA, Greifer I, Olbing H, Bernstein J, Bennett B, Spitzer A. The significance of focal sclerotic lesions of glomeruli in children. J Pediatr 1976;88(5):806–813.

    Article  PubMed  CAS  Google Scholar 

  88. Couser WG, Stilmant MM. Mesangial lesions and focal glomerular sclerosis in the aging rat. Lab Invest 1975;33(5):491–501.

    PubMed  CAS  Google Scholar 

  89. Jao W, Pollak VE, Norris SH, Lewy P, Pirani CL. Lipoid nephrosis: an approach to the clinicopathologic analysis and dismemberment of idiopathic nephrotic syndrome with minimal glomerular changes. Medicine (Baltimore) 1973;52(5):445–468.

    Article  CAS  Google Scholar 

  90. Michael AF, McLean RH, Roy LP, Westberg NG, Hoyer JR, Fish AJ et al. Immunologic aspects of the nephrotic syndrome. Kidney Int 1973;3(2):105–115.

    Article  PubMed  CAS  Google Scholar 

  91. Cohen AH, Border WA, Glassock RJ. Nephrotic syndrome with glomerular mesangial IgM deposits. Lab Invest 1978;38(5):610–619.

    PubMed  CAS  Google Scholar 

  92. Habib R, Girardin E, Gagnadoux MF, Hinglais N, Levy M, Broyer M. Immunopathological findings in idiopathic nephrosis: clinical significance of glomerular “immune deposits”. Pediatr Nephrol 1988;2(4):402–408.

    Article  PubMed  CAS  Google Scholar 

  93. Association of IgA nephropathy with steroid-responsive nephrotic syndrome. A report of the Southwest Pediatric Nephrology Study Group. Am J Kidney Dis 1985;5(3):157–164.

    Google Scholar 

  94. Lai KN, Lai FM, Chan KW, Ho CP, Leung AC, Vallance-Owen J. An overlapping syndrome of IgA nephropathy and lipoid nephrosis. Am J Clin Pathol 1986;86(6):716–723.

    PubMed  CAS  Google Scholar 

  95. Tejani A. Morphological transition in minimal change nephrotic syndrome. Nephron 1985;39(3):157–159.

    Article  PubMed  CAS  Google Scholar 

  96. Hirszel P, Yamase HT, Carney WR, Galen MA, Graeber CW, Johnson KJ et al. Mesangial proliferative glomerulonephritis with IgM deposits. Clinicopathologic analysis and evidence for morphologic transitions. Nephron 1984;38(2):100–108.

    Article  PubMed  CAS  Google Scholar 

  97. The primary nephrotic syndrome in children. Identification of patients with minimal change nephrotic syndrome from initial response to prednisone. A report of the International Study of Kidney Disease in Children. J Pediatr 1981;98(4):561–564.

    Article  Google Scholar 

  98. Boyer O, Moulder JK, Somers MJ. Focal and segmental glomerulosclerosis in children: a longitudinal assessment. Pediatr Nephrol 2007;22(8):1159–1166.

    Article  PubMed  Google Scholar 

  99. Robson AM, Giangiacomo J, Kienstra RA, Naqvi ST, Ingelfinger JR. Normal glomerular permeability and its modification by minimal change nephrotic syndrmone. J Clin Invest 1974;54(5):1190–1199.

    Article  PubMed  CAS  Google Scholar 

  100. Carrie BJ, Salyer WR, Myers BD. Minimal change nephropathy: an electrochemical disorder of the glomerular membrane. Am J Med 1981;70(2):262–268.

    Article  PubMed  CAS  Google Scholar 

  101. Kitano Y, Yoshikawa N, Nakamura H. Glomerular anionic sites in minimal change nephrotic syndrome and focal segmental glomerulosclerosis. Clin Nephrol 1993;40(4):199–204.

    PubMed  CAS  Google Scholar 

  102. Blau EB, Haas JE. Glomerular sialic acid and proteinuria in human renal disease. Lab Invest 1973;28(4):477–481.

    PubMed  CAS  Google Scholar 

  103. van den Born J, van den Heuvel LP, Bakker MA, Veerkamp JH, Assmann KJ, Berden JH. A monoclonal antibody against GBM heparan sulfate induces an acute selective proteinuria in rats. Kidney Int 1992;41(1):115–123.

    Article  PubMed  Google Scholar 

  104. Boulton-Jones JM, McWilliams G, Chandrachud L. Variation in charge on red cells of patients with different glomerulopathies. Lancet 1986;2(8500):186–189.

    Article  PubMed  CAS  Google Scholar 

  105. Levin M, Smith C, Walters MD, Gascoine P, Barratt TM. Steroid-responsive nephrotic syndrome: a generalised disorder of membrane negative charge. Lancet 1985;2(8449):239–242.

    Article  PubMed  CAS  Google Scholar 

  106. Shalhoub RJ. Pathogenesis of lipoid nephrosis: a disorder of T-cell function. Lancet 1974;2(7880):556–560.

    Article  PubMed  CAS  Google Scholar 

  107. Garin EH. Circulating mediators of proteinuria in idiopathic minimal lesion nephrotic syndrome. Pediatr Nephrol 2000;14(8–9):872–878.

    Article  PubMed  CAS  Google Scholar 

  108. Ritz E. Pathogenesis of “idiopathic” nephrotic syndrome. N Engl J Med 1994;330(1):61–62.

    Article  PubMed  CAS  Google Scholar 

  109. Savin VJ, Sharma R, Sharma M, McCarthy ET, Swan SK, Ellis E et al. Circulating factor associated with increased glomerular permeability to albumin in recurrent focal segmental glomerulosclerosis. N Engl J Med 1996;334(14):878–883.

    Article  PubMed  CAS  Google Scholar 

  110. Hoyer JR, Vernier RL, Najarian JS, Raij L, Simmons RL, Michael AF. Recurrence of idiopathic nephrotic syndrome after renal transplantation. Lancet 1972;2(7773):343–348.

    Article  PubMed  CAS  Google Scholar 

  111. Ali AA, Wilson E, Moorhead JF, Amlot P, Abdulla A, Fernando ON et al. Minimal-change glomerular nephritis. Normal kidneys in an abnormal environment? Transplantation 1994;58(7):849–852.

    PubMed  CAS  Google Scholar 

  112. Lagrue G, Niaudet P, Guillot F, Lang P. Pregnancy and glomerulonephritis. Lancet 1989;2(8670):1037.

    Article  PubMed  CAS  Google Scholar 

  113. Zimmerman SW. Increased urinary protein excretion in the rat produced by serum from a patient with recurrent focal glomerular sclerosis after renal transplantation. Clin Nephrol 1984;22(1):32–38.

    PubMed  CAS  Google Scholar 

  114. Le Berre L, Godfrin Y, Gunther E, Buzelin F, Perretto S, Smit H et al. Extrarenal effects on the pathogenesis and relapse of idiopathic nephrotic syndrome in Buffalo/Mna rats. J Clin Invest 2002;109(4):491–498.

    PubMed  CAS  Google Scholar 

  115. Tesar V, Zima T. Recent progress in the pathogenesis of nephrotic proteinuria. Crit Rev Clin Lab Sci 2008;45(2):139–220.

    Article  PubMed  CAS  Google Scholar 

  116. Lagrue G, Branellec A, Blanc C, Xheneumont S, Beaudoux F, Sobel A et al. A vascular permeability factor in lymphocyte culture supernants from patients with nephrotic syndrome. II. Pharmacological and physicochemical properties. Biomedicine 1975;23(2):73–75.

    PubMed  CAS  Google Scholar 

  117. Heslan JM, Branellec A, Laurent J, Lagrue G. The vascular permeability factor is a T lymphocyte product. Nephron 1986;42(2):187–188.

    Article  PubMed  CAS  Google Scholar 

  118. Sobel AT, Branellec AI, Blanc CJ, Lagrue GA. Physicochemical characterization of a vascular permeability factor produced by con A-stimulated human lymphocytes. J Immunol 1977;119(4):1230–1234.

    PubMed  CAS  Google Scholar 

  119. Maruyama K, Tomizawa S, Shimabukuro N, Fukuda T, Johshita T, Kuroume T. Effect of supernatants derived from T lymphocyte culture in minimal change nephrotic syndrome on rat kidney capillaries. Nephron 1989;51(1):73–76.

    Article  PubMed  CAS  Google Scholar 

  120. Tanaka R, Yoshikawa N, Nakamura H, Ito H. Infusion of peripheral blood mononuclear cell products from nephrotic children increases albuminuria in rats. Nephron 1992;60(1):35–41.

    Article  PubMed  CAS  Google Scholar 

  121. Koyama A, Fujisaki M, Kobayashi M, Igarashi M, Narita M. A glomerular permeability factor produced by human T cell hybridomas. Kidney Int 1991;40(3):453–460.

    Article  PubMed  CAS  Google Scholar 

  122. Dantal J, Bigot E, Bogers W, Testa A, Kriaa F, Jacques Y et al. Effect of plasma protein adsorption on protein excretion in kidney-transplant recipients with recurrent nephrotic syndrome. N Engl J Med 1994;330(1):7–14.

    Article  PubMed  CAS  Google Scholar 

  123. Dantal J, Godfrin Y, Koll R, Perretto S, Naulet J, Bouhours JF et al. Antihuman immunoglobulin affinity immunoadsorption strongly decreases proteinuria in patients with relapsing nephrotic syndrome. J Am Soc Nephrol 1998;9(9):1709–1715.

