Pediatric Nephrology

, Volume 28, Issue 12, pp 2289–2298 | Cite as

Substantial practice variation exists in the management of childhood nephrotic syndrome

  • Susan Samuel
  • Catherine J. Morgan
  • Martin Bitzan
  • Cherry Mammen
  • Allison B. Dart
  • Braden J. Manns
  • R. Todd Alexander
  • Robin L. Erickson
  • Silviu Grisaru
  • Andrew W. Wade
  • Tom Blydt-Hansen
  • Janusz Feber
  • Steven Arora
  • Christoph Licht
  • Michael ZappitelliEmail author
Original Article



Practice variation is common for nephrotic syndrome (NS) treatment.


A cross-sectional, web-based survey on NS treatment was administered to 58 Canadian pediatric nephrologists with the aim to document existing practice variation and compare practice with the recommendations of the Kidney Disease Improving Global Outcomes Clinical Practice Guideline for NS.


Of the 58 nephrologists asked to participate in the survey, 40 (69 %) responded. Among these, 62 % prescribed initial daily glucocorticoid (GC) therapy for 6 weeks, 26 % for 4 weeks by 26 %, and 10 % prescribed ‘other’. Alternate-day GC was continued for 6 weeks by 63 % of respondents and for >6 and <6 weeks by 32 and 6 %, respectively. For biopsy-confirmed minimal change disease, 65 and 46 % of respondents chose oral cyclophosphamide for frequently relapsing and steroid-dependent phenotypes, respectively; calcineurin inhibitors or mycophenolate were the second most popular choices. Kidney biopsy was ‘always’ performed by 16, 39, and 97 % of respondents for frequently relapsing, steroid-dependent, and steroid-resistant patients, respectively. Rituximab had been administered by 60 % of respondents; 22, 56, and 72 % reported that they would consider rituximab for frequently relapsing, steroid-dependent, and steroid-resistant patients, respectively. Most notable differences between practice and Guideline recommendations were first presentation GC duration, GC-sparing agent choices in frequently relapsing and steroid-dependent patients, and biopsy practices.


There is substantial Canadian practice variation in NS treatment. Assessment of factors driving variation and strategies to implement Guideline recommendations are needed.


Corticosteroids Calcineurin inhibitors Knowledge translation Treatment Minimal change disease Relapse Kidney biopsy 



We sincerely thank all the Canadian pediatric nephrologists who completed the online survey regarding their management practices in childhood nephrotic syndrome. We gratefully acknowledge the support provided by the Canadian Kidney Knowledge Translation and Generation Network and the Canadian Association of Pediatric Nephrologists in the conduct and completion of this study.

Disclosures (funding)

SS received salary award support from the Kidney Research Scientist Core Education and National Training Program (Kidney Foundation of Canada, Canadian Institutes for Health Research, Canadian Society of Nephrology) and Canadian Child Health Clinician Scientist Program. MZ received salary support from the Fonds de Recherche du Québec–Santé.


  1. 1.
    Gipson DS, Massengill SF, Yao L, Nagaraj S, Smoyer WE, Mahan JD, Wigfall D, Miles P, Powell L, Lin JJ, Trachtman H, Greenbaum LA (2009) Management of childhood onset nephrotic syndrome. Pediatrics 124:747–757PubMedCrossRefGoogle Scholar
  2. 2.
    Churg J, Habib R, White RH (1970) Pathology of the nephrotic syndrome in children: a report for the International Study of Kidney Disease in Children. Lancet 760:1299–1302PubMedCrossRefGoogle Scholar
  3. 3.
    Churg J, Habib R, White RH (1979) Alternate-day versus intermittent prednisone in frequently relapsing nephrotic syndrome. A report of "Arbetsgemeinschaft fur Padiatrische Nephrologie". Lancet 1:401–403Google Scholar
  4. 4.
    Ehrich JH, Brodehl J (1993) Long versus standard prednisone therapy for initial treatment of idiopathic nephrotic syndrome in children. Arbeitsgemeinschaft fur Padiatrische Nephrologie. Eur J Pediatr 152:357–361PubMedCrossRefGoogle Scholar
  5. 5.
    Lande MB, Leonard MB (2000) Variability among pediatric nephrologists in the initial therapy of nephrotic syndrome. Pediatr Nephrol 14:766–769PubMedCrossRefGoogle Scholar
  6. 6.
    MacHardy N, Miles PV, Massengill SF, Smoyer WE, Mahan JD, Greenbaum L, Massie S, Yao L, Nagaraj S, Lin JJ, Wigfall D, Trachtman H, Hