Clinical and Experimental Nephrology

, Volume 22, Issue 1, pp 126–132 | Cite as

The incidence of the nephrotic syndrome in childhood in Germany

  • Ingo Franke
  • Malik AydinEmail author
  • Corinna Elke Llamas Lopez
  • Lisa Kurylowicz
  • Rainer Ganschow
  • Michael Lentze
  • Mark Born
Original article



The incidence of childhood nephrotic syndrome (NS) in Germany is not well known.


An ESPED-based nationwide collection of epidemiological data of children in 2005 and 2006.


The mean age of NS at onset was 5.5 ± 3.7 years. The gender ratio of boys to girls was 1.8:1. The average length of stay was 15.5 ± 11.2 days, with younger children remaining significantly longer in hospital. Steroid-resistance was more common in children ≥8 years (p = 0.023). Focal-segmental glomerulosclerosis (FSGS) was more common in children >10 years (p = 0.029). The ratio of males to females with FSGS was 1:1.9, thus the FSGS risk for girls at onset was 3.3-times greater. Considering the available data, the incidence of NS in Germany is 1.2/100,000 in the population <18 years, of which 1.0/100,000 are steroid-sensitive.


Compared with international data, which primarily focused on regional and small populations, this is the largest study about the incidence of the childhood NS.


Nephrotic syndrome Childhood Epidemiology Incidence ESPED 



Average length of stay


Arbeitsgemeinschaft für Pädiatrische Nephrologie (Society of Paediatric nephrology)


Acute renal failure


Congenital nephrotic syndrome


Deutsche Gesellschaft für Kinder- und Jugendmedizin


Diffuse mesangial sclerosis


Erhebungseinheit für Seltene Pädiatrische Erkrankungen in Deutschland


Focal-segmental glomerulosclerosis


Human imunnodeficiency virus


Henoch-Schönlein purpura


Lower respiratory tract infection




Mesangioproliferative glomerulonephritis


Membranoproliferative glomerulonephritis


Nephrotic syndrome


Primary nephrotic syndrome


Secondary nephrotic syndrome


Steroid-resistant nephrotic syndrome


Steroid-sensitive nephrotic syndrome



The questionnaire was developed in consultation with the members of the Arbeitsgemeinschaft für Pädiatrische Nephrologie (APN) study committee and the survey was inaugurated as an APN-supported study. This study was funded by Bonner Elternverein für nierenkranke Kinder. We thank the Erhebungseinheit für Seltene Pädiatrische Erkrankungen in Deutschland (ESPED) for their exceptional cooperation and collaboration.

Compliance with ethical standards

Conflict of interest

The authors have declared that no conflicts of interest exist.

Human and animal rights

All procedures performed in studies involving human participants were in accordance with the ethical standards of the Faculty of Medicine, Rheinische Friedrich-Wilhelms-University, Bonn/Germany at which the studies were conducted (IRB Approval Number 081/05) and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

Informed consent

Informed consent was obtained from all individual participants included in the study.


