Skip to main content
Erschienen in: Monatsschrift Kinderheilkunde 6/2019

26.03.2019 | Ödeme | Leitthema

Nephrotisches Syndrom im Kindesalter

verfasst von: Dr. M. R. Benz, R. Ehren, B. Tönshoff, L. T. Weber

Erschienen in: Monatsschrift Kinderheilkunde | Ausgabe 6/2019

Einloggen, um Zugang zu erhalten

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.
Literatur
1.
Zurück zum Zitat 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 93:226–228CrossRef 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 93:226–228CrossRef
2.
Zurück zum Zitat 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 98:561–564CrossRef 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 98:561–564CrossRef
4.
Zurück zum Zitat Benz M (2011) Pipilotta und der Nierendetektiv, Familienschulung des nephrotischen Syndroms. Monatsschr Kinderheilkd 159:172 Benz M (2011) Pipilotta und der Nierendetektiv, Familienschulung des nephrotischen Syndroms. Monatsschr Kinderheilkd 159:172
5.
Zurück zum Zitat Benz Mr KM (2015) Nephrotisches Syndrom im Kindesalter. Monatsschr Kinderheilkd 163:310–322CrossRef Benz Mr KM (2015) Nephrotisches Syndrom im Kindesalter. Monatsschr Kinderheilkd 163:310–322CrossRef
6.
Zurück zum Zitat Benz M, Weber L (2012) Nephrotisches Syndrom im Kindesalter. Monatsschr Kinderheilkd 160(8):787–804CrossRef Benz M, Weber L (2012) Nephrotisches Syndrom im Kindesalter. Monatsschr Kinderheilkd 160(8):787–804CrossRef
7.
Zurück zum Zitat Benz MR, Tönshoff B, Weber LT (2014) Treatment of children with frequent relapsing steroid-sensitive nephrotic syndrome: recent trial results. Clin Investig 4(11):1043–1054CrossRef Benz MR, Tönshoff B, Weber LT (2014) Treatment of children with frequent relapsing steroid-sensitive nephrotic syndrome: recent trial results. Clin Investig 4(11):1043–1054CrossRef
8.
Zurück zum Zitat Brodehl J (1991) The treatment of minimal change nephrotic syndrome: lessons learned from multicentre co-operative studies. Eur J Pediatr 150:380–387CrossRef Brodehl J (1991) The treatment of minimal change nephrotic syndrome: lessons learned from multicentre co-operative studies. Eur J Pediatr 150:380–387CrossRef
9.
Zurück zum Zitat 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 26:178–184CrossRef 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 26:178–184CrossRef
10.
Zurück zum Zitat 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 53:760–769CrossRef 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 53:760–769CrossRef
11.
Zurück zum Zitat 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 29:2000–2013CrossRef 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 29:2000–2013CrossRef
12.
Zurück zum Zitat Eddy AA, Symons JM (2003) Nephrotic syndrome in childhood. Lancet 362:629–639CrossRef Eddy AA, Symons JM (2003) Nephrotic syndrome in childhood. Lancet 362:629–639CrossRef
13.
Zurück zum Zitat 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:e24882CrossRef 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:e24882CrossRef
14.
Zurück zum Zitat 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 28:2235–2238CrossRef 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 28:2235–2238CrossRef
15.
Zurück zum Zitat 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 26:1701–1710CrossRef 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 26:1701–1710CrossRef
16.
Zurück zum Zitat Gee HY, Ashraf S, Wan X et al (2014) Mutations in EMP2 cause childhood-onset nephrotic syndrome. Am J Hum Genet 94:884–890CrossRef Gee HY, Ashraf S, Wan X et al (2014) Mutations in EMP2 cause childhood-onset nephrotic syndrome. Am J Hum Genet 94:884–890CrossRef
17.
Zurück zum Zitat 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 27:1970–1978CrossRef 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 27:1970–1978CrossRef
18.
Zurück zum Zitat Gipson DS, Massengill SF, Yao L et al (2009) Management of childhood onset nephrotic syndrome. Pediatr Electron Pages 124:747–757 Gipson DS, Massengill SF, Yao L et al (2009) Management of childhood onset nephrotic syndrome. Pediatr Electron Pages 124:747–757
19.
Zurück zum Zitat 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 6:63–69CrossRef 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 6:63–69CrossRef
20.
Zurück zum Zitat 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 38:274–279CrossRef 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 38:274–279CrossRef
22.
Zurück zum Zitat Hoyer PF (2015) New lessons from randomized trials in steroid-sensitive nephrotic syndrome: clear evidence against long steroid therapy. Kidney Int 87:17–19CrossRef Hoyer PF (2015) New lessons from randomized trials in steroid-sensitive nephrotic syndrome: clear evidence against long steroid therapy. Kidney Int 87:17–19CrossRef
23.
Zurück zum Zitat 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 384:1273–1281CrossRef 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 384:1273–1281CrossRef
24.