    PubMed  CAS  Google Scholar 

  124. Musante L, Candiano G, Bruschi M, Zennaro C, Carraro M, Artero M et al. Characterization of plasma factors that alter the permeability to albumin within isolated glomeruli. Proteomics 2002;2(2):197–205.

    Article  PubMed  CAS  Google Scholar 

  125. Bakker WW, van Luijk WH. Do circulating factors play a role in the pathogenesis of minimal change nephrotic syndrome? Pediatr Nephrol 1989;3(3):341–349.

    Article  PubMed  CAS  Google Scholar 

  126. Heslan JM, Branellec AI, Pilatte Y, Lang P, Lagrue G. Differentiation between vascular permeability factor and IL-2 in lymphocyte supernatants from patients with minimal-change nephrotic syndrome. Clin Exp Immunol 1991;86(1):157–162.

    Article  PubMed  CAS  Google Scholar 

  127. Yap HK, Cheung W, Murugasu B, Sim SK, Seah CC, Jordan SC. Th1 and Th2 cytokine mRNA profiles in childhood nephrotic syndrome: evidence for increased IL-13 mRNA expression in relapse. J Am Soc Nephrol 1999;10(3):529–537.

    PubMed  CAS  Google Scholar 

  128. Sahali D, Pawlak A, Valanciute A, Grimbert P, Lang P, Remy P et al. A novel approach to investigation of the pathogenesis of active minimal-change nephrotic syndrome using subtracted cDNA library screening. J Am Soc Nephrol 2002;13(5):1238–1247.

    PubMed  CAS  Google Scholar 

  129. Wei CL, Cheung W, Heng CK, Arty N, Chong SS, Lee BW et al. Interleukin-13 genetic polymorphisms in Singapore Chinese children correlate with long-term outcome of minimal-change disease. Nephrol Dial Transplant 2005;20(4):728–734.

    Article  PubMed  CAS  Google Scholar 

  130. Van Den Berg JG, Aten J, Chand MA, Claessen N, Dijkink L, Wijdenes J et al. Interleukin-4 and interleukin-13 act on glomerular visceral epithelial cells. J Am Soc Nephrol 2000;11(3):413–422.

    Google Scholar 

  131. Sahali D, Pawlak A, Le Gouvello S, Lang P, Valanciute A, Remy P et al. Transcriptional and post-transcriptional alterations of IkappaBalpha in active minimal-change nephrotic syndrome. J Am Soc Nephrol 2001;12(8):1648–1658.

    PubMed  CAS  Google Scholar 

  132. Grimbert P, Audard V, Remy P, Lang P, Sahali D. Recent approaches to the pathogenesis of minimal-change nephrotic syndrome. Nephrol Dial Transplant 2003;18(2):245–248.

    Article  PubMed  CAS  Google Scholar 

  133. Araya CE, Wasserfall CH, Brusko TM, Mu W, Segal MS, Johnson RJ et al. A case of unfulfilled expectations. Cytokines in idiopathic minimal lesion nephrotic syndrome. Pediatr Nephrol 2006;21(5):603–610.

    Article  PubMed  Google Scholar 

  134. Bustos C, Gonzalez E, Muley R, Alonso JL, Egido J. Increase of tumour necrosis factor alpha synthesis and gene expression in peripheral blood mononuclear cells of children with idiopathic nephrotic syndrome. Eur J Clin Invest 1994;24(12):799–805.

    Article  PubMed  CAS  Google Scholar 

  135. Daniel V, Trautmann Y, Konrad M, Nayir A, Scharer K. T-lymphocyte populations, cytokines and other growth factors in serum and urine of children with idiopathic nephrotic syndrome. Clin Nephrol 1997;47(5):289–297.

    PubMed  CAS  Google Scholar 

  136. Suranyi MG, Guasch A, Hall BM, Myers BD. Elevated levels of tumor necrosis factor-alpha in the nephrotic syndrome in humans. Am J Kidney Dis 1993;21(3):251–259.

    PubMed  CAS  Google Scholar 

  137. Neuhaus TJ, Wadhwa M, Callard R, Barratt TM. Increased IL-2, IL-4 and interferon-gamma (IFN-gamma) in steroid-sensitive nephrotic syndrome. Clin Exp Immunol 1995;100(3):475–479.

    Article  PubMed  CAS  Google Scholar 

  138. Shimoyama H, Nakajima M, Naka H, Maruhashi Y, Akazawa H, Ueda T et al. Up-regulation of interleukin-2 mRNA in children with idiopathic nephrotic syndrome. Pediatr Nephrol 2004;19(10):1115–1121.

    Article  PubMed  Google Scholar 

  139. Lama G, Luongo I, Tirino G, Borriello A, Carangio C, Salsano ME. T-lymphocyte populations and cytokines in childhood nephrotic syndrome. Am J Kidney Dis 2002;39(5):958–965.

    Article  PubMed  CAS  Google Scholar 

  140. Hulton SA, Shah V, Byrne MR, Morgan G, Barratt TM, Dillon MJ. Lymphocyte subpopulations, interleukin-2 and interleukin-2receptor expression in childhood nephrotic syndrome. Pediatr Nephrol 1994;8(2):135–139.

    Article  PubMed  CAS  Google Scholar 

  141. Kemper MJ, Meyer-Jark T, Lilova M, Muller-Wiefel DE. Combined T- and B-cell activation in childhood steroid-sensitive nephrotic syndrome. Clin Nephrol 2003;60(4):242–247.

    PubMed  CAS  Google Scholar 

  142. Mandreoli M, Beltrandi E, Casadei-Maldini M, Mancini R, Zucchelli A, Zucchelli P. Lymphocyte release of soluble IL-2 receptors in patients with minimal change nephropathy. Clin Nephrol 1992;37(4):177–182.

    PubMed  CAS  Google Scholar 

  143. Wada T, Yokoyama H, Tomosugi N, Hisada Y, Ohta S, Naito T et al. Detection of urinary interleukin-8 in glomerular diseases. Kidney Int 1994;46(2):455–460.

    Article  PubMed  CAS  Google Scholar 

  144. Garin EH, Blanchard DK, Matsushima K, Djeu JY. IL-8 production by peripheral blood mononuclear cells in nephrotic patients. Kidney Int 1994;45(5):1311–1317.

    Article  PubMed  CAS  Google Scholar 

  145. Kemper MJ, MeyerJark T, Lilova M, MullerWiefel DE. Combined T- and B-cell activation in childhood steroid-sensitive nephrotic syndrome. Clin Nephrol 2003;60(4):242–247.

    PubMed  CAS  Google Scholar 

  146. Topaloglu R, Saatci U, Arikan M, Canpinar H, Bakkaloglu A, Kansu E. T-cell subsets, interleukin-2 receptor expression and production of interleukin-2 in minimal change nephrotic syndrome. Pediatr Nephrol 1994;8(6):649–652.

    Article  PubMed  CAS  Google Scholar 

  147. Saxena S, Mittal A, Andal A. Pattern of interleukins in minimal-change nephrotic syndrome of childhood. Nephron 1993;65(1):56–61.

    Article  PubMed  CAS  Google Scholar 

  148. Hinoshita F, Noma T, Tomura S, Shiigai T, Yata J. Decreased production and responsiveness of interleukin 2 in lymphocytes of patients with nephrotic syndrome. Nephron 1990;54(2):122–126.

    Article  PubMed  CAS  Google Scholar 

  149. Stachowski J, Barth C, Michalkiewicz J, Krynicki T, Jarmolinski T, Runowski D et al. Th1/Th2 balance and CD45-positive T cell subsets in primary nephrotic syndrome. Pediatr Nephrol 2000;14(8–9):779–785.

    Article  PubMed  CAS  Google Scholar 

  150. Stefanovic V, Golubovic E, Mitic-Zlatkovic M, Vlahovic P, Jovanovic O, Bogdanovic R. Interleukin-12 and interferon-gamma production in childhood idiopathic nephrotic syndrome. Pediatr Nephrol 1998;12(6):463–466.

    Article  PubMed  CAS  Google Scholar 

  151. Cho BS, Yoon SR, Jang JY, Pyun KH, Lee CE. Up-regulation of interleukin-4 and CD23/FcepsilonRII in minimal change nephrotic syndrome. Pediatr Nephrol 1999;13(3):199–204.

    Article  PubMed  CAS  Google Scholar 

  152. Kang J, Bai KM, Wang BL, Yao Z, Pang XW, Chen WF. Increased production of interleukin 4 in children with simple idiopathic nephrotic syndrome. Chin Med J 1994;107(5):347–350.

    PubMed  CAS  Google Scholar 

  153. Matsumoto K. Decreased release of IL-10 by monocytes from patients with lipoid nephrosis. Clin Exp Immunol 1995;102(3):603–607.

    Article  PubMed  CAS  Google Scholar 

  154. Matsumoto K, Kanmatsuse K. Increased IL-12 release by monocytes in nephrotic patients. Clin Exp Immunol 1999;117(2):361–367.

    Article  PubMed  CAS  Google Scholar 

  155. Matsumoto K, Kanmatsuse K. Augmented interleukin-18 production by peripheral blood monocytes in patients with minimal-change nephrotic syndrome. Am J Nephrol 2001;21(1):20–27.

    Article  PubMed  CAS  Google Scholar 

  156. Bakr A, Shokeir M, El-Chenawi F, El-Husseni F, Abdel-Rahman A, El-Ashry R. Tumor necrosis factor-alpha production from mononuclear cells in nephrotic syndrome. Pediatr Nephrol 2003;18(6):516–520.

    PubMed  Google Scholar 

  157. Laflam PF, Haraguchi S, Garin EH. Cytokine mRNA profile in lipoid nephrosis: evidence for increased IL-8 mRNA stability. Nephron 2002;91(4):620–626.

    Article  PubMed  CAS  Google Scholar 

  158. Zachwieja J, Bobkowski W, Dobrowolska-Zachwieja A, Lewandowska-Stachowiak M, Zaniew M, Maciejewski J. Intracellular cytokines of peripheral blood lymphocytes in nephrotic syndrome. Pediatr Nephrol 2002;17(9):733–740.

    Article  PubMed  CAS  Google Scholar 

  159. Cheung W, Wei CL, Seah CC, Jordan SC, Yap HK. Atopy, serum IgE, and interleukin-13 in steroid-responsive nephrotic syndrome. Pediatr Nephrol 2004;19(6):627–632.

    Article  PubMed  Google Scholar 

  160. Kaneko K, Tuchiya K, Fujinaga S, Kawamura R, Ohtomo Y, Shimizu T et al. Th1/Th2 balance in childhood idiopathic nephrotic syndrome. Clin Nephrol 2002;58(6):393–397.

    PubMed  CAS  Google Scholar 

  161. Sellier-Leclerc AL, Duval A, Riveron S, Macher MA, Deschenes G, Loirat C et al. A humanized mouse model of idiopathic nephrotic syndrome suggests a pathogenic role for immature cells. J Am Soc Nephrol 2007;18(10):2732–2739.

    Article  PubMed  Google Scholar 

  162. Sharma R, Sharma M, McCarthy ET, Ge XL, Savin VJ. Components of normal serum block the focal segmental glomerulosclerosis factor activity in vitro. Kidney Int 2000;58(5):1973–1979.

    Article  PubMed  CAS  Google Scholar 

  163. Carraro M, Zennaro C, Candiano G, Musante L, Bruschi M, Ghiggeri GM et al. Nephrotic urine prevents increased rat glomerular albumin permeability induced by serum from the same patient with idiopathic nephrotic syndrome. Nephrol Dial Transplant 2003;18(4):689–693.

    Article  PubMed  CAS  Google Scholar 

  164. Candiano G, Musante L, Carraro M, Faccini L, Campanacci L, Zennaro C et al. Apolipoproteins prevent glomerular albumin permeability induced in vitro by serum from patients with focal segmental glomerulosclerosis. J Am Soc Nephrol 2001;12(1):143–150.

    PubMed  CAS  Google Scholar 

  165. Ghiggeri GM, Bruschi M, Candiano G, Rastaldi MP, Scolari F, Passerini P et al. Depletion of clusterin in renal diseases causing nephrotic syndrome. Kidney Int 2002;62(6):2184–2194.

    Article  PubMed  CAS  Google Scholar 

  166. Doucet A, Favre G, Deschenes G. Molecular mechanism of edema formation in nephrotic syndrome: therapeutic implications. Pediatr Nephrol 2007;22(12):1983–1990.

    Article  PubMed  Google Scholar 

  167. Haws RM, Baum M. Efficacy of albumin and diuretic therapy in children with nephrotic syndrome. Pediatrics 1993;91(6):1142–1146.

    PubMed  CAS  Google Scholar 

  168. Rascher W, Tulassay T, Seyberth HW, Himbert U, Lang U, Scharer K. Diuretic and hormonal responses to head-out water immersion in nephrotic syndrome. J Pediatr 1986;109(4):609–614.

    Article  PubMed  CAS  Google Scholar 

  169. Taylor B, Andrews N, Stowe J, Hamidi-Manesh L, Miller E. No increased risk of relapse after meningococcal C conjugate vaccine in nephrotic syndrome. Arch Dis Child 2007;92(10):887–889.

    Article  PubMed  Google Scholar 

  170. Alpay H, Yildiz N, Onar A, Temizer H, Ozcay S. Varicella vaccination in children with steroid-sensitive nephrotic syndrome. Pediatr Nephrol 2002;17(3):181–183.

    Article  PubMed  Google Scholar 

  171. Furth SL, Arbus GS, Hogg R, Tarver J, Chan C, Fivush BA. Varicella vaccination in children with nephrotic syndrome: a report of the Southwest Pediatric Nephrology Study Group. J Pediatr 2003;142(2):145–148.

    Article  PubMed  Google Scholar 

  172. Elisaf M, Dardamanis M, Pappas M, Sferopoulos G, Siamopoulos KC. Treatment of nephrotic hyperlipidemia with lovastatin. Clin Nephrol 1991;36(1):50–52.

    PubMed  CAS  Google Scholar 

  173. Wheeler DC, Bernard DB. Lipid abnormalities in the nephrotic syndrome: causes, consequences, and treatment. Am J Kidney Dis 1994;23(3):331–346.

    PubMed  CAS  Google Scholar 

  174. Prescott WA, Jr., Streetman DA, Streetman DS. The potential role of HMG-CoA reductase inhibitors in pediatric nephrotic syndrome. Ann Pharmacother 2004;38(12):2105–2114.

    Article  PubMed  CAS  Google Scholar 

  175. Bak M, Serdaroglu E, Guclu R. Prophylactic calcium and vitamin D treatments in steroid-treated children with nephrotic syndrome. Pediatr Nephrol 2006;21(3):350–354.

    Article  PubMed  Google Scholar 

  176. Woroniecki RP, Orlova TN, Mendelev N, Shatat IF, Hailpern SM, Kaskel FJ et al. Urinary proteome of steroid-sensitive and steroid-resistant idiopathic nephrotic syndrome of childhood. Am J Nephrol 2006;26(3):258–267.

    Article  PubMed  CAS  Google Scholar 

  177. Trainin EB, Boichis H, Spitzer A, Edelmann CM, Jr., Greifer I. Late nonresponsiveness to steroids in children with the nephrotic syndrome. J Pediatr 1975;87(4):519–523.

    Article  PubMed  CAS  Google Scholar 

  178. Tune BM, Mendoza SA. Treatment of the idiopathic nephrotic syndrome: regimens and outcomes in children and adults. J Am Soc Nephrol 1997;8(5):824–832.

    PubMed  CAS  Google Scholar 

  179. Alternate-day versus intermittent prednisone in frequently relapsing nephrotic syndrome. A report of “Arbetsgemeinschaft fur Padiatrische Nephrologie”. Lancet 1979;1(8113):401–403.

    Google Scholar 

  180. Murnaghan K, Vasmant D, Bensman A. Pulse methylprednisolone therapy in severe idiopathic childhood nephrotic syndrome. Acta Paediatr Scand 1984;73(6):733–739.

    Article  PubMed  CAS  Google Scholar 

  181. Bagga A, Hari P, Srivastava RN. Prolonged versus standard prednisolone therapy for initial episode of nephrotic syndrome. Pediatr Nephrol 1999;13(9):824–827.

    Article  PubMed  CAS  Google Scholar 

  182. Ehrich JH, Brodehl J. Long versus standard prednisone therapy for initial treatment of idiopathic nephrotic syndrome in children. Arbeitsgemeinschaft fur Padiatrische Nephrologie. Eur J Pediatr 1993;152(4):357–361.

    Article  PubMed  CAS  Google Scholar 

  183. Ksiazek J, Wyszynska T. Short versus long initial prednisone treatment in steroid-sensitive nephrotic syndrome in children. Acta Paediatr 1995;84(8):889–893.

    Article  PubMed  CAS  Google Scholar 

  184. Norero C, Delucchi A, Lagos E, Rosati P. [Initial therapy of primary nephrotic syndrome in children: evaluation in a period of 18 months of two prednisone treatment schedules. Chilean Co-operative Group of Study of Nephrotic Syndrome in Children]. Rev Med Chil 1996;124(5):567–572.

    PubMed  CAS  Google Scholar 

  185. Ueda N, Chihara M, Kawaguchi S, Niinomi Y, Nonoda T, Matsumoto J et al. Intermittent versus long-term tapering prednisolone for initial therapy in children with idiopathic nephrotic syndrome. J Pediatr 1988;112(1):122–126.

    Article  PubMed  CAS  Google Scholar 

  186. Hodson EM, Knight JF, Willis NS, Craig JC. Corticosteroid therapy for nephrotic syndrome in children. Cochrane Database Syst Rev 2000;(4):CD001533.

    Google Scholar 

  187. Hodson EM, Willis NS, Craig JC. Corticosteroid therapy for nephrotic syndrome in children. Cochrane Database Syst Rev 2007;(4):CD001533.

    Google Scholar 

  188. Leisti S, Hallman N, Koskimies O, Perheentupa J, Rapola J, Vilska J. Association of postmedication hypocortisolism with early first relapse of idiopathic nephrotic syndrome. Lancet 1977;2(8042):795–796.

    Article  PubMed  CAS  Google Scholar 

  189. Leisti S, Koskimies O, Perheentupa J, Vilska J, Hallman N. Idiopathic nephrotic syndrome: prevention of early relapse. Br Med J 1978;1(6117):892.

    Article  PubMed  CAS  Google Scholar 

  190. Schoeneman MJ. Minimal change nephrotic syndrome: treatment with low doses of hydrocortisone. J Pediatr 1983;102(5):791–793.

    Article  PubMed  CAS  Google Scholar 

  191. Abeyagunawardena AS, Hindmarsh P, Trompeter RS. Adrenocortical suppression increases the risk of relapse in nephrotic syndrome. Arch Dis Child 2007;92(7):585–588.

    Article  PubMed  Google Scholar 

  192. Hoyer PF, Brodeh J. Initial treatment of idiopathic nephrotic syndrome in children: prednisone versus prednisone plus cyclosporine A: a prospective, randomized trial. J Am Soc Nephrol 2006;17(4):1151–1157.