  1. 1.
    Eddy AA, Symons JM. Nephrotic syndrome in childhood. Lancet. 2003;362(629–639):16.Google Scholar
  2. 2.
    Hussain N, Zello JA, Vasilevska-Ritovska J, Banh TM, Patel VP, Patel P, et al. The rationale and design of insight into nephrotic syndrome: investigating genes, health and therapeutics (INSIGHT): a prospective cohort study of childhood nephrotic syndrome. BMC Nephrol. 2013;14:25.CrossRefPubMedPubMedCentralGoogle Scholar
  3. 3.
    Sorof JM, Hawkins EP, Brewer ED, Boydstun II, Kale RS, Powell DR. Age and ethnicity affect the risk and outcome of focal segmental glomerulosclerosis. Pediatr Nephrol. 1998;12:764–8.CrossRefPubMedGoogle Scholar
  4. 4.
    Egli F, Elmiger P, Stalder G. Thrombosis as a complication of nephrotic syndrome. Helv Paediatr Acta. 1973;30(Suppl):20–1.Google Scholar
  5. 5. Last download 8 Dec 2016.
  6. 6.
  7. 7.
    Statistisches Bundesamt Deutschland (2008);jsessionid=2208252EE46F77104F992F3563BA185A.tc21. Zugriffsdatum 17.08.2008 (Tabelle: Bevölkerung: Deutschland, Stichtag, Altersjahre) -,vollanzeige.csp&ID=1018555. Date: 17.08.2008 (Leben in Deutschland- Ergebnisse des Mikrozensus 2005- Pressebroschüre),vollanzeige.csp&ID=1020313. Date of access: 17.08.2008 (Bevölkerung mit Migrationshintergrund-Ergebnisse des. Mikrozensus 2005-Fachserie 1 Reihe 2.2—2005).
  8. 8.
    Chesney RW. The idiopathic nephrotic syndrome. Curr Opin Pediatr. 1999;11:158–64.CrossRefPubMedGoogle Scholar
  9. 9.
    Sharples PM, Poulton J, White RHR. Steroid responsive nephrotic syndrome is more common in Asians. Arch Dis Child. 1985;60:1014–7.CrossRefPubMedPubMedCentralGoogle Scholar
  10. 10.
    Rothenberg MB, Heymann W. The incidence of the nephrotic syndrome in children. Pediatrics. 1957;19:446–52.PubMedGoogle Scholar
  11. 11.
    El Bakkali L, Pereira RB, Kuik DJ, Ket JCF, Wijk JA. Nephrotic syndrome in The Netherlands: a population-based cohort study and a review of the literature. Pediatr Nephrol. 2011;26:1241–6.CrossRefPubMedPubMedCentralGoogle Scholar
  12. 12.
    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 Pediatr Child Health. 2007;43:337–41.CrossRefGoogle Scholar
  13. 13.
    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.CrossRefPubMedGoogle Scholar
  14. 14.
    Kikunaga K, Ishikura K, Terano C, Sato M, Komaki F, Hamasaki Y et al. High incidence of idiopathic nephrotic syndrome in East Asian children: a nationwide survey in Japan (JP-SHINE study). Clin Exp Nephrol 2016. doi  10.1007/s1057-016-1319-z (epub ahead of print).
  15. 15.
    Chang JW, Tsai HL, Yang LY, Chen TJ. Epidemiology and predictors of end-stage renal disease in Taiwanese children with idiopathic nephrotic syndrome. J Epidemiol. 2012;22(6):517–22.CrossRefPubMedPubMedCentralGoogle Scholar
  16. 16.
    Simpson AK, Wong W, Morris MC. Pediatric nephrotic syndrome in Auckland, New Zealand. J Peadiatr Child Health. 1998;34:360–2.CrossRefGoogle Scholar
  17. 17.
    Bircan Z, Yilmaz AY, Katar S, Vitrinel A, Yildrim M. Childhood idiopathic nephrotic syndrome in Turkey. Pediatr Int. 2002;44:608–11.CrossRefPubMedGoogle Scholar
  18. 18.
    Özkaya N, Çakar N, Ekim M, Kara N, Akkök N, Yalçinkaya F. Primary nephrotic syndrome during childhood in Turkey. Pediatr Int. 2004;46:436–8.CrossRefPubMedGoogle Scholar
  19. 19.
    Landau D, Oved T, Geiger D, Abizov L, Shalev H, Parvari R. Familial steroid sensitive nephrotic syndrome in Southern Israel: clinical and genetic observations. Pediatr Nephrol. 2007;22:661–9.CrossRefPubMedGoogle Scholar
  20. 20.
    Srivastava T, Simon SD, Alon US. High incidence of focal segmental glomerulosclerosis in nephrotic syndrome of childhood. Pediatr Nephrol. 1999;13:13–8.CrossRefPubMedGoogle Scholar
  21. 21.
    Kim JS, Bellew CA, Silverstein DM, Aviles DH, Boineau FG, Vehaskari VM. High incidence of initial and late steroid resistance of initial and late steroid resistance in childhood nephrotic syndrome. Kidney Int. 2005;68:1275–81.CrossRefPubMedGoogle Scholar
  22. 22.
    Koskimies O, Vilska J, Rapola J, Halman N. Long-term outcome of primary nephrotic syndrome. Arch Dis Child. 1982;57:544–8.CrossRefPubMedPubMedCentralGoogle Scholar
  23. 23.
    McKinney PA, Feltbower RG, Brocklebank JT, Fitzpatrick MM. Time trends and ethnic patterns of childhood nephrotic syndrome in Yorkshire, UK. Pediatr Nephrol. 2001;16:1040–4.CrossRefPubMedGoogle Scholar
  24. 24.
    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;55:1885–90.CrossRefPubMedGoogle Scholar
