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Childhood Onset Nephrotic Syndrome

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Core Concepts in Parenchymal Kidney Disease

Abstract

Childhood onset nephrotic syndrome is one of the most common nephrology conditions affecting children. This chapter will review the epidemiology, pathophysiology, treatment options, and long-term outcome of nephrotic syndrome in children.

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References

  1. Nachman P, Jennette J, Falk R. Primary glomerular diseases. In: Brenner BM, editor. Brenner & Rector’s the kidney. 8th ed. Philadelphia: W.B. Saunders Company; 2008.

    Google Scholar 

  2. ISKDC. 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):159–65.

    Google Scholar 

  3. ISKDC. 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):561–4.

    Google Scholar 

  4. Mekahli D, Liutkus A, Ranchin B, Yu A, Bessenay L, Girardin E, et al. Long-term outcome of idiopathic steroid-resistant nephrotic syndrome: a multicenter study. Pediatr Nephrol. 2009;24(8):1525–32.

    PubMed  Google Scholar 

  5. 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):389–95.

    PubMed  Google Scholar 

  6. Hinkes BG, Mucha B, Vlangos CN, Gbadegesin R, Liu J, Hasselbacher K, et al. Nephrotic syndrome in the first year of life: two thirds of cases are caused by mutations in 4 genes (NPHS1, NPHS2, WT1, and LAMB2). Pediatrics. 2007;119(4):e907–19.

    PubMed  Google Scholar 

  7. Lee JH, Han KH, Lee H, Kang HG, Moon KC, Shin JI, et al. Genetic basis of congenital and infantile nephrotic syndromes. Am J Kidney Dis. 2011;58(6):1042–3.

    PubMed  Google Scholar 

  8. Machuca E, Benoit G, Nevo F, Tete MJ, Gribouval O, Pawtowski A, et al. Genotype-phenotype correlations in non-Finnish congenital nephrotic syndrome. J Am Soc Nephrol. 2010;21(7):1209–17.

    PubMed  CAS  Google Scholar 

  9. Mubarak M, Kazi JI, Lanewala A, Hashmi S, Akhter F. Pathology of idiopathic nephrotic syndrome in children: are the adolescents different from young children? Nephrol Dial Transplant. 2012;27(2):722–6.

    PubMed  Google Scholar 

  10. Zhou FD, Chen M. The renal histopathological spectrum of patients with nephrotic syndrome: an analysis of 1523 patients in a single Chinese center. Nephrol Dial Transplant. 2011;26:3993.

    PubMed  Google Scholar 

  11. McEnery PT, Strife CF. Nephrotic syndrome in childhood. Management and treatment in patients with minimal change disease, mesangial proliferation, or focal glomerulosclerosis. Pediatr Clin North Am. 1982;29(4):875–94.

    PubMed  CAS  Google Scholar 

  12. Lin CY, Lee BH, Lin CC, Chen WP. A study of the relationship between childhood nephrotic syndrome and allergic diseases. Chest. 1990;97(6):1408–11.

    PubMed  CAS  Google Scholar 

  13. Bonilla-Felix M, Parra C, Dajani T, Ferris M, Swinford RD, Portman RJ, et al. Changing patterns in the histopathology of idiopathic nephrotic syndrome in children. Kidney Int. 1999;55(5):1885–90.

    PubMed  CAS  Google Scholar 

  14. Gulati S, Sharma AP, Sharma RK, Gupta A. Changing trends of histopathology in childhood nephrotic syndrome. Am J Kidney Dis. 1999;34(4):646–50.

    PubMed  CAS  Google Scholar 

  15. Srivastava T, Simon SD, Alon US. High incidence of focal segmental glomerulosclerosis in nephrotic syndrome of childhood. Pediatr Nephrol. 1999;13(1):13–8.

    PubMed  CAS  Google Scholar 

  16. Banaszak B, Banaszak P. The increasing incidence of initial steroid resistance in childhood nephrotic syndrome. Pediatr Nephrol. 2012;27(6):927–32.

    PubMed  Google Scholar 

  17. Mundel P, Reiser J. Proteinuria: an enzymatic disease of the podocyte? Kidney Int. 2010;77(7):571–80.

    PubMed  CAS  Google Scholar 

  18. George B, Verma R, Soofi AA, Garg P, Zhang J, Park TJ, et al. Crk1/2-dependent signaling is necessary for podocyte foot process spreading in mouse models of glomerular disease. J Clin Invest. 2012;122(2):674–92.

    PubMed  CAS  Google Scholar 

  19. Clement LC, Avila-Casado C, Mace C, Soria E, Bakker WW, Kersten S, et al. Podocyte-secreted angiopoietin-like-4 mediates proteinuria in glucocorticoid-sensitive nephrotic syndrome. Nat Med. 2011;17(1):117–22.

    PubMed  CAS  Google Scholar 

  20. Garin EH, Diaz LN, Mu W, Wasserfall C, Araya C, Segal M, et al. Urinary CD80 excretion increases in idiopathic minimal-change disease. J Am Soc Nephrol. 2009;20(2):260–6.

    PubMed  CAS  Google Scholar 

  21. Russo LM, Sandoval RM, Campos SB, Molitoris BA, Comper WD, Brown D. Impaired tubular uptake explains albuminuria in early diabetic nephropathy. J Am Soc Nephrol. 2009;20(3):489–94.

    PubMed  Google Scholar 

  22. Russo LM, Sandoval RM, McKee M, Osicka TM, Collins AB, Brown D, et al. The normal kidney filters nephrotic levels of albumin retrieved by proximal tubule cells: retrieval is disrupted in nephrotic states. Kidney Int. 2007;71(6):504–13.

    PubMed  CAS  Google Scholar 

  23. McCarthy ET, Sharma M, Savin VJ. Circulating permeability factors in idiopathic nephrotic syndrome and focal segmental glomerulosclerosis. Clin J Am Soc Nephrol. 2010;5(11):2115–21.

    PubMed  Google Scholar 

  24. Wei C, El Hindi S, Li J, Fornoni A, Goes N, Sageshima J, et al. Circulating urokinase receptor as a cause of focal segmental glomerulosclerosis. Nat Med. 2011;17(8):952–60.

    PubMed  CAS  Google Scholar 

  25. Shalhoub RJ. Pathogenesis of lipoid nephrosis: a disorder of T-cell function. Lancet. 1974;2(7880):556–60.

    PubMed  CAS  Google Scholar 

  26. Liu LL, Qin Y, Cai JF, Wang HY, Tao JL, Li H, et al. Th17/Treg imbalance in adult patients with minimal change nephrotic syndrome. Clin Immunol. 2011;139(3):314–20.

    PubMed  CAS  Google Scholar 

  27. 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):2732–9.

    PubMed  Google Scholar 

  28. Schrier RW, Fassett RG. A critique of the overfill hypothesis of sodium and water retention in the nephrotic syndrome. Kidney Int. 1998;53(5):1111–7.

    PubMed  CAS  Google Scholar 

  29. Couser WG. Basic and translational concepts of immune-mediated glomerular diseases. J Am Soc Nephrol. 2012;23(3):381–99.

    PubMed  CAS  Google Scholar 

  30. Hodgin JB, Rasoulpour M, Markowitz GS, D'Agati VD. Very low birth weight is a risk factor for secondary focal segmental glomerulosclerosis. Clin J Am Soc Nephrol. 2009;4(1):71–6.

    PubMed  Google Scholar 

  31. Ritz E, Koleganova N, Piecha G. Is there an obesity-metabolic syndrome related glomerulopathy? Curr Opin Nephrol Hypertens. 2011;20(1):44–9.

    PubMed  Google Scholar 

  32. Hildebrandt F. Genetic kidney diseases. Lancet. 2010;375(9722):1287–95.

    PubMed  CAS  Google Scholar 

  33. D’Agati VD, Kaskel FJ, Falk RJ. Focal segmental glomerulosclerosis. N Engl J Med. 2011;365(25):2398–411.

    PubMed  Google Scholar 

  34. Has C, Sparta G, Kiritsi D, Weibel L, Moeller A, Vega-Warner V, et al. Integrin alpha3 mutations with kidney, lung, and skin disease. N Engl J Med. 2012;366(16):1508–14.