    Article  PubMed  CAS  Google Scholar 

  193. Wingen AM, Muller-Wiefel DE, Scharer K. Spontaneous remissions in frequently relapsing and steroid dependent idiopathic nephrotic syndrome. Clin Nephrol 1985;23(1):35–40.

    PubMed  CAS  Google Scholar 

  194. Broyer M, Terzi F, Lehnert A, Gagnadoux MF, Guest G, Niaudet P. A controlled study of deflazacort in the treatment of idiopathic nephrotic syndrome. Pediatr Nephrol 1997;11(4):418–422.

    Article  PubMed  CAS  Google Scholar 

  195. MacDonald NE, Wolfish N, McLaine P, Phipps P, Rossier E. Role of respiratory viruses in exacerbations of primary nephrotic syndrome. J Pediatr 1986;108(3):378–382.

    Article  PubMed  CAS  Google Scholar 

  196. Abeyagunawardena AS, Trompeter RS. Increasing the dose of prednisolone during viral infections reduces the risk of relapse in nephrotic syndrome: a randomised controlled trial. Arch Dis Child 2008;93(3):226–228.

    Article  PubMed  CAS  Google Scholar 

  197. Mattoo TK, Mahmoud MA. Increased maintenance corticosteroids during upper respiratory infection decrease the risk of relapse in nephrotic syndrome. Nephron 2000;85(4):343–345.

    Article  PubMed  CAS  Google Scholar 

  198. Polito C, Oporto MR, Totino SF, La Manna A, Di Toro R. Normal growth of nephrotic children during long-term alternate-day prednisone therapy. Acta Paediatr Scand 1986;75(2):245–250.

    Article  PubMed  CAS  Google Scholar 

  199. Donatti TL, Koch VH, Fujimura MD, Okay Y. Growth in steroid-responsive nephrotic syndrome: a study of 85 pediatric patients. Pediatr Nephrol 2003;18(8):789–795.

    Article  PubMed  Google Scholar 

  200. Emma F, Sesto A, Rizzoni G. Long-term linear growth of children with severe steroid-responsive nephrotic syndrome. Pediatr Nephrol 2003;18(8):783–788.

    Article  PubMed  Google Scholar 

  201. Fakhouri F, Bocquet N, Taupin P, Presne C, Gagnadoux MF, Landais P et al. Steroid-sensitive nephrotic syndrome: from childhood to adulthood. Am J Kidney Dis 2003;41(3):550–557.

    Article  PubMed  Google Scholar 

  202. Leonard MB, Feldman HI, Shults J, Zemel BS, Foster BJ, Stallings VA. Long-term, high-dose glucocorticoids and bone mineral content in childhood glucocorticoid-sensitive nephrotic syndrome. N Engl J Med 2004;351(9):868–875.

    Article  PubMed  CAS  Google Scholar 

  203. Biyikli NK, Emre S, Sirin A, Bilge I. Biochemical bone markers in nephrotic children. Pediatr Nephrol 2004;19(8):869–873.

    Article  PubMed  Google Scholar 

  204. Kim SD, Cho BS. Pamidronate therapy for preventing steroid-induced osteoporosis in children with nephropathy. Nephron Clin Pract 2006;102(3–4):c81–c87.

    Article  PubMed  CAS  Google Scholar 

  205. Tanphaichitr P, Tanphaichitr D, Sureeratanan J, Chatasingh S. Treatment of nephrotic syndrome with levamisole. J Pediatr 1980;96(3 Pt 1):490–493.

    PubMed  CAS  Google Scholar 

  206. Drachman R, Schlesinger M, Alon U, Mor J, Etzioni A, Shapira H et al. Immunoregulation with levamisole in children with frequently relapsing steroid responsive nephrotic syndrome. Acta Paediatr Scand 1988;77(5):721–726.

    Article  PubMed  CAS  Google Scholar 

  207. Mehta KP, Ali U, Kutty M, Kolhatkar U. Immunoregulatory treatment for minimal change nephrotic syndrome. Arch Dis Child 1986;61(2):153–158.

    Article  PubMed  CAS  Google Scholar 

  208. Mongeau JG, Robitaille PO, Roy F. Clinical efficacy of levamisole in the treatment of primary nephrosis in children. Pediatr Nephrol 1988;2(4):398–401.

    Article  PubMed  CAS  Google Scholar 

  209. Niaudet P, Drachman R, Gagnadoux MF, Broyer M. Treatment of idiopathic nephrotic syndrome with levamisole. Acta Paediatr Scand 1984;73(5):637–641.

    Article  PubMed  CAS  Google Scholar 

  210. Boyer O, Moulder JK, Grandin L, Somers MJ. Short- and long-term efficacy of levamisole as adjunctive therapy in childhood nephrotic syndrome. Pediatr Nephrol 2008;23(4):575–580.

    Article  PubMed  Google Scholar 

  211. Levamisole for corticosteroid-dependent nephrotic syndrome in childhood. British Association for Paediatric Nephrology. Lancet 1991;337(8757):1555–1557.

    Google Scholar 

  212. Dayal U, Dayal AK, Shastry JC, Raghupathy P. Use of levamisole in maintaining remission in steroid-sensitive nephrotic syndrome in children. Nephron 1994;66(4):408–412.

    Article  PubMed  CAS  Google Scholar 

  213. Alsaran K, Grisaru S, Stephens D, Arbus G. Levamisole vs. cyclophosphamide for frequently-relapsing steroid-dependent nephrotic syndrome. Clin Nephrol 2001;56(4):289–294.

    PubMed  CAS  Google Scholar 

  214. Barbano G, Ginevri F, Ghiggeri GM, Gusmano R. Disseminated autoimmune disease during levamisole treatment of nephrotic syndrome. Pediatr Nephrol 1999;13(7):602–603.

    Article  PubMed  CAS  Google Scholar 

  215. Palcoux JB, Niaudet P, Goumy P. Side effects of levamisole in children with nephrosis. Pediatr Nephrol 1994;8(2):263–264.

    Article  PubMed  CAS  Google Scholar 

  216. Coldbeck JH. Experience with alkylating agents in the treatment of children with the nephrotic syndrome. Med J Aust 1963;2987–989.

    PubMed  CAS  Google Scholar 

  217. Barratt TM, Soothill JF. Controlled trial of cyclophosphamide in steroid-sensitive relapsing nephrotic syndrome of childhood. Lancet 1970;2(7671):479–482.

    Article  PubMed  CAS  Google Scholar 

  218. Prospective, controlled trial of cyclophosphamide therapy in children with nephrotic syndrome. Report of the International study of Kidney Disease in Children. Lancet 1974;2(7878):423–427.

    Google Scholar 

  219. Pennisi AJ, Grushkin CM, Lieberman E. Cyclophosphamide in the treatment of idiopathic nephrotic syndrome. Pediatrics 1976;57(6):948–951.

    PubMed  CAS  Google Scholar 

  220. Ueda N, Kuno K, Ito S. Eight and 12 week courses of cyclophosphamide in nephrotic syndrome. Arch Dis Child 1990;65(10):1147–1150.

    Article  PubMed  CAS  Google Scholar 

  221. Cyclophosphamide treatment of steroid dependent nephrotic syndrome: comparison of eight week with 12 week course. Report of Arbeitsgemeinschaft fur Padiatrische Nephrologie. Arch Dis Child 1987;62(11):1102–1106.

    Article  Google Scholar 

  222. Cameron JS, Chantler C, Ogg CS, White RH. Long-term stability of remission in nephrotic syndrome after treatment with cyclophosphamide. Br Med J 1974;4(5935):7–11.

    Article  PubMed  CAS  Google Scholar 

  223. Dundon S, O’Callaghan U, Raftery J. Stability of remission in minimal lesion nephrotic syndrome after treatment with prednisolone and cyclophosphamide. Int J Pediatr Nephrol 1980;1(1):22–25.

    PubMed  CAS  Google Scholar 

  224. Effect of cytotoxic drugs in frequently relapsing nephrotic syndrome with and without steroid dependence. N Engl J Med 1982;306(8):451–454.

    Article  Google Scholar 

  225. Siegel NJ, Gaudio KM, Krassner LS, McDonald BM, Anderson FP, Kashgarian M. Steroid-dependent nephrotic syndrome in children: histopathology and relapses after cyclophosphamide treatment. Kidney Int 1981;19(3):454–459.

    Article  PubMed  CAS  Google Scholar 

  226. Kemper MJ, Altrogge H, Ludwig K, Timmermann K, Muller-Wiefel DE. Unfavorable response to cyclophosphamide in steroid-dependent nephrotic syndrome. Pediatr Nephrol 2000;14(8–9):772–775.

    Article  PubMed  CAS  Google Scholar 

  227. Vester U, Kranz B, Zimmermann S, Buscher R, Hoyer PF. The response to cyclophosphamide in steroid-sensitive nephrotic syndrome is influenced by polymorphic expression of glutathion-S-transferases-M1 and -P1. Pediatr Nephrol 2005;20(4):478–481.

    Article  PubMed  Google Scholar 

  228. Vester U, Kranz B, Zimmermann S, Hoyer PF. Cyclophosphamide in steroid-sensitive nephrotic syndrome: outcome and outlook. Pediatr Nephrol 2003;18(7):661–664.

    PubMed  Google Scholar 

  229. Kyrieleis HA, Levtchenko EN, Wetzels JF. Long-term outcome after cyclophosphamide treatment in children with steroid-dependent and frequently relapsing minimal change nephrotic syndrome. Am J Kidney Dis 2007;49(5):592–597.

