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Nephrotisches Syndrom im Kindesalter

Nephrotic syndrome in childhood

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Zusammenfassung

Das nephrotische Syndrom im Kindesalter erfüllt zwar die Kriterien einer seltenen Erkrankung, ist aber gleichzeitig die häufigste Glomerulopathie in dieser Altersgruppe. Die Diagnose wird über den Nachweis einer großen Proteinurie (täglich >1 g/m2 Körperoberfläche und Tag) und Hypalbuminämie (<2,5 g/dl) gestellt. Ödeme sind bei der Erstmanifestation auch meist vorhanden. Die Heterogenität der Erkrankung bezüglich Alter bei Erstmanifestation, Ursache, histologischen Veränderungen und Ansprechen auf eine Standardtherapie mit Glukokortikoiden beeinflusst die Differenzialdiagnostik ebenso wie die Therapie. Da die Prognose des steroidsensiblen nephrotischen Syndroms hinsichtlich der Nierenfunktion generell gut ist, müssen neben der Wirksamkeit einer immunsuppressiven Medikation auch die potenziellen Nebenwirkungsspektren einzelner Medikamente in die Planung der Behandlung einbezogen werden.

Abstract

Despite fulfilling the criteria for an orphan disease, idiopathic nephrotic syndrome in children is the most frequent glomerular disease in this age group. Nephrotic syndrome is defined by the detection of a high proteinuria (>1 g/m2 body surface area daily) and hypoalbuminemia (<2,5 g/dl). Edema is also mostly present, especially at the first manifestation. Nephrotic syndrome in children is a heterogeneous disease with respect to age of onset, etiology, histological alterations and response to a standard treatment of glucocorticoids. This diversity strongly influences both the differential diagnostic approach and the treatment. As the prognosis of steroid-sensitive nephrotic syndrome with respect to renal function is generally good, not only the efficacy of an immunosuppressive medication but also the side effect profiles of different immunosuppressive regimens should be kept in mind for treatment planning and optimization.

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Literatur

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

    Article  CAS  Google Scholar 

  2. Anonymous (1981) 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 98:561–564

    Article  Google Scholar 

  3. AWMF, Gesellschaft für Pädiatrische Nephrologie (GPN) (Hrsg) (2016) AWMF Leitlinie (S2 e): Idiopathisches Nephrotisches Syndrom im Kindesalter: Diagnostik und Therapie. AWMF online. https://www.awmf.org/uploads/tx_szleitlinien/166-001l_S2e_Idiopathisches_Nephrotisches_Syndrom_Kinderalter_2017-04.pdf

    Google Scholar 

  4. Benz M (2011) Pipilotta und der Nierendetektiv, Familienschulung des nephrotischen Syndroms. Monatsschr Kinderheilkd 159:172

    Google Scholar 

  5. Benz Mr KM (2015) Nephrotisches Syndrom im Kindesalter. Monatsschr Kinderheilkd 163:310–322

    Article  Google Scholar 

  6. Benz M, Weber L (2012) Nephrotisches Syndrom im Kindesalter. Monatsschr Kinderheilkd 160(8):787–804

    Article  Google Scholar 

  7. Benz MR, Tönshoff B, Weber LT (2014) Treatment of children with frequent relapsing steroid-sensitive nephrotic syndrome: recent trial results. Clin Investig 4(11):1043–1054

    Article  CAS  Google Scholar 

  8. Brodehl J (1991) The treatment of minimal change nephrotic syndrome: lessons learned from multicentre co-operative studies. Eur J Pediatr 150:380–387

    Article  CAS  Google Scholar 

  9. Cammas B, Harambat J, Bertholet-Thomas A et al (2011) Long-term effects of cyclophosphamide therapy in steroid-dependent or frequently relapsing idiopathic nephrotic syndrome. Nephrol Dial Transplant 26:178–184

    Article  CAS  Google Scholar 

  10. Choudhry S, Bagga A, Hari P et al (2009) Efficacy and safety of tacrolimus versus cyclosporine in children with steroid-resistant nephrotic syndrome: a randomized controlled trial. Am J Kidney Dis 53:760–769

    Article  CAS  Google Scholar 

  11. Debiec H, Dossier C, Letouze E et al (2018) Transethnic, genome-wide analysis reveals immune-related risk alleles and phenotypic correlates in pediatric steroid-sensitive nephrotic syndrome. J Am Soc Nephrol 29:2000–2013

