Clinical and Experimental Pediatrics

대한소아청소년과학회 (The Korean Pediatric Society)
권호 표지
DOI QR코드

DOI QR Code

Nephrotic syndrome: what's new, what's hot?

  • Kang, Hee Gyung (Department of Pediatrics, Research Coordination Center for Rare Diseases, Seoul National University Children's Hospital) ;
  • Cheong, Hae Il (Department of Pediatrics, Research Coordination Center for Rare Diseases, Seoul National University Children's Hospital)
  • 투고 : 2015.04.22
  • 심사 : 2015.06.18
  • 발행 : 2015.08.10
PDF 다운로드
⟨ 이전 논문 다음 논문 ⟩

초록

While the incidence of nephrotic syndrome (NS) is decreasing in Korea, the morbidity of difficult-to-treat NS is significant. Efforts to minimize treatment toxicity showed that prolonged treatment after an initial treatment for 2-3 months with glucocorticosteroids was not effective in reducing frequent relapses. For steroid-dependent NS, rituximab, a monoclonal antibody against the CD20 antigen on B cells, was proven to be as effective, and short-term daily low-dose steroids during upper respiratory infections reduced relapses. Steroid resistance or congenital NS are indications for genetic study and renal biopsy, since the list of genes involved in NS is lengthening.