    PubMed  CAS  Google Scholar 

  35. Heeringa SF, Chernin G, Chaki M, Zhou W, Sloan AJ, Ji Z, et al. COQ6 mutations in human patients produce nephrotic syndrome with sensorineural deafness. J Clin Invest. 2011;121(5):2013–24.

    PubMed  CAS  Google Scholar 

  36. Mucha B, Ozaltin F, Hinkes BG, Hasselbacher K, Ruf RG, Schultheiss M, et al. Mutations in the Wilms’ tumor 1 gene cause isolated steroid resistant nephrotic syndrome and occur in exons 8 and 9. Pediatr Res. 2006;59(2):325–31.

    PubMed  CAS  Google Scholar 

  37. Benoit G, Machuca E, Antignac C. Hereditary nephrotic syndrome: a systematic approach for genetic testing and a review of associated podocyte gene mutations. Pediatr Nephrol. 2010;25(9):1621–32.

    PubMed  Google Scholar 

  38. Rood IM, Deegens JK, Wetzels JF. Genetic causes of focal segmental glomerulosclerosis: implications for clinical practice. Nephrol Dial Transplant. 2012;27(3):882–90.

    PubMed  CAS  Google Scholar 

  39. Santin S, Tazon-Vega B, Silva I, Cobo MA, Gimenez I, Ruiz P, et al. Clinical value of NPHS2 analysis in early- and adult-onset steroid-resistant nephrotic syndrome. Clin J Am Soc Nephrol. 2011;6(2):344–54.

    PubMed  CAS  Google Scholar 

  40. Schoeb DS, Chernin G, Heeringa SF, Matejas V, Held S, Vega-Warner V, et al. Nineteen novel NPHS1 mutations in a worldwide cohort of patients with congenital nephrotic syndrome (CNS). Nephrol Dial Transplant. 2010;25(9):2970–6.

    PubMed  CAS  Google Scholar 

  41. Santin S, Garcia-Maset R, Ruiz P, Gimenez I, Zamora I, Pena A, et al. Nephrin mutations cause childhood- and adult-onset focal segmental glomerulosclerosis. Kidney Int. 2009;76(12):1268–76.

    PubMed  CAS  Google Scholar 

  42. Lowik M, Levtchenko E, Westra D, Groenen P, Steenbergen E, Weening J, et al. Bigenic heterozygosity and the development of steroid-resistant focal segmental glomerulosclerosis. Nephrol Dial Transplant. 2008;23(10):3146–51.

    PubMed  Google Scholar 

  43. Hinkes B, Vlangos C, Heeringa S, Mucha B, Gbadegesin R, Liu J, et al. Specific podocin mutations correlate with age of onset in steroid-resistant nephrotic syndrome. J Am Soc Nephrol. 2008;19(2):365–71.

    PubMed  Google Scholar 

  44. Chernin G, Heeringa SF, Gbadegesin R, Liu J, Hinkes BG, Vlangos CN, et al. Low prevalence of NPHS2 mutations in African American children with steroid-resistant nephrotic syndrome. Pediatr Nephrol. 2008;23(9):1455–60.

    PubMed  Google Scholar 

  45. Mannikko M, Kestaila M, Holmberg C, Norio R, Ryynanen M, Olsen A, et al. Fine mapping and haplotype analysis of the locus for congenital nephrotic syndrome on chromosome 19q13.1. Am J Hum Genet. 1995;57(6):1377–83.

    PubMed  CAS  Google Scholar 

  46. Koziell A, Grech V, Hussain S, Lee G, Lenkkeri U, Tryggvason K, et al. Genotype/phenotype correlations of NPHS1 and NPHS2 mutations in nephrotic syndrome advocate a functional inter-relationship in glomerular filtration. Hum Mol Genet. 2002;11(4):379–88.

    PubMed  CAS  Google Scholar 

  47. Frishberg Y, Rinat C, Megged O, Shapira E, Feinstein S, Raas-Rothschild A. Mutations in NPHS2 encoding podocin are a prevalent cause of steroid-resistant nephrotic syndrome among Israeli-Arab children. J Am Soc Nephrol. 2002;13(2):400–5.

    PubMed  CAS  Google Scholar 

  48. Philippe A, Nevo F, Esquivel EL, Reklaityte D, Gribouval O, Tete MJ, et al. Nephrin mutations can cause childhood-onset steroid-resistant nephrotic syndrome. J Am Soc Nephrol. 2008;19(10):1871–8.

    PubMed  CAS  Google Scholar 

  49. Ruf RG, Schultheiss M, Lichtenberger A, Karle SM, Zalewski I, Mucha B, et al. Prevalence of WT1 mutations in a large cohort of patients with steroid-resistant and steroid-sensitive nephrotic syndrome. Kidney Int. 2004;66(2):564–70.

    PubMed  CAS  Google Scholar 

  50. Caridi G, Perfumo F, Ghiggeri GM. NPHS2 (Podocin) mutations in nephrotic syndrome. Clinical spectrum and fine mechanisms. Pediatr Res. 2005;57(5 Pt 2):54R–61.

    PubMed  CAS  Google Scholar 

  51. Boute N, Gribouval O, Roselli S, Benessy F, Lee H, Fuchshuber A, et al. NPHS2, encoding the glomerular protein podocin, is mutated in autosomal recessive steroid-resistant nephrotic syndrome. Nat Genet. 2000;24(4):349–54.

    PubMed  CAS  Google Scholar 

  52. Karle SM, Uetz B, Ronner V, Glaeser L, Hildebrandt F, Fuchshuber A. Novel mutations in NPHS2 detected in both familial and sporadic steroid-resistant nephrotic syndrome. J Am Soc Nephrol. 2002;13(2):388–93.

    PubMed  CAS  Google Scholar 

  53. Boyer O, Benoit G, Gribouval O, Nevo F, Pawtowski A, Bilge I, et al. Mutational analysis of the PLCE1 gene in steroid resistant nephrotic syndrome. J Med Genet. 2010;47(7):445–52.

    PubMed  CAS  Google Scholar 

  54. Mbarek IB, Abroug S, Omezzine A, Pawtowski A, Gubler MC, Bouslama A, et al. Novel mutations in steroid-resistant nephrotic syndrome diagnosed in Tunisian children. Pediatr Nephrol. 2011;26(2):241–9.

    PubMed  Google Scholar 

  55. Gbadegesin RA, Lavin PJ, Hall G, Bartkowiak B, Homstad A, Jiang R, et al. Inverted formin 2 mutations with variable expression in patients with sporadic and hereditary focal and segmental glomerulosclerosis. Kidney Int. 2012;81(1):94–9.

    PubMed  CAS  Google Scholar 

  56. Gigante M, Caridi G, Montemurno E, Soccio M, d’Apolito M, Cerullo G, et al. TRPC6 mutations in children with steroid-resistant nephrotic syndrome and atypical phenotype. Clin J Am Soc Nephrol. 2011;6(7):1626–34.

    PubMed  CAS  Google Scholar 

  57. Benoit G, Machuca E, Nevo F, Gribouval O, Lepage D, Antignac C. Analysis of recessive CD2AP and ACTN4 mutations in steroid-resistant nephrotic syndrome. Pediatr Nephrol. 2010;25(3):445–51.

    PubMed  Google Scholar 

  58. Ruf RG, Lichtenberger A, Karle SM, Haas JP, Anacleto FE, Schultheiss M, et al. Patients with mutations in NPHS2 (podocin) do not respond to standard steroid treatment of nephrotic syndrome. J Am Soc Nephrol. 2004;15(3):722–32.

    PubMed  Google Scholar 

  59. Buscher AK, Kranz B, Buscher R, Hildebrandt F, Dworniczak B, Pennekamp P, et al. Immunosuppression and renal outcome in congenital and pediatric steroid-resistant nephrotic syndrome. Clin J Am Soc Nephrol. 2010;5(11):2075–84.