    Article  PubMed  CAS  Google Scholar 

  230. Gulati S, Pokhariyal S, Sharma RK, Elhence R, Kher V, Pandey CM et al. Pulse cyclophosphamide therapy in frequently relapsing nephrotic syndrome. Nephrol Dial Transplant 2001;16(10):2013–2017.

    Article  PubMed  CAS  Google Scholar 

  231. Donia AF, Gazareen SH, Ahmed HA, Moustafa FE, Shoeib AA, Ismail AM et al. Pulse cyclophosphamide inadequately suppresses reoccurrence of minimal change nephrotic syndrome in corticoid-dependent children. Nephrol Dial Transplant 2003;18(10):2054–2058.

    Article  PubMed  CAS  Google Scholar 

  232. Prasad N, Gulati S, Sharma RK, Singh U, Ahmed M. Pulse cyclophosphamide therapy in steroid-dependent nephrotic syndrome. Pediatr Nephrol 2004;19(5):494–498.

    Article  PubMed  Google Scholar 

  233. Grupe WE. Chlorambucil in steroid-dependent nephrotic syndrome. J Pediatr 1973;82(4):598–606.

    Article  PubMed  CAS  Google Scholar 

  234. Alatas H, Wirya IG, Tambunan T, Himawan S. Controlled trial of chlorambucil in frequently relapsing nephrotic syndrome in children (a preliminary report). J Med Assoc Thai 1978;61(Suppl 1):222–228.

    PubMed  Google Scholar 

  235. Baluarte HJ, Hiner L, Gruskin AB. Chlorambucil dosage in frequently relapsing nephrotic syndrome: a controlled clinical trial. J Pediatr 1978;92(2):295–298.

    Article  PubMed  CAS  Google Scholar 

  236. Williams SA, Makker SP, Ingelfinger JR, Grupe WE. Long-term evaluation of chlorambucil plus prednisone in the idiopathic nephrotic syndrome of childhood. N Engl J Med 1980;302(17):929–933.

    Article  PubMed  CAS  Google Scholar 

  237. Latta K, von Schnakenburg C, Ehrich JH. A meta-analysis of cytotoxic treatment for frequently relapsing nephrotic syndrome in children. Pediatr Nephrol 2001;16(3):271–282.

    Article  PubMed  CAS  Google Scholar 

  238. Hsu AC, Folami AO, Bain J, Rance CP. Gonadal function in males treated with cyclophosphamide for nephrotic syndrome. Fertil Steril 1979;31(2):173–177.

    PubMed  CAS  Google Scholar 

  239. Penso J, Lippe B, Ehrlich R, Smith FG, Jr. Testicular function in prepubertal and pubertal male patients treated with cyclophosphamide for nephrotic syndrome. J Pediatr 1974;84(6):831–836.

    Article  PubMed  CAS  Google Scholar 

  240. Trompeter RS, Evans PR, Barratt TM. Gonadal function in boys with steroid-responsive nephrotic syndrome treated with cyclophosphamide for short periods. Lancet 1981;1(8231):1177–1179.

    Article  PubMed  CAS  Google Scholar 

  241. Buchanan JD, Fairley KF, Barrie JU. Return of spermatogenesis after stopping cyclophosphamide therapy. Lancet 1975;2(7926):156–157.

    Article  PubMed  CAS  Google Scholar 

  242. Watson AR, Taylor J, Rance CP, Bain J. Gonadal function in women treated with cyclophosphamide for childhood nephrotic syndrome: a long-term follow-up study. Fertil Steril 1986;46(2):331–333.

    PubMed  CAS  Google Scholar 

  243. Niaudet P, Habib R. Cyclosporine in the treatment of idiopathic nephrosis. J Am Soc Nephrol 1994;5(4):1049–1056.

    PubMed  CAS  Google Scholar 

  244. Hulton SA, Neuhaus TJ, Dillon MJ, Barratt TM. Long-term cyclosporin A treatment of minimal-change nephrotic syndrome of childhood. Pediatr Nephrol 1994;8(4):401–403.

    Article  PubMed  CAS  Google Scholar 

  245. Niaudet P. Comparison of cyclosporin and chlorambucil in the treatment of steroid-dependent idiopathic nephrotic syndrome: a multicentre randomized controlled trial. The French Society of Paediatric Nephrology. Pediatr Nephrol 1992;6(1):1–3.

    Article  PubMed  CAS  Google Scholar 

  246. Ponticelli C, Edefonti A, Ghio L, Rizzoni G, Rinaldi S, Gusmano R et al. Cyclosporin versus cyclophosphamide for patients with steroid-dependent and frequently relapsing idiopathic nephrotic syndrome: a multicentre randomized controlled trial. Nephrol Dial Transplant 1993;8(12):1326–1332.

    PubMed  CAS  Google Scholar 

  247. Tejani A, Suthanthiran M, Pomrantz A. A randomized controlled trial of low-dose prednisone and ciclosporin versus high-dose prednisone in nephrotic syndrome of children. Nephron 1991;59(1):96–99.

    Article  PubMed  CAS  Google Scholar 

  248. Hirano T, Kawamura T, Fukuda S, Kohsaka S, Yoshikawa N, Yoshida M et al. Implication of cholesterol in cyclosporine pharmacodynamics in minimal change nephrotic syndrome. Clin Pharmacol Ther 2003;74(6):581–590.

    Article  PubMed  CAS  Google Scholar 

  249. Ingulli E, Tejani A. Severe hypercholesterolemia inhibits cyclosporin A efficacy in a dose-dependent manner in children with nephrotic syndrome. J Am Soc Nephrol 1992;3(2):254–259.

    PubMed  CAS  Google Scholar 

  250. Ishikura K, Ikeda M, Hattori S, Yoshikawa N, Sasaki S, Iijima K et al. Effective and safe treatment with cyclosporine in nephrotic children: a prospective, randomized multicenter trial. Kidney Int 2008;73(10):1167–1173.

    Article  PubMed  CAS  Google Scholar 

  251. Habib R, Niaudet P. Comparison between pre- and posttreatment renal biopsies in children receiving ciclosporine for idiopathic nephrosis. Clin Nephrol 1994;42(3):141–146.

    PubMed  CAS  Google Scholar 

  252. Meyrier A, Noel LH, Auriche P, Callard P. Long-term renal tolerance of cyclosporin A treatment in adult idiopathic nephrotic syndrome. Collaborative Group of the Societe de Nephrologie. Kidney Int 1994;45(5):1446–1456.

    Article  PubMed  CAS  Google Scholar 

  253. Iijima K, Hamahira K, Tanaka R, Kobayashi A, Nozu K, Nakamura H et al. Risk factors for cyclosporine-induced tubulointerstitial lesions in children with minimal change nephrotic syndrome. Kidney Int 2002;61(5):1801–1805.

    Article  PubMed  Google Scholar 

  254. Freundlich M. Bone mineral content and mineral metabolism during cyclosporine treatment of nephrotic Syndrome. J Pediatr 2006;149(3):383–389.

    Article  PubMed  CAS  Google Scholar 

  255. Sinha MD, MacLeod R, Rigby E, Clark AG. Treatment of severe steroid-dependent nephrotic syndrome (SDNS) in children with tacrolimus. Nephrol Dial Transplant 2006;21(7):1848–1854.

    Article  PubMed  CAS  Google Scholar 

  256. Dittrich K, Knerr I, Rascher W, Dotsch J. Transient insulin-dependent diabetes mellitus in children with steroid-dependent idiopathic nephrotic syndrome during tacrolimus treatment. Pediatr Nephrol 2006;21(7):958–961.

    Article  PubMed  Google Scholar 

  257. Abramowicz M, Barnett HL, Edelmann CM, Jr., Greifer I, Kobayashi O, Arneil GC et al. Controlled trial of azathioprine in children with nephrotic syndrome. A report for the international study of kidney disease in children. Lancet 1970;1(7654):959–961.

    Article  PubMed  CAS  Google Scholar 

  258. Barratt TM, Cameron JS, Chantler C, Counahan R, Ogg CS, Soothill JF. Controlled trial of azathioprine in treatment of steroid-responsive nephrotic syndrome of childhood. Arch Dis Child 1977;52(6):462–463.

    Article  PubMed  CAS  Google Scholar 

  259. Durkan AM, Hodson EM, Willis NS, Craig JC. Immunosuppressive agents in childhood nephrotic syndrome: a meta-analysis of randomized controlled trials. Kidney Int 2001;59(5):1919–1927.

    Article  PubMed  CAS  Google Scholar 

  260. Afzal K, Bagga A, Menon S, Hari P, Jordan SC. Treatment with mycophenolate mofetil and prednisolone for steroid-dependent nephrotic syndrome. Pediatr Nephrol 2007;22(12):2059–2065.

    Article  PubMed  Google Scholar 

  261. Al-Akash S, Al-Makdama A. Mycophenolate mofetil in children with steroid-dependent and/or frequently relapsing nephrotic syndrome. Ann Saudi Med 2005;25(5):380–384.

    PubMed  Google Scholar 

  262. Bagga A, Hari P, Moudgil A, Jordan SC. Mycophenolate mofetil and prednisolone therapy in children with steroid-dependent nephrotic syndrome. Am J Kidney Dis 2003;42(6):1114–1120.

    Article  PubMed  CAS  Google Scholar 

  263. Barletta GM, Smoyer WE, Bunchman TE, Flynn JT, Kershaw DB. Use of mycophenolate mofetil in steroid-dependent and -resistant nephrotic syndrome. Pediatr Nephrol 2003;18(8):833–837.