    Article  CAS  Google Scholar 

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

    Article  Google Scholar 

  13. Ehren R, Benz MR, Doetsch J et al (2018) Initial treatment of steroid-sensitive idiopathic nephrotic syndrome in children with mycophenolate mofetil versus prednisone: protocol for a randomised, controlled, multicentre trial (INTENT study). BMJ Open 8:e24882

    Article  Google Scholar 

  14. Fujinaga S, Endo A, Ohtomo Y et al (2013) Uncertainty in management of childhood-onset idiopathic nephrotic syndrome: is the long-term prognosis really favorable? Pediatr Nephrol 28:2235–2238

    Article  Google Scholar 

  15. Gbadegesin RA, Adeyemo A, Webb NJ et al (2015) HLA-DQA1 and PLCG2 are candidate risk loci for childhood-onset steroid-sensitive nephrotic syndrome. J Am Soc Nephrol 26:1701–1710

    Article  CAS  Google Scholar 

  16. Gee HY, Ashraf S, Wan X et al (2014) Mutations in EMP2 cause childhood-onset nephrotic syndrome. Am J Hum Genet 94:884–890

    Article  CAS  Google Scholar 

  17. Gellermann J, Ehrich JH, Querfeld U (2012) Sequential maintenance therapy with cyclosporin A and mycophenolate mofetil for sustained remission of childhood steroid-resistant nephrotic syndrome. Nephrol Dial Transplant 27:1970–1978

    Article  CAS  Google Scholar 

  18. Gipson DS, Massengill SF, Yao L et al (2009) Management of childhood onset nephrotic syndrome. Pediatr Electron Pages 124:747–757

    Google Scholar 

  19. Gulati A, Sinha A, Sreenivas V et al (2011) Daily corticosteroids reduce infection-associated relapses in frequently relapsing nephrotic syndrome: a randomized controlled trial. Clin J Am Soc Nephrol 6:63–69

    Article  CAS  Google Scholar 

  20. Hackl A, Cseprekal O, Gessner M et al (2016) Mycophenolate mofetil therapy in children with idiopathic nephrotic syndrome: does therapeutic drug monitoring make a difference? Ther Drug Monit 38:274–279

    Article  CAS  Google Scholar 

  21. Hahn D, Hodson EM, Willis NS et al (2015) Corticosteroid therapy for nephrotic syndrome in children. Cochrane Database Syst Rev. https://doi.org/10.1002/14651858.CD001533.pub5

    Article  PubMed  Google Scholar 

  22. Hoyer PF (2015) New lessons from randomized trials in steroid-sensitive nephrotic syndrome: clear evidence against long steroid therapy. Kidney Int 87:17–19

    Article  CAS  Google Scholar 

  23. Iijima K, Sako M, Nozu K et al (2014) Rituximab for childhood-onset, complicated, frequently relapsing nephrotic syndrome or steroid-dependent nephrotic syndrome: a multicentre, double-blind, randomised, placebo-controlled trial. Lancet 384:1273–1281

    Article  CAS  Google Scholar 

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

    Article  CAS  Google Scholar 

  25. Jia X, Horinouchi T, Hitomi Y et al (2018) Strong association of the HLA-DR/DQ locus with childhood steroid-sensitive nephrotic syndrome in the Japanese population. J Am Soc Nephrol 29:2189–2199

    Article  CAS  Google Scholar 

  26. Kamei K, Miyairi I, Ishikura K et al (2018) Prospective study of live attenuated vaccines for patients with nephrotic syndrome receiving immunosuppressive agents. J Pediatr 196:217–222e1

    Article  CAS  Google Scholar 

  27. Kemper Mj LA, Zawischa A, Oh J (2014) Is rituximab effective in childhood nephrotic syndrome? Yes and no. Pediatr Nephrol 29:1305–1311

    Article  Google Scholar 

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

    Article  CAS  Google Scholar 

  29. Lawrenz, CE (2010) Inzidenz-Analyse des Nephrotischen Syndroms im Kindes- und Jugendalter in den Jahren 2005 und 2006. Dissertation. http://hss.ulb.uni-bonn.de/2010/2170/2170.pdf

  30. Lombel RM, Gipson DS, Hodson EM et al (2013) Treatment of steroid-sensitive nephrotic syndrome: new guidelines from KDIGO. Pediatr Nephrol 28:415–426

    Article  Google Scholar 

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

    Article  CAS  Google Scholar 

  32. Minamikawa S, Nozu K, Maeta S et al (2018) The utility of urinary CD80 as a diagnostic marker in patients with renal diseases. Sci Rep 8:17322