    PubMed  Google Scholar 

  60. Weber S, Gribouval O, Esquivel EL, Moriniere V, Tete MJ, Legendre C, et al. NPHS2 mutation analysis shows genetic heterogeneity of steroid-resistant nephrotic syndrome and low post-transplant recurrence. Kidney Int. 2004;66(2):571–9.

    PubMed  CAS  Google Scholar 

  61. Gbadegesin R, Hinkes B, Vlangos C, Mucha B, Liu J, Hopcian J, et al. Mutational analysis of NPHS2 and WT1 in frequently relapsing and steroid-dependent nephrotic syndrome. Pediatr Nephrol. 2007;22(4):509–13.

    PubMed  Google Scholar 

  62. Schwaderer P, Knuppel T, Konrad M, Mehls O, Scharer K, Schaefer F, et al. Clinical course and NPHS2 analysis in patients with late steroid-resistant nephrotic syndrome. Pediatr Nephrol. 2008;23(2):251–6.

    PubMed  Google Scholar 

  63. Hinkes B, Wiggins RC, Gbadegesin R, Vlangos CN, Seelow D, Nurnberg G, et al. Positional cloning uncovers mutations in PLCE1 responsible for a nephrotic syndrome variant that may be reversible. Nat Genet. 2006;38(12):1397–405.

    PubMed  CAS  Google Scholar 

  64. Kitamura A, Tsukaguchi H, Hiramoto R, Shono A, Doi T, Kagami S, et al. A familial childhood-onset relapsing nephrotic syndrome. Kidney Int. 2007;71(9):946–51.

    PubMed  CAS  Google Scholar 

  65. Ovunc B, Otto EA, Vega-Warner V, Saisawat P, Ashraf S, Ramaswami G, et al. Exome sequencing reveals cubilin mutation as a single-gene cause of proteinuria. J Am Soc Nephrol. 2011;22(10):1815–20.

    PubMed  CAS  Google Scholar 

  66. Sanna-Cherchi S, Burgess KE, Nees SN, Caridi G, Weng PL, Dagnino M, et al. Exome sequencing identified MYO1E and NEIL1 as candidate genes for human autosomal recessive steroid-resistant nephrotic syndrome. Kidney Int. 2011;80(4):389–96.

    PubMed  CAS  Google Scholar 

  67. Mele C, Iatropoulos P, Donadelli R, Calabria A, Maranta R, Cassis P, et al. MYO1E mutations and childhood familial focal segmental glomerulosclerosis. N Engl J Med. 2011;365(4):295–306.

    PubMed  CAS  Google Scholar 

  68. Franceschini N, North KE, Kopp JB, McKenzie L, Winkler C. NPHS2 gene, nephrotic syndrome and focal segmental glomerulosclerosis: a HuGE review. Genet Med. 2006;8(2):63–75.

    PubMed  CAS  Google Scholar 

  69. Caridi G, Gigante M, Ravani P, Trivelli A, Barbano G, Scolari F, et al. Clinical features and long-term outcome of nephrotic syndrome associated with heterozygous NPHS1 and NPHS2 mutations. Clin J Am Soc Nephrol. 2009;4(6):1065–72.

    PubMed  CAS  Google Scholar 

  70. Machuca E, Hummel A, Nevo F, Dantal J, Martinez F, Al-Sabban E, et al. Clinical and epidemiological assessment of steroid-resistant nephrotic syndrome associated with the NPHS2 R229Q variant. Kidney Int. 2009;75(7):727–35.

    PubMed  CAS  Google Scholar 

  71. Kopp JB, Smith MW, Nelson GW, Johnson RC, Freedman BI, Bowden DW, et al. MYH9 is a major-effect risk gene for focal segmental glomerulosclerosis. Nat Genet. 2008;40(10):1175–84.

    PubMed  CAS  Google Scholar 

  72. Genovese G, Friedman DJ, Ross MD, Lecordier L, Uzureau P, Freedman BI, et al. Association of trypanolytic ApoL1 variants with kidney disease in African Americans. Science. 2010;329(5993):841–5.