    Article  PubMed  Google Scholar 

  264. Chandra M, Susin M, Abitbol C. Remission of relapsing childhood nephrotic syndrome with mycophenolate mofetil. Pediatr Nephrol 2000;14(3):224–226.

    Article  PubMed  CAS  Google Scholar 

  265. Fujinaga S, Ohtomo Y, Umino D, Takemoto M, Shimizu T, Yamashiro Y et al. A prospective study on the use of mycophenolate mofetil in children with cyclosporine-dependent nephrotic syndrome. Pediatr Nephrol 2007;22(1):71–76.

    Article  PubMed  Google Scholar 

  266. Gellermann J, Querfeld U. Frequently relapsing nephrotic syndrome: treatment with mycophenolate mofetil. Pediatr Nephrol 2004;19(1):101–104.

    Article  PubMed  Google Scholar 

  267. Hogg RJ, Fitzgibbons L, Bruick J, Bunke M, Ault B, Baqi N et al. Mycophenolate mofetil in children with frequently relapsing nephrotic syndrome: a report from the Southwest Pediatric Nephrology Study Group. Clin J Am Soc Nephrol 2006;1(6):1173–1178.

    Article  PubMed  CAS  Google Scholar 

  268. Mendizabal S, Zamora I, Berbel O, Sanahuja MJ, Fuentes J, Simon J. Mycophenolate mofetil in steroid/cyclosporine-dependent/resistant nephrotic syndrome. Pediatr Nephrol 2005;20(7):914–919.

    Article  PubMed  CAS  Google Scholar 

  269. Moudgil A, Bagga A, Jordan SC. Mycophenolate mofetil therapy in frequently relapsing steroid-dependent and steroid-resistant nephrotic syndrome of childhood: current status and future directions. Pediatr Nephrol 2005;20(10):1376–1381.

    Article  PubMed  Google Scholar 

  270. Novak I, Frank R, Vento S, Vergara M, Gauthier B, Trachtman H. Efficacy of mycophenolate mofetil in pediatric patients with steroid-dependent nephrotic syndrome. Pediatr Nephrol 2005;20(9):1265–1268.

    Article  PubMed  Google Scholar 

  271. Okada M, Sugimoto K, Yagi K, Yanagida H, Tabata N, Takemura T. Mycophenolate mofetil therapy for children with intractable nephrotic syndrome. Pediatr Int 2007;49(6):933–937.

    Article  PubMed  CAS  Google Scholar 

  272. Ulinski T, Dubourg L, Said MH, Parchoux B, Ranchin B, Cochat P. Switch from cyclosporine A to mycophenolate mofetil in nephrotic children. Pediatr Nephrol 2005;20(4):482–485.

    Article  PubMed  Google Scholar 

  273. Benz K, Dotsch J, Rascher W, Stachel D. Change of the course of steroid-dependent nephrotic syndrome after rituximab therapy. Pediatr Nephrol 2004;19(7):794–797.

    Article  PubMed  Google Scholar 

  274. Francois H, Daugas E, Bensman A, Ronco P. Unexpected efficacy of rituximab in multirelapsing minimal change nephrotic syndrome in the adult: first case report and pathophysiological considerations. Am J Kidney Dis 2007;49(1):158–161.

    Article  PubMed  CAS  Google Scholar 

  275. Gilbert RD, Hulse E, Rigden S. Rituximab therapy for steroid-dependent minimal change nephrotic syndrome. Pediatr Nephrol 2006;21(11):1698–1700.

    Article  PubMed  Google Scholar 

  276. Hofstra JM, Deegens JK, Wetzels JF. Rituximab: effective treatment for severe steroid-dependent minimal change nephrotic syndrome? Nephrol Dial Transplant 2007;22(7):2100–2102.

    Article  PubMed  Google Scholar 

  277. Smith GC. Is there a role for rituximab in the treatment of idiopathic childhood nephrotic syndrome? Pediatr Nephrol 2007;22(6):893–898.

    Article  PubMed  Google Scholar 

  278. Guigonis V, Dallocchio A, Baudouin V, Dehennault M, Hachon-Le Camus C, Afanetti M et al. Rituximab treatment for severe steroid- or cyclosporine-dependent nephrotic syndrome: a multicentric series of 22 cases. Pediatr Nephrol 2008;23(8):1269–1279.

    Article  PubMed  Google Scholar 

  279. Scharer K, Minges U. Long term prognosis of the nephrotic syndrome in childhood. Clin Nephrol 1973;1(3):182–187.

    PubMed  CAS  Google Scholar 

  280. Trompeter RS, Lloyd BW, Hicks J, White RH, Cameron JS. Long-term outcome for children with minimal-change nephrotic syndrome. Lancet 1985;1(8425):368–370.

    Article  PubMed  CAS  Google Scholar 

  281. Wynn SR, Stickler GB, Burke EC. Long-term prognosis for children with nephrotic syndrome. Clin Pediatr (Phila) 1988;27(2):63–68.

    Article  CAS  Google Scholar 

  282. Lewis MA, Baildom EM, Davis N, Houston IB, Postlethwaite RJ. Nephrotic syndrome: from toddlers to twenties. Lancet 1989;1(8632):255–259.

    Article  PubMed  CAS  Google Scholar 

  283. Koskimies O, Vilska J, Rapola J, Hallman N. Long-term outcome of primary nephrotic syndrome. Arch Dis Child 1982;57(7):544–548.

    Article  PubMed  CAS  Google Scholar 

  284. Lahdenkari AT, Suvanto M, Kajantie E, Koskimies O, Kestila M, Jalanko H. Clinical features and outcome of childhood minimal change nephrotic syndrome: is genetics involved? Pediatr Nephrol 2005;20(8):1073–1080.

    Article  PubMed  Google Scholar 

  285. Ruth EM, Kemper MJ, Leumann EP, Laube GF, Neuhaus TJ. Children with steroid-sensitive nephrotic syndrome come of age: long-term outcome. J Pediatr 2005;147(2):202–207.

    Article  PubMed  Google Scholar 

  286. Arbus GS, Poucell S, Bacheyie GS, Baumal R. Focal segmental glomerulosclerosis with idiopathic nephrotic syndrome: three types of clinical response. J Pediatr 1982;101(1):40–45.

    Article  PubMed  CAS  Google Scholar 

  287. Niaudet P. Treatment of childhood steroid-resistant idiopathic nephrosis with a combination of cyclosporine and prednisone. French Society of Pediatric Nephrology. J Pediatr 1994;125(6 Pt 1):981–986.

    PubMed  CAS  Google Scholar 

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

    Article  PubMed  CAS  Google Scholar 

  289. Tune BM, Kirpekar R, Sibley RK, Reznik VM, Griswold WR, Mendoza SA. Intravenous methylprednisolone and oral alkylating agent therapy of prednisone-resistant pediatric focal segmental glomerulosclerosis: a long-term follow-up. Clin Nephrol 1995;43(2):84–88.

    PubMed  CAS  Google Scholar 

  290. Yorgin PD, Krasher J, Al-Uzri AY. Pulse methylprednisolone treatment of idiopathic steroid-resistant nephrotic syndrome. Pediatr Nephrol 2001;16(3):245–250.

    Article  PubMed  CAS  Google Scholar 

  291. Pena A, Bravo J, Melgosa M, Fernandez C, Meseguer C, Espinosa L et al. Steroid-resistant nephrotic syndrome: long-term evolution after sequential therapy. Pediatr Nephrol 2007;22(11):1875–1880.

    Article  PubMed  Google Scholar 

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

    Article  PubMed  CAS  Google Scholar 

  293. Hari P, Bagga A, Jindal N, Srivastava RN. Treatment of focal glomerulosclerosis with pulse steroids and oral cyclophosphamide. Pediatr Nephrol 2001;16(11):901–905.

    Article  PubMed  CAS  Google Scholar 

  294. Tarshish P, Tobin JN, Bernstein J, Edelmann CM, Jr. Cyclophosphamide does not benefit patients with focal segmental glomerulosclerosis. A report of the International Study of Kidney Disease in Children. Pediatr Nephrol 1996;10(5):590–593.

    Article  PubMed  CAS  Google Scholar 

  295. Geary DF, Farine M, Thorner P, Baumal R. Response to cyclophosphamide in steroid-resistant focal segmental glomerulosclerosis: a reappraisal. Clin Nephrol 1984;22(3):109–113.

    PubMed  CAS  Google Scholar 

  296. Bergstrand A, Bollgren I, Samuelsson A, Tornroth T, Wasserman J, Winberg J. Idiopathic nephrotic syndrome of childhood: cyclophosphamide induced conversion from steroid refractory to highly steroid sensitive disease. Clin Nephrol 1973;1(5):302–306.

    PubMed  CAS  Google Scholar 

  297. White RH, Glasgow EF. Focal glomerulosclerosis – a progressive lesion associated with steroid-resistant nephrotic syndrome. Arch Dis Child 1971;46(250):877–878.

    Article  PubMed  CAS  Google Scholar 

  298. Tejani A, Nicastri AD, Sen D, Chen CK, Phadke K, Adamson O et al. Long-term evaluation of children with nephrotic syndrome and focal segmental glomerular sclerosis. Nephron 1983;35(4):225–231.

    Article  PubMed  CAS  Google Scholar 

  299. Elhence R, Gulati S, Kher V, Gupta A, Sharma RK. Intravenous pulse cyclophosphamide – a new regime for steroid-resistant minimal change nephrotic syndrome. Pediatr Nephrol 1994;8(1):1–3.