키워드

참고문헌

  1. Eddy AA, Symons JM. Nephrotic syndrome in childhood. Lancet 2003;362:629-39. https://doi.org/10.1016/S0140-6736(03)14184-0
  2. 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;17:1151-7. https://doi.org/10.1681/ASN.2005090922
  3. Hoyer PF. New lessons from randomized trials in steroid-sensitive nephrotic syndrome: clear evidence against long steroid therapy. Kidney Int 2015;87:17-9. https://doi.org/10.1038/ki.2014.354
  4. Sureshkumar P, Hodson EM, Willis NS, Barzi F, Craig JC. Predictors of remission and relapse in idiopathic nephrotic syndrome: a prospective cohort study. Pediatr Nephrol 2014;29:1039-46. https://doi.org/10.1007/s00467-013-2736-9
  5. Teeninga N, Kist-van Holthe JE, van den Akker EL, Kersten MC, Boersma E, Krabbe HG, et al. Genetic and in vivo determinants of glucocorticoid sensitivity in relation to clinical outcome of childhood nephrotic syndrome. Kidney Int 2014;85:1444-53. https://doi.org/10.1038/ki.2013.531
  6. Tarshish P, Tobin JN, Bernstein J, Edelmann CM Jr. Prognostic significance of the early course of minimal change nephrotic syndrome: report of the International Study of Kidney Disease in Children. J Am Soc Nephrol 1997;8:769-76.
  7. Kausman JY, Powell HR. Paediatric nephrology: the last 50 years. J Paediatr Child Health 2015;51:94-7. https://doi.org/10.1111/jpc.12812
  8. Trautmann A, Bodria M, Ozaltin F, Gheisari A, Melk A, Azocar M, et al. Spectrum of steroid-resistant and congenital nephrotic syndrome in children: the PodoNet registry cohort. Clin J Am Soc Nephrol 2015;10:592-600. https://doi.org/10.2215/CJN.06260614
  9. Giglio S, Provenzano A, Mazzinghi B, Becherucci F, Giunti L, Sansavini G, et al. Heterogeneous genetic alterations in sporadic nephrotic syndrome associate with resistance to immunosuppression. J Am Soc Nephrol 2015;26:230-6. https://doi.org/10.1681/ASN.2013111155
  10. 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;58:1042-3. https://doi.org/10.1053/j.ajkd.2011.09.007
  11. Maas RJ, Deegens JK, Wetzels JF. Permeability factors in idiopathic nephrotic syndrome: historical perspectives and lessons for the future. Nephrol Dial Transplant 2014;29:2207-16. https://doi.org/10.1093/ndt/gfu355
  12. 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;17:952-60. https://doi.org/10.1038/nm.2411
  13. Spinale JM, Mariani LH, Kapoor S, Zhang J, Weyant R, Song PX, et al. A reassessment of soluble urokinase-type plasminogen activator receptor in glomerular disease. Kidney Int 2015;87:564-74. https://doi.org/10.1038/ki.2014.346
  14. Clement LC, Mace C, Del Nogal Avila M, Marshall CB, Chugh SS. The proteinuria-hypertriglyceridemia connection as a basis for novel therapeutics for nephrotic syndrome. Transl Res 2015;165:499-504. https://doi.org/10.1016/j.trsl.2014.06.004
  15. Clement LC, Mace 1, Avila-Casado C, Joles JA, Kersten S, Chugh SS. Circulating angiopoietin-like 4 links proteinuria with hypertriglyceridemia in nephrotic syndrome. Nat Med 2014;20:37-46. https://doi.org/10.1038/nm.3396
  16. Debiec H, Guigonis V, Mougenot B, Decobert F, Haymann JP, Bensman A, et al. Antenatal membranous glomerulonephritis due to anti-neutral endopeptidase antibodies. N Engl J Med 2002;346:2053-60. https://doi.org/10.1056/NEJMoa012895
  17. Beck LH Jr, Bonegio RG, Lambeau G, Beck DM, Powell DW, Cummins TD, et al. M-type phospholipase A2 receptor as target antigen in idiopathic membranous nephropathy. N Engl J Med 2009;361:11-21. https://doi.org/10.1056/NEJMoa0810457
  18. Tomas NM, Beck LH Jr, Meyer-Schwesinger C, Seitz-Polski B, Ma H, Zahner G, et al. Thrombospondin type-1 domain-containing 7A in idiopathic membranous nephropathy. N Engl J Med 2014;371:2277-87. https://doi.org/10.1056/NEJMoa1409354
  19. Hama T, Nakanishi K, Shima Y, Sato M, Mukaiyama H, Togawa H, et al. Renal biopsy criterion in idiopathic nephrotic syndrome with microscopic hematuria at onset. Pediatr Nephrol 2015;30:445-50. https://doi.org/10.1007/s00467-014-2946-9