    PubMed  CAS  Google Scholar 

  73. Gipson DS, Massengill SF, Yao L, Nagaraj S, Smoyer WE, Mahan JD, et al. Management of childhood onset nephrotic syndrome. Pediatrics. 2009;124(2):747–57.

    PubMed  Google Scholar 

  74. Hogg RJ, Portman RJ, Milliner D, Lemley KV, Eddy A, Ingelfinger J. Evaluation and management of proteinuria and nephrotic syndrome in children: recommendations from a pediatric nephrology panel established at the National Kidney Foundation conference on proteinuria, albuminuria, risk, assessment, detection, and elimination (PARADE). Pediatrics. 2000;105(6):1242–9.

    PubMed  CAS  Google Scholar 

  75. Abitbol C, Zilleruelo G, Freundlich M, Strauss J. Quantitation of proteinuria with urinary protein/creatinine ratios and random testing with dipsticks in nephrotic children. J Pediatr. 1990;116(2):243–7.

    PubMed  CAS  Google Scholar 

  76. Bak M, Serdaroglu E, Guclu R. Prophylactic calcium and vitamin D treatments in steroid-treated children with nephrotic syndrome. Pediatr Nephrol. 2006;21(3):350–4.

    PubMed  Google Scholar 

  77. Chesney R. The changing face of childhood nephrotic syndrome. Kidney Int. 2004;66(3):1294–302.

    PubMed  Google Scholar 

  78. Dijkman HB, Wetzels JF, Gemmink JH, Baede J, Levtchenko EN, Steenbergen EJ. Glomerular involution in children with frequently relapsing minimal change nephrotic syndrome: an unrecognized form of glomerulosclerosis? Kidney Int. 2007;71(1):44–52.

    PubMed  CAS  Google Scholar 

  79. Stadermann MB, Lilien MR, van de Kar NC, Monnens LA, Schroder CH. Is biopsy required prior to cyclophosphamide in steroid-sensitive nephrotic syndrome? Clin Nephrol. 2003;60(5):315–7.

    PubMed  CAS  Google Scholar 

  80. Tain YL, Lin G, Cher TW. Microbiological spectrum of septicemia and peritonitis in nephrotic children. Pediatr Nephrol. 1999;13(9):835–7.

    PubMed  CAS  Google Scholar 

  81. Feinstein EI, Chesney RW, Zelikovic I. Peritonitis in childhood renal disease. Am J Nephrol. 1988;8(2):147–65.

    PubMed  CAS  Google Scholar 

  82. Lilova MI, Velkovski IG, Topalov IB. Thromboembolic complications in children with nephrotic syndrome in Bulgaria (1974–1996). Pediatr Nephrol. 2000;15(1–2):74–8.

    PubMed  CAS  Google Scholar 

  83. Mehls O, Andrassy K, Koderisch J, Herzog U, Ritz E. Hemostasis and thromboembolism in children with nephrotic syndrome: differences from adults. J Pediatr. 1987;110(6):862–7.

    PubMed  CAS  Google Scholar 

  84. Monagle P, Chan AK, Goldenberg NA, Ichord RN, Journeycake JM, Nowak-Gottl U, et al. Antithrombotic therapy in neonates and children: antithrombotic therapy and prevention of thrombosis, 9th ed: American College of Chest Physicians Evidence-Based Clinical Practice Guidelines. Chest. 2012;141(2 Suppl):e737S–801.

    PubMed  CAS  Google Scholar 

  85. Cameron JS. The nephrotic syndrome and its complications. Am J Kidney Dis. 1987;10(3):157–71.

    PubMed  CAS  Google Scholar 

  86. Vivarelli M, Moscaritolo E, Tsalkidis A, Massella L, Emma F. Time for initial response to steroids is a major prognostic factor in idiopathic nephrotic syndrome. J Pediatr. 2010;156(6):965–71.

    PubMed  Google Scholar 

  87. 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):1919–27.