    Article  PubMed  CAS  Google Scholar 

  300. Alshaya HO, Al-Maghrabi JA, Kari JA. Intravenous pulse cyclophosphamide – is it effective in children with steroid-resistant nephrotic syndrome? Pediatr Nephrol 2003;18(11):1143–1146.

    Article  PubMed  Google Scholar 

  301. Rennert WP, Kala UK, Jacobs D, Goetsch S, Verhaart S. Pulse cyclophosphamide for steroid-resistant focal segmental glomerulosclerosis. Pediatr Nephrol 1999;13(2):113–116.

    Article  PubMed  CAS  Google Scholar 

  302. Bajpai A, Bagga A, Hari P, Dinda A, Srivastava RN. Intravenous cyclophosphamide in steroid-resistant nephrotic syndrome. Pediatr Nephrol 2003;18(4):351–356.

    PubMed  Google Scholar 

  303. Cade R, Mars D, Privette M, Thompson R, Croker B, Peterson J et al. Effect of long-term azathioprine administration in adults with minimal-change glomerulonephritis and nephrotic syndrome resistant to corticosteroids. Arch Intern Med 1986;146(4):737–741.

    Article  PubMed  CAS  Google Scholar 

  304. Brandis M, Burghard R, Leititis J, Zimmerhackl B, Hildebrandt F, Helmchen U. Cyclosporine A for treatment of nephrotic syndromes. Transplant Proc 1988;20(3 Suppl 4):275–279.

    PubMed  CAS  Google Scholar 

  305. Brodehl J, Hoyer PF, Oemar BS, Helmchen U, Wonigeit K. Cyclosporine treatment of nephrotic syndrome in children. Transplant Proc 1988;20(3 Suppl 4):269–274.

    PubMed  CAS  Google Scholar 

  306. Capodicasa G, De Santo NG, Nuzzi F, Giordano C. Cyclosporin A in nephrotic syndrome of childhood – a 14 month experience. Int J Pediatr Nephrol 1986;7(2):69–72.

    PubMed  CAS  Google Scholar 

  307. Garin EH, Orak JK, Hiott KL, Sutherland SE. Cyclosporine therapy for steroid-resistant nephrotic syndrome. A controlled study. Am J Dis Child 1988;142(9):985–988.

    PubMed  CAS  Google Scholar 

  308. Niaudet P, Habib R, Tete MJ, Hinglais N, Broyer M. Cyclosporin in the treatment of idiopathic nephrotic syndrome in children. Pediatr Nephrol 1987;1(4):566–573.

    Article  PubMed  CAS  Google Scholar 

  309. Waldo FB, Kohaut EC. Therapy of focal segmental glomerulosclerosis with cyclosporine A. Pediatr Nephrol 1987;1(2):180–182.

    Article  PubMed  CAS  Google Scholar 

  310. Safety and tolerability of cyclosporin A (Sandimmun) in idiopathic nephrotic syndrome. Collaborative Study Group of Sandimmun in Nephrotic Syndrome. Clin Nephrol 1991;35(Suppl 1):S48–S60.

    Google Scholar 

  311. Ingulli E, Singh A, Baqi N, Ahmad H, Moazami S, Tejani A. Aggressive, long-term cyclosporine therapy for steroid-resistant focal segmental glomerulosclerosis. J Am Soc Nephrol 1995;5(10):1820–1825.

    PubMed  CAS  Google Scholar 

  312. Cattran DC, Appel GB, Hebert LA, Hunsicker LG, Pohl MA, Hoy WE et al. A randomized trial of cyclosporine in patients with steroid-resistant focal segmental glomerulosclerosis. North America Nephrotic Syndrome Study Group. Kidney Int 1999;56(6):2220–2226.

    Article  PubMed  CAS  Google Scholar 

  313. Ponticelli C, Rizzoni G, Edefonti A, Altieri P, Rivolta E, Rinaldi S et al. A randomized trial of cyclosporine in steroid-resistant idiopathic nephrotic syndrome. Kidney Int 1993;43(6):1377–1384.

    Article  PubMed  CAS  Google Scholar 

  314. Lieberman KV, Tejani A. A randomized double-blind placebo-controlled trial of cyclosporine in steroid-resistant idiopathic focal segmental glomerulosclerosis in children. J Am Soc Nephrol 1996;7(1):56–63.

    PubMed  CAS  Google Scholar 

  315. Gregory MJ, Smoyer WE, Sedman A, Kershaw DB, Valentini RP, Johnson K et al. Long-term cyclosporine therapy for pediatric nephrotic syndrome: a clinical and histologic analysis. J Am Soc Nephrol 1996;7(4):543–549.

    PubMed  CAS  Google Scholar 

  316. Singh A, Tejani C, Tejani A. One-center experience with cyclosporine in refractory nephrotic syndrome in children. Pediatr Nephrol 1999;13(1):26–32.

    Article  PubMed  CAS  Google Scholar 

  317. Ehrich JH, Geerlings C, Zivicnjak M, Franke D, Geerlings H, Gellermann J. Steroid-resistant idiopathic childhood nephrosis: overdiagnosed and undertreated. Nephrol Dial Transplant 2007;22(8):2183–2193.

    Article  PubMed  Google Scholar 

  318. Loeffler K, Gowrishankar M, Yiu V. Tacrolimus therapy in pediatric patients with treatment-resistant nephrotic syndrome. Pediatr Nephrol 2004;19(3):281–287.

    Article  PubMed  Google Scholar 

  319. Bhimma R, Adhikari M, Asharam K, Connolly C. Management of steroid-resistant focal segmental glomerulosclerosis in children using tacrolimus. Am J Nephrol 2006;26(6):544–551.

    Article  PubMed  CAS  Google Scholar 

  320. Gulati S, Prasad N, Sharma RK, Kumar A, Gupta A, Baburaj VP. Tacrolimus: a new therapy for steroid-resistant nephrotic syndrome in children. Nephrol Dial Transplant 2008;23(3):910–913.

    Article  PubMed  CAS  Google Scholar 

  321. Montane B, Abitbol C, Chandar J, Strauss J, Zilleruelo G. Novel therapy of focal glomerulosclerosis with mycophenolate and angiotensin blockade. Pediatr Nephrol 2003;18(8):772–777.

    Article  PubMed  Google Scholar 

  322. Cattran DC, Wang MM, Appel G, Matalon A, Briggs W. Mycophenolate mofetil in the treatment of focal segmental glomerulosclerosis. Clin Nephrol 2004;62(6):405–411.

    PubMed  CAS  Google Scholar 

  323. Tumlin JA, Miller D, Near M, Selvaraj S, Hennigar R, Guasch A. A prospective, open-label trial of sirolimus in the treatment of focal segmental glomerulosclerosis. Clin J Am Soc Nephrol 2006;1(1):109–116.

    Article  PubMed  CAS  Google Scholar 

  324. Nakayama M, Kamei K, Nozu K, Matsuoka K, Nakagawa A, Sako M et al. Rituximab for refractory focal segmental glomerulosclerosis. Pediatr Nephrol 2008;23(3):481–485.

    Article  PubMed  Google Scholar 

  325. Bagga A, Sinha A, Moudgil A. Rituximab in patients with the steroid-resistant nephrotic syndrome. N Engl J Med 2007;356(26):2751–2752.

    Article  PubMed  CAS  Google Scholar 

  326. Donker AJ, Brentjens JR, van der Hem GK, Arisz L. Treatment of the nephrotic syndrome with indomethacin. Nephron 1978;22(4–6):374–381.

    Article  PubMed  CAS  Google Scholar 

  327. Velosa JA, Torres VE. Benefits and risks of nonsteroidal antiinflammatory drugs in steroid-resistant nephrotic syndrome. Am J Kidney Dis 1986;8(5):345–350.

    PubMed  CAS  Google Scholar 

  328. Bennett WM. The adverse renal effects of nonsteroidal anti-inflammatory drugs: increasing problems or overrated risk. Am J Kidney Dis 1983;2(4):477.

    PubMed  CAS  Google Scholar 

  329. Clive DM, Stoff JS. Renal syndromes associated with nonsteroidal antiinflammatory drugs. N Engl J Med 1984;310(9):563–572.

    Article  PubMed  CAS  Google Scholar 

  330. Kleinknecht C, Broyer M, Gubler MC, Palcoux JB. Irreversible renal failure after indomethacin in steroid-resistant nephrosis. N Engl J Med 1980;302(12):691.

    PubMed  CAS  Google Scholar 

  331. Martinez Vea A, Garcia Ruiz C, Carrera M, Oliver JA, Richart C. Effect of captopril in nephrotic-range proteinuria due to renovascular hypertension. Nephron 1987;45(2):162–163.

    Article  PubMed  CAS  Google Scholar 

  332. Ferder LF, Inserra F, Daccordi H, Smith RD. Enalapril improved renal function and proteinuria in chronic glomerulopathies. Nephron 1990;55(Suppl 1):90–95.

    Article  PubMed  Google Scholar 

  333. Milliner DS, Morgenstern BZ. Angiotensin converting enzyme inhibitors for reduction of proteinuria in children with steroid-resistant nephrotic syndrome. Pediatr Nephrol 1991;5(5):587–590.

    Article  PubMed  CAS  Google Scholar 

  334. Crosson JT. Focal segmental glomerulosclerosis and renal transplantation. Transplant Proc 2007;39(3):737–743.

    Article  PubMed  CAS  Google Scholar 

  335. Senggutuvan P, Cameron JS, Hartley RB, Rigden S, Chantler C, Haycock G et al. Recurrence of focal segmental glomerulosclerosis in transplanted kidneys: analysis of incidence and risk factors in 59 allografts. Pediatr Nephrol 1990;4(1):21–28.