    Article  Google Scholar 

  33. Schijvens AM, Dorresteijn EM, Roeleveld N et al (2017) REducing STEroids in Relapsing Nephrotic syndrome: the RESTERN study-protocol of a national, double-blind, randomised, placebo-controlled, non-inferiority intervention study. BMJ Open 7:e18148

    Article  CAS  Google Scholar 

  34. Sinha A, Saha A, Kumar M et al (2015) 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 87:217–224

    Article  CAS  Google Scholar 

  35. Sinha A, Puraswani M, Kalaivani M et al (2019) Efficacy and safety of mycophenolate mofetil versus levamisole in frequently relapsing nephrotic syndrome: an open-label randomized controlled trial. Kidney Int 95:210–218

    Article  CAS  Google Scholar 

  36. Sobiak J, Resztak M, Ostalska-Nowicka D et al (2015) Monitoring of mycophenolate mofetil metabolites in children with nephrotic syndrome and the proposed novel target values of pharmacokinetic parameters. Eur J Pharm Sci 77:189–196

    Article  CAS  Google Scholar 

  37. Teeninga N, Kist-Van Holthe JE, Van Rijswijk N et al (2013) Extending prednisolone treatment does not reduce relapses in childhood nephrotic syndrome. J Am Soc Nephrol 24:149–159

    Article  CAS  Google Scholar 

  38. Vogd J, Hackl A, Habbig S et al (2019) Discontinuation of maintenance therapy in frequently relapsing nephrotic syndrome. Clin Nephrol 91:25–31

    Article  Google Scholar 

  39. Webb NJ, Frew E, Brettell EA et al (2014) Short course daily prednisolone therapy during an upper respiratory tract infection in children with relapsing steroid-sensitive nephrotic syndrome (PREDNOS 2): protocol for a randomised controlled trial. Trials 15:147

    Article  Google Scholar 

  40. Weber LT (2015) Therapeutic drug monitoring in pediatric renal transplantation. Pediatr Nephrol 30:253–265

    Article  Google Scholar 

  41. Wei CC, Lin CL, Shen TC et al (2015) Risk of idiopathic nephrotic syndrome among children with asthma: a nationwide, population-based cohort study. Pediatr Res 78:212–217

    Article  Google Scholar 

  42. Wei CC, Lin CL, Shen TC et al (2015) Occurrence of common allergic diseases in children with idiopathic nephrotic syndrome. J Epidemiol 25:370–377

    Article  Google Scholar 

  43. Yoshikawa N, Nakanishi K, Sako M et al (2015) A multicenter randomized trial indicates initial prednisolone treatment for childhood nephrotic syndrome for two months is not inferior to six-month treatment. Kidney Int 87:225–232

    Article  CAS  Google Scholar 

  44. Zhang L, Dai Y, Peng W et al (2009) Genome-wide analysis of histone H3 lysine 4 trimethylation in peripheral blood mononuclear cells of minimal change nephrotic syndrome patients. Am J Nephrol 30:505–51342

    Article  Google Scholar 

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Author information

Authors and Affiliations

  1. Pädiatrische Nephrologie, Klinik für Kinder- und Jugendmedizin, Medizinische Fakultät und Uniklinik Köln, Universität zu Köln, Kerpener Str. 62, 50937, Köln, Deutschland

    M. R. Benz, R. Ehren & L. T. Weber

  2. Kindernephrologie in Dachau, Dachau, Deutschland

    M. R. Benz

  3. Klinik Kinderheilkunde I, Zentrum für Kinder- und Jugendmedizin, Universitätsklinikum Heidelberg, Heidelberg, Deutschland

    B. Tönshoff

Authors
  1. M. R. Benz
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  2. R. Ehren
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  3. B. Tönshoff
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  4. L. T. Weber
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Corresponding author

Correspondence to M. R. Benz.

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Interessenkonflikt

M.R. Benz, R. Ehren, B. Tönshoff und L.T. Weber geben an, dass kein Interessenkonflikt besteht.

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Redaktion

D. Haffner, Hannover

Alle Autoren sind Teil des Projektmanagements oder Principal Investigator der im Beitrag enthaltenen Studie Initialtherapie des idiopathischen nephrotischen Syndroms im Kindesalter: Mycophenolatmofetil versus Prednison (INTENT; Förderung durch das Bundesministerium für Bildung und Forschung, BMBF). Die Studie wird nicht eigens für diesen Beitrag durchgeführt.

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Benz, M.R., Ehren, R., Tönshoff, B. et al. Nephrotisches Syndrom im Kindesalter. Monatsschr Kinderheilkd 167, 488–499 (2019). https://doi.org/10.1007/s00112-019-0677-5

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