  20. Sethi S, Glassock RJ, Fervenza FC. Focal segmental glomerulosclerosis: towards a better understanding for the practicing nephrologist. Nephrol Dial Transplant 2015;30:375-84. https://doi.org/10.1093/ndt/gfu035
  21. Ling C, Liu X, Shen Y, Chen Z, Fan J, Jiang Y, et al. Urinary CD80 levels as a diagnostic biomarker of minimal change disease. Pediatr Nephrol 2015;30:309-16. https://doi.org/10.1007/s00467-014-2915-3
  22. Chapter 3: Steroid-sensitive nephrotic syndrome in children. Kidney Int Suppl (2011) 2012;2:163-71. https://doi.org/10.1038/kisup.2012.16
  23. Chapter 4: Steroid-resistant nephrotic syndrome in children. Kidney Int Suppl (2011) 2012;2:172-6. https://doi.org/10.1038/kisup.2012.17
  24. Yoshikawa N, Nakanishi K, Sako M, Oba MS, Mori R, Ota E, et al. A multicenter randomized trial indicates initial prednisolone treatment for childhood nephrotic syndrome for two months is not inferior to six-month treatment. Kidney Int 2015;87:225-32. https://doi.org/10.1038/ki.2014.260
  25. Sinha A, Saha A, Kumar M, Sharma S, Afzal K, Mehta A, et al. Extending initial prednisolone treatment in a randomized control trial from 3 to 6 months did not significantly influence the course of illness in children with steroid-sensitive nephrotic syndrome. Kidney Int 2015;87:217-24. https://doi.org/10.1038/ki.2014.240
  26. Teeninga N, Kist-van Holthe JE, van Rijswijk N, de Mos NI, Hop WC, Wetzels JF, et al. Extending prednisolone treatment does not reduce relapses in childhood nephrotic syndrome. J Am Soc Nephrol 2013;24:149-59. https://doi.org/10.1681/ASN.2012070646
  27. Nozu K, Iijima K, Fujisawa M, Nakagawa A, Yoshikawa N, Matsuo M. Rituximab treatment for posttransplant lymphoproliferative disorder (PTLD) induces complete remission of recurrent nephrotic syndrome. Pediatr Nephrol 2005;20:1660-3. https://doi.org/10.1007/s00467-005-2013-7
  28. Iijima K, Sako M, Nozu K, Mori R, Tuchida N, Kamei K, et al. Rituximab for childhood-onset, complicated, frequently relapsing nephrotic syndrome or steroid-dependent nephrotic syndrome: a multicentre, double-blind, randomised, placebo-controlled trial. Lancet 2014;384:1273-81. https://doi.org/10.1016/S0140-6736(14)60541-9
  29. Ravani P, Rossi R, Bonanni A, Quinn RR, Sica F, Bodria M, et al. Rituximab in children with steroid-dependent nephrotic syndrome: a multicenter, open-label, noninferiority, randomized controlled trial. J Am Soc Nephrol 2015 Jan 15 [Epub]. http://dx.doi.org/10.1681/ASN.2014080799.
  30. Sinha A, Bhatia D, Gulati A, Rawat M, Dinda AK, Hari P, et al. Efficacy and safety of rituximab in children with difficult-to-treat nephrotic syndrome. Nephrol Dial Transplant 2015;30:96-106. https://doi.org/10.1093/ndt/gfu267
  31. Sato M, Ito S, Ogura M, Kamei K. Impact of rituximab on height and weight in children with refractory steroid-dependent nephrotic syndrome. Pediatr Nephrol 2014;29:1373-9. https://doi.org/10.1007/s00467-014-2792-9
  32. Kamei K, Takahashi M, Fuyama M, Saida K, Machida H, Sato M, et al. Rituximab-associated agranulocytosis in children with refractory idiopathic nephrotic syndrome: case series and review of literature. Nephrol Dial Transplant 2015;30:91-6. https://doi.org/10.1093/ndt/gfu258
  33. Fujinaga S, Hirano D. Risk factors for early relapse during maintenance therapy after a single infusion of rituximab in children with steroid-dependent nephrotic syndrome. Pediatr Nephrol 2014;29:491-2. https://doi.org/10.1007/s00467-013-2674-6
  34. Fujinaga S, Sakuraya K, Yamada A, Urushihara Y, Ohtomo Y, Shimizu T. Positive role of rituximab in switching from cyclosporine to mycophenolate mofetil for children with high-dose steroid-dependent nephrotic syndrome. Pediatr Nephrol 2015;30:687-91. https://doi.org/10.1007/s00467-014-3034-x
  35. Ahn YH, Kang HG, Lee JM, Choi HJ, Ha IS, Cheong HI. Development of antirituximab antibodies in children with nephrotic syndrome. Pediatr Nephrol 2014;29:1461-4. https://doi.org/10.1007/s00467-014-2794-7