    Article  PubMed  CAS  Google Scholar 

  336. Habib R, Hebert D, Gagnadoux MF, Broyer M. Transplantation in idiopathic nephrosis. Transplant Proc 1982;14(3):489–495.

    PubMed  CAS  Google Scholar 

  337. Rizzoni G, Ehrich JH, Brunner FP, Geerlings W, Fassbinder W, Landais P et al. Combined report on regular dialysis and transplantation of children in Europe, 1990. Nephrol Dial Transplant 1991;6(Suppl 4):31–42.

    PubMed  Google Scholar 

  338. Leumann EP, Briner J, Donckerwolcke RA, Kuijten R, Largiader F. Recurrence of focal segmental glomerulosclerosis in the transplanted kidney. Nephron 1980;25(2):65–71.

    Article  PubMed  CAS  Google Scholar 

  339. Fine RN. Recurrence of nephrotic syndrome/focal segmental glomerulosclerosis following renal transplantation in children. Pediatr Nephrol 2007;22(4):496–502.

    Article  PubMed  Google Scholar 

  340. Maizel SE, Sibley RK, Horstman JP, Kjellstrand CM, Simmons RL. Incidence and significance of recurrent focal segmental glomerulosclerosis in renal allograft recipients. Transplantation 1981;32(6):512–516.

    Article  PubMed  CAS  Google Scholar 

  341. Pinto J, Lacerda G, Cameron JS, Turner DR, Bewick M, Ogg CS. Recurrence of focal segmental glomerulosclerosis in renal allografts. Transplantation 1981;32(2):83–89.

    Article  PubMed  CAS  Google Scholar 

  342. Cameron JS. Recurrent primary disease and de novo nephritis following renal transplantation. Pediatr Nephrol 1991;5(4):412–421.

    Article  PubMed  CAS  Google Scholar 

  343. Ramos EL, Tisher CC. Recurrent diseases in the kidney transplant. Am J Kidney Dis 1994;24(1):142–154.

    PubMed  CAS  Google Scholar 

  344. Striegel JE, Sibley RK, Fryd DS, Mauer SM. Recurrence of focal segmental sclerosis in children following renal transplantation. Kidney Int Suppl 1986;19:S44–S50.

    PubMed  CAS  Google Scholar 

  345. Weber S, Tonshoff B. Recurrence of focal-segmental glomerulosclerosis in children after renal transplantation: clinical and genetic aspects. Transplantation 2005;80(1 Suppl):S128–S134.

    Article  PubMed  Google Scholar 

  346. Kim EM, Striegel J, Kim Y, Matas AJ, Najarian JS, Mauer SM. Recurrence of steroid-resistant nephrotic syndrome in kidney transplants is associated with increased acute renal failure and acute rejection. Kidney Int 1994;45(5):1440–1445.

    Article  PubMed  CAS  Google Scholar 

  347. Korbet SM, Schwartz MM, Lewis EJ. Recurrent nephrotic syndrome in renal allografts. Am J Kidney Dis 1988;11(3):270–276.

    PubMed  CAS  Google Scholar 

  348. Morales JM, Andres A, Prieto C, Martinez MA, Praga M, Ruilope LM et al. Clinical and histological sequence of recurrent focal segmental glomerulosclerosis. Nephron 1988;48(3):241–242.

    Article  PubMed  CAS  Google Scholar 

  349. Verani RR, Hawkins EP. Recurrent focal segmental glomerulosclerosis. A pathological study of the early lesion. Am J Nephrol 1986;6(4):263–270.

    Article  PubMed  CAS  Google Scholar 

  350. Dantal J, Baatard R, Hourmant M, Cantarovich D, Buzelin F, Soulillou JP. Recurrent nephrotic syndrome following renal transplantation in patients with focal glomerulosclerosis. A one-center study of plasma exchange effects. Transplantation 1991;52(5):827–831.

    Article  PubMed  CAS  Google Scholar 

  351. Artero M, Biava C, Amend W, Tomlanovich S, Vincenti F. Recurrent focal glomerulosclerosis: natural history and response to therapy. Am J Med 1992;92(4):375–383.

    Article  PubMed  CAS  Google Scholar 

  352. Banfi G, Colturi C, Montagnino G, Ponticelli C. The recurrence of focal segmental glomerulosclerosis in kidney transplant patients treated with cyclosporine. Transplantation 1990;50(4):594–596.

    Article  PubMed  CAS  Google Scholar 

  353. Pirson Y, Squifflet JP, Marbaix E, Alexandre GP, van Ypersele de Strihou C. Recurrence of focal glomerulosclerosis despite cyclosporin treatment after renal transplantation. Br Med J (Clin Res Ed) 1986;292(6531):1336.

    Article  CAS  Google Scholar 

  354. Schwarz A, Krause PH, Offermann G, Keller F. Recurrent and de novo renal disease after kidney transplantation with or without cyclosporine A. Am J Kidney Dis 1991;17(5):524–531.

    PubMed  CAS  Google Scholar 

  355. Vincenti F, Biava C, Tomlanovitch S, Amend WJ Jr., Garovoy M, Melzer J et al. Inability of cyclosporine to completely prevent the recurrence of focal glomerulosclerosis after kidney transplantation. Transplantation 1989;47(4):595–598.

    Article  PubMed  CAS  Google Scholar 

  356. Voets AJ, Hoitsma AJ, Koene RA. Recurrence of nephrotic syndrome during cyclosporin treatment after renal transplantation. Lancet 1986;1(8475):266–267.

    Article  PubMed  CAS  Google Scholar 

  357. Mowry J, Marik J, Cohen A, Hogg R, Sahney S, Ettenger R. Treatment of recurrent focal segmental glomerulosclerosis with high-dose cyclosporine A and plasmapheresis. Transplant Proc 1993;25(1 Pt 2):1345–1346.

    PubMed  CAS  Google Scholar 

  358. Ingulli E, Tejani A, Butt KM, Rajpoot D, Gonzalez R, Pomrantz A et al. High-dose cyclosporine therapy in recurrent nephrotic syndrome following renal transplantation. Transplantation 1990;49(1):219–221.

    Article  PubMed  CAS  Google Scholar 

  359. Srivastava RN, Kalia A, Travis LB, Diven SC, Gugliuzza KK, Rajaraman S. Prompt remission of post-renal transplant nephrotic syndrome with high-dose cyclosporine. Pediatr Nephrol 1994;8(1):94–95.

    Article  PubMed  CAS  Google Scholar 

  360. Salomon R, Gagnadoux MF, Niaudet P. Intravenous cyclosporine therapy in recurrent nephrotic syndrome after renal transplantation in children. Transplantation 2003;75(6):810–814.

    Article  PubMed  CAS  Google Scholar 

  361. Raafat RH, Kalia A, Travis LB, Diven SC. High-dose oral cyclosporin therapy for recurrent focal segmental glomerulosclerosis in children. Am J Kidney Dis 2004;44(1):50–56.

    Article  PubMed  CAS  Google Scholar 

  362. McCauley J, Shapiro R, Jordan M, Scantlebury V, Vivas C, Jensen C et al. FK 506 in the management of nephrotic syndrome after renal transplantation. Transplant Proc 1993;25(1 Pt 2):1351–1354.

    PubMed  CAS  Google Scholar 

  363. Artero ML, Sharma R, Savin VJ, Vincenti F. Plasmapheresis reduces proteinuria and serum capacity to injure glomeruli in patients with recurrent focal glomerulosclerosis. Am J Kidney Dis 1994;23(4):574–581.

    PubMed  CAS  Google Scholar 

  364. Cochat P, Kassir A, Colon S, Glastre C, Tourniaire B, Parchoux B et al. Recurrent nephrotic syndrome after transplantation: early treatment with plasmaphaeresis and cyclophosphamide. Pediatr Nephrol 1993;7(1):50–54.

    Article  PubMed  CAS  Google Scholar 

  365. Li PK, Lai FM, Leung CB, Lui SF, Wang A, Lai KN. Plasma exchange in the treatment of early recurrent focal glomerulosclerosis after renal transplantation. Report and review. Am J Nephrol 1993;13(4):289–292.

    Article  PubMed  CAS  Google Scholar 

  366. Munoz J, Sanchez M, Perez-Garcia R, Anaya F, Valderrabano F. Recurrent focal glomerulosclerosis in renal transplants proteinuria relapsing following plasma exchange. Clin Nephrol 1985;24(4):213–214.

    PubMed  CAS  Google Scholar 

  367. Solomon LR, Cairns SA, Lawler W, Johnson RW, Mallick NP. Reduction of post-transplant proteinuria due to recurrent mesangial proliferative (IgM) glomerulonephritis following plasma exchange. Clin Nephrol 1981;16(1):44–50.

    PubMed  CAS  Google Scholar 

  368. Zimmerman SW. Plasmapheresis and dipyridamole for recurrent focal glomerular sclerosis. Nephron 1985;40(2):241–245.

    Article  PubMed  CAS  Google Scholar 

  369. Cameron JS. The enigma of focal segmental glomerulosclerosis. Kidney Int Suppl 1996;57:S119–S131.

    PubMed  CAS  Google Scholar 

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Niaudet, P., Boyer, O. (2009). Idiopathic Nephrotic Syndrome in Children: Clinical Aspects. In: Avner, E., Harmon, W., Niaudet, P., Yoshikawa, N. (eds) Pediatric Nephrology. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-540-76341-3_28

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