  25. 25.
    Kari JA. Changing trends of histopathology in childhood nephrotic syndrome in Western Saudi Arabia. Saudi Med J. 2002;23:317–23.PubMedGoogle Scholar
  26. 26.
    Kumar J, Gulati S, Sharma PA, Sharma RK, Gupta RK. Histopathological spectrum of childhood nephrotic syndrome in Indian children. Pediatr Nephrol. 2003;18:657–60.PubMedGoogle Scholar
  27. 27.
    Nammalwar BR, Vijaykumar M, Prahlad N, Dilipkumar VJ. Steroid resistant nephrotic syndrome: is sustained remission attainable. Indian Pediatr. 2006;43:39–43.PubMedGoogle Scholar
  28. 28.
    Thomson PD. Renal problems in black South African children (occasional survey). Pediatr Nephrol. 1997;11:508–12.CrossRefPubMedGoogle Scholar
  29. 29.
    Choi IJ, Joeng HJ, Han DS, Lee JS, Choi KH, Kang SW, Ha SK, Lee HY, Kim PK. An analysis of 4514 cases of renal biopsy in Korea. Yonsei Med J. 2001;42:247–54.CrossRefPubMedGoogle Scholar
  30. 30.
    Roberts L, Balkaran BN, Asgarali Z, Mohammed W, Khan-Hosein J. Nephrotic syndrome in Trinidadian children. West Ind Med J. 1996;45:92–4.Google Scholar
  31. 31.
    Doe JY, Funk M, Mengel M, Doehring E, Ehrich JHH. Nephrotic syndrome in African children: lack of evidence for ‘tropical nephrotic syndrome’. Nephrol Dial Transplant. 2006;21:672–6.CrossRefPubMedGoogle Scholar
  32. 32.
    Fuchshuber A, Gribouval O, Ronner V, Kroiss S, Karle S, Brandis M, et al. Clinical and genetic evaluation of familial steroid-responsive nephrotic syndrome in childhood. J Am Soc Nephrol. 2001;12:374–8.PubMedGoogle Scholar
  33. 33.
    Nammalwar BR, Vijayakumar M, Prahlad N. Experience of renal biopsy in children with nephrotic syndrome. Pediatr Nephrol. 2006;21:286–8.CrossRefPubMedGoogle Scholar
  34. 34.
    Behrmann RE, Kliegmann RM, Jenson HB. Nephrotic syndrome. In: Nelson textbook of pediatrics. 17th edn. Philadelphia: Saunders; 2004:1753–1758.Google Scholar
  35. 35.
    Batinic D, Scukanec-Spoljar M, Milosevic D, Subat-Dezulović M, Saraga M, Delmis J, et al. Clinical and histological characteristics of biopsy proven renal diseases in Croatia. Acta Med Croat. 2007;61:361–4.Google Scholar
  36. 36.
    Coppo R, Amore A, Gianoglio B. Clinical features of Henoch Schonlein Purpura. Ann Med Intern. 1999;150:143–50.Google Scholar
  37. 37.
    De Almeida JL, Campos LM, Paim LB, Leone C, Koch VH, Silva CA. Renal involvement in Henoch-Schonlein purpura: a multivariate analysis of initial prognostic factors. J Pediatr (Rio J). 2007;83:295–366.Google Scholar
  38. 38.
    Nong BR, Huang YF, Chuang CM, Liu CC, Hsieh KS. Fifteen-year experience of children with Henoch-Schonlein purpura in southern Taiwan 1991–2005. J Microbiol Immunol Infect. 2007;40:371–6.PubMedGoogle Scholar
  39. 39.
    Kelsch RC, Sedman AB. Nephrotic syndrome. Pediatr Rev. 1993;14:30–8.CrossRefPubMedGoogle Scholar
  40. 40.
    Schwartz MW, Schwartz GJ, Cornfeld D. A 16-year follow-up study of 163 children with nephrotic syndrome. Pediatrics. 1974;54:547–52.PubMedGoogle Scholar
  41. 41.
    International Study of Kidney Disease in Children. Minimal change nephrotic syndrome in children: deaths during the first 5 to 15 years’ observation. Pediatrics. 1984;73:497–550.Google Scholar
  42. 42.
    Chang JW, Tsai HL, Wang HH, Yang LY. Clinicopatholigical features and prognosis of Chinese children with idiopathic nephrotic syndrome between different age groups. Eur J Pediatr. 2009;168:1189–94.CrossRefPubMedGoogle Scholar
  43. 43.
    Ladapo TA, Esezobor CI, Lesi FE. High steroid sensitivity among children with nephrotic syndrome in Southwestern Nigeria. Int J Nephrol 2014; 2014 (Article ID 350640).Google Scholar

Copyright information

© Japanese Society of Nephrology 2017

Authors and Affiliations

  • Ingo Franke
    • 1
  • Malik Aydin
    • 1
    • 2
    • 4
    Email author
  • Corinna Elke Llamas Lopez
    • 1
  • Lisa Kurylowicz
    • 1
  • Rainer Ganschow
    • 1
  • Michael Lentze
    • 1
  • Mark Born
    • 3
  1. 1.Department of General PaediatricsUniversity Children’s Hospital BonnBonnGermany
  2. 2.Centre of Biomedical Education and Research, Helios University Children’s Hospital WuppertalUniversity of Witten/HerdeckeWittenGermany
  3. 3.Department of Radiology, Paediatric RadiologyUniversity of BonnBonnGermany
  4. 4.Helios University Children’s Hospital WuppertalUniversity of Witten/HerdeckeWuppertalGermany

Personalised recommendations