Zurück zum Zitat 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 73:1167–1173CrossRef 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 73:1167–1173CrossRef
25.
Zurück zum Zitat 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 29:2189–2199CrossRef 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 29:2189–2199CrossRef
26.
Zurück zum Zitat 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 196:217–222e1CrossRef 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 196:217–222e1CrossRef
27.
Zurück zum Zitat Kemper Mj LA, Zawischa A, Oh J (2014) Is rituximab effective in childhood nephrotic syndrome? Yes and no. Pediatr Nephrol 29:1305–1311CrossRef Kemper Mj LA, Zawischa A, Oh J (2014) Is rituximab effective in childhood nephrotic syndrome? Yes and no. Pediatr Nephrol 29:1305–1311CrossRef
28.
Zurück zum Zitat Latta K, Von Schnakenburg C, Ehrich JH (2001) A meta-analysis of cytotoxic treatment for frequently relapsing nephrotic syndrome in children. Pediatr Nephrol 16:271–282CrossRef Latta K, Von Schnakenburg C, Ehrich JH (2001) A meta-analysis of cytotoxic treatment for frequently relapsing nephrotic syndrome in children. Pediatr Nephrol 16:271–282CrossRef
30.
Zurück zum Zitat Lombel RM, Gipson DS, Hodson EM et al (2013) Treatment of steroid-sensitive nephrotic syndrome: new guidelines from KDIGO. Pediatr Nephrol 28:415–426CrossRef Lombel RM, Gipson DS, Hodson EM et al (2013) Treatment of steroid-sensitive nephrotic syndrome: new guidelines from KDIGO. Pediatr Nephrol 28:415–426CrossRef
31.
Zurück zum Zitat Mattoo TK, Mahmoud MA (2000) Increased maintenance corticosteroids during upper respiratory infection decrease the risk of relapse in nephrotic syndrome. Nephron 85:343–345CrossRef Mattoo TK, Mahmoud MA (2000) Increased maintenance corticosteroids during upper respiratory infection decrease the risk of relapse in nephrotic syndrome. Nephron 85:343–345CrossRef
32.
Zurück zum Zitat 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 8:17322CrossRef 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 8:17322CrossRef
33.
Zurück zum Zitat 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:e18148CrossRef 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:e18148CrossRef
34.
Zurück zum Zitat 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 87:217–224CrossRef 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 87:217–224CrossRef
35.
Zurück zum Zitat 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 95:210–218CrossRef 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 95:210–218CrossRef
36.
Zurück zum Zitat 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 77:189–196CrossRef 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 77:189–196CrossRef
37.
Zurück zum Zitat 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 24:149–159CrossRef 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 24:149–159CrossRef
38.
Zurück zum Zitat Vogd J, Hackl A, Habbig S et al (2019) Discontinuation of maintenance therapy in frequently relapsing nephrotic syndrome. Clin Nephrol 91:25–31CrossRef Vogd J, Hackl A, Habbig S et al (2019) Discontinuation of maintenance therapy in frequently relapsing nephrotic syndrome. Clin Nephrol 91:25–31CrossRef
39.
Zurück zum Zitat 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 15:147CrossRef 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 15:147CrossRef
40.
Zurück zum Zitat Weber LT (2015) Therapeutic drug monitoring in pediatric renal transplantation. Pediatr Nephrol 30:253–265CrossRef Weber LT (2015) Therapeutic drug monitoring in pediatric renal transplantation. Pediatr Nephrol 30:253–265CrossRef
41.
Zurück zum Zitat 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 78:212–217CrossRef 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 78:212–217CrossRef
42.
Zurück zum Zitat Wei CC, Lin CL, Shen TC et al (2015) Occurrence of common allergic diseases in children with idiopathic nephrotic syndrome. J Epidemiol 25:370–377CrossRef Wei CC, Lin CL, Shen TC et al (2015) Occurrence of common allergic diseases in children with idiopathic nephrotic syndrome. J Epidemiol 25:370–377CrossRef
43.
Zurück zum Zitat 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 87:225–232CrossRef 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 87:225–232CrossRef
44.
Zurück zum Zitat 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 30:505–51342CrossRef 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 30:505–51342CrossRef
Metadaten
Titel
Nephrotisches Syndrom im Kindesalter
verfasst von
Dr. M. R. Benz
R. Ehren
B. Tönshoff
L. T. Weber
Publikationsdatum
26.03.2019
Verlag
Springer Medizin
Erschienen in
Monatsschrift Kinderheilkunde / Ausgabe 6/2019
Print ISSN: 0026-9298
Elektronische ISSN: 1433-0474
DOI
https://doi.org/10.1007/s00112-019-0677-5

Weitere Artikel der Ausgabe 6/2019

Monatsschrift Kinderheilkunde 6/2019 Zur Ausgabe

Mitteilungen der ÖGKJ

Mitteilungen der ÖGKJ

Pädiatrie aktuell

Für Sie gelesen

Update Pädiatrie

Bestellen Sie unseren Fach-Newsletter und bleiben Sie gut informiert.