  36. Basu B. Ofatumumab for rituximab-resistant nephrotic syndrome. N Engl J Med 2014;370:1268-70. https://doi.org/10.1056/NEJMc1308488
  37. Trachtman H, Savin VJ. Galactose treatment in focal segmental glomerulosclerosis. Pediatr Nephrol 2014;29:931. https://doi.org/10.1007/s00467-013-2700-8
  38. De Smet E, Rioux JP, Ammann H, Deziel C, Querin S. FSGS permeability factor-associated nephrotic syndrome: remission after oral galactose therapy. Nephrol Dial Transplant 2009;24:2938-40. https://doi.org/10.1093/ndt/gfp278
  39. Sgambat K, Banks M, Moudgil A. Effect of galactose on glomerular permeability and proteinuria in steroid-resistant nephrotic syndrome. Pediatr Nephrol 2013;28:2131-5. https://doi.org/10.1007/s00467-013-2539-z
  40. Uwaezuoke SN. Steroid-sensitive nephrotic syndrome in children: triggers of relapse and evolving hypotheses on pathogenesis. Ital J Pediatr 2015;41:19. https://doi.org/10.1186/s13052-015-0123-9
  41. Sinha A, Bagga A, Gulati A, Hari P. Short-term efficacy of rituximab versus tacrolimus in steroid-dependent nephrotic syndrome. Pediatr Nephrol 2012;27:235-41. https://doi.org/10.1007/s00467-011-1997-4
  42. Mattoo TK, Mahmoud MA. Increased maintenance corticosteroids during upper respiratory infection decrease the risk of relapse in nephrotic syndrome. Nephron 2000;85:343-5. https://doi.org/10.1159/000045684
  43. 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;93:226-8. https://doi.org/10.1136/adc.2007.116079
  44. Arun S, Bhatnagar S, Menon S, Saini S, Hari P, Bagga A. Efficacy of zinc supplements in reducing relapses in steroid-sensitive nephrotic syndrome. Pediatr Nephrol 2009;24:1583-6. https://doi.org/10.1007/s00467-009-1170-5
  45. Andolino TP, Reid-Adam J. Nephrotic syndrome. Pediatr Rev 2015;36:117-25. https://doi.org/10.1542/pir.36-3-117
  46. Sandberg DH, Bernstein CW, McIntosh RM, Carr R, Strauss J. Severe steroid-responsive nephrosis associated with hypersensitivity. Lancet 1977;1:388-91.
  47. Sieniawska M, Szymanik-Grzelak H, Kowalewska M, Wasik M, Koleska D. The role of cow's milk protein intolerance in steroidresistant nephrotic syndrome. Acta Paediatr 1992;81:1007-12. https://doi.org/10.1111/j.1651-2227.1992.tb12164.x
  48. Uy N, Graf L, Lemley KV, Kaskel F. Effects of gluten-free, dairyfree diet on childhood nephrotic syndrome and gut microbiota. Pediatr Res 2015;77:252-5. https://doi.org/10.1038/pr.2014.159
  49. Ishikura K, Yoshikawa N, Nakazato H, Sasaki S, Nakanishi K, Matsuyama T, et al. Morbidity in children with frequently relapsing nephrosis: 10-year follow-up of a randomized controlled trial. Pediatr Nephrol 2015;30:459-68. https://doi.org/10.1007/s00467-014-2955-8
  50. Ribeiro D, Zawadynski S, Pittet LF, Chevalley T, Girardin E, Parvex P. Effect of glucocorticoids on growth and bone mineral density in children with nephrotic syndrome. Eur J Pediatr 2015;174:911-7. https://doi.org/10.1007/s00431-014-2479-z
  51. Selewski DT, Troost JP, Massengill SF, Gbadegesin RA, Greenbaum LA, Shatat IF, et al. The impact of disease duration on quality of life in children with nephrotic syndrome: a Midwest Pediatric Nephrology Consortium study. Pediatr Nephrol 2015 Mar 18 [Epub]. http://dx.doi.org/10.1007/s00467-015-3074-x.

피인용 문헌

  1. Recent Advances in Treatments of Primary Focal Segmental Glomerulosclerosis in Children vol.2016, pp.None, 2016, https://doi.org/10.1155/2016/3053706
  2. Low-dose rituximab is no less effective for nephrotic syndrome measured by 12-month outcome vol.34, pp.5, 2019, https://doi.org/10.1007/s00467-018-4172-3
  3. Genetic Basis of Steroid Resistant Nephrotic Syndrome vol.23, pp.2, 2015, https://doi.org/10.3339/jkspn.2019.23.2.86
  4. Diversity and molecular network patterns of symptom phenotypes vol.7, pp.1, 2015, https://doi.org/10.1038/s41540-021-00206-5
  5. Reliability of generic quality-of-life instruments in assessing health-related quality of life among children and adolescents with idiopathic nephrotic syndrome: a systematic review vol.19, pp.1, 2021, https://doi.org/10.1186/s12955-021-01786-w