    PubMed  CAS  Google Scholar 

  88. Brodehl J. The treatment of minimal change nephrotic syndrome: lessons learned from multicentre co-operative studies. Eur J Pediatr. 1991;150(6):380–7.

    PubMed  CAS  Google Scholar 

  89. 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.

    PubMed  CAS  Google Scholar 

  90. 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):550–7.

    PubMed  Google Scholar 

  91. 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):592–7.

    PubMed  CAS  Google Scholar 

  92. Orth SR, Ritz E. Nephrotic syndrome. Internist (Berl). 1998;39(12):1246–52.

    CAS  Google Scholar 

  93. Baudouin V, Alberti C, Lapeyraque AL, Bensman A, Andre JL, Broux F, et al. Mycophenolate mofetil for steroid-dependent nephrotic syndrome: a phase II Bayesian trial. Pediatr Nephrol. 2012;27(3):389–96.

    PubMed  Google Scholar 

  94. Hiraoka M, Tsukahara H, Hori C, Ohshima Y, Momoi T, Seo A, et al. Efficacy of long-term azathioprine for relapsing nephrotic syndrome. Pediatr Nephrol. 2000;14(8–9):776–8.

    PubMed  CAS  Google Scholar 

  95. 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):1265–8.

    PubMed  Google Scholar 

  96. 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):1848–54.

    PubMed  CAS  Google Scholar 

  97. Roberti I, Vyas S. Long-term outcome of children with steroid-resistant nephrotic syndrome treated with tacrolimus. Pediatr Nephrol. 2010;25(6):1117–24.

    PubMed  Google Scholar 

  98. Gulati A, Sinha A, Jordan SC, Hari P, Dinda AK, Sharma S, et al. Efficacy and safety of treatment with rituximab for difficult steroid-resistant and -dependent nephrotic syndrome: multicentric report. Clin J Am Soc Nephrol. 2010;5(12):2207–12.

    PubMed  CAS  Google Scholar 

  99. Sellier-Leclerc AL, Macher MA, Loirat C, Guerin V, Watier H, Peuchmaur M, et al. Rituximab efficiency in children with steroid-dependent nephrotic syndrome. Pediatr Nephrol. 2010;25(6):1109–15.

    PubMed  Google Scholar 

  100. van Husen M, Kemper MJ. New therapies in steroid-sensitive and steroid-resistant idiopathic nephrotic syndrome. Pediatr Nephrol. 2011;26(6):881–92.

    PubMed  Google Scholar 

  101. Magnasco A, Ravani P, Edefonti A, Murer L, Ghio L, Belingheri M, et al. Rituximab in children with resistant idiopathic nephrotic syndrome. J Am Soc Nephrol. 2012;23(6):1117–24.

    PubMed  CAS  Google Scholar 

  102. Lechner BL, Bockenhauer D, Iragorri S, Kennedy TL, Siegel NJ. The risk of cardiovascular disease in adults who have had childhood nephrotic syndrome. Pediatr Nephrol. 2004;19(7):744–8.

    PubMed  Google Scholar 

  103. Kyrieleis HA, Lowik MM, Pronk I, Cruysberg HR, Kremer JA, Oyen WJ, et al. Long-term outcome of biopsy-proven, frequently relapsing minimal-change nephrotic syndrome in children. Clin J Am Soc Nephrol. 2009;4(10):1593–600.

    PubMed  Google Scholar 

  104. 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):202–7.

    PubMed  Google Scholar 

  105. Burnham JM, Shults J, Petit MA, Semeao E, Beck TJ, Zemel BS, et al. Alterations in proximal femur geometry in children treated with glucocorticoids for Crohn disease or nephrotic syndrome: impact of the underlying disease. J Bone Miner Res. 2007;22(4):551–9.

    PubMed  CAS  Google Scholar 

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Trachtman, H., Sampson, M., Sethna, C.B., Gipson, D.S. (2014). Childhood Onset Nephrotic Syndrome. In: Fervenza, F., Lin, J., Sethi, S., Singh, A. (eds) Core Concepts in Parenchymal Kidney Disease. Springer, New York, NY. https://doi.org/10.1007/978-1-4614-8166-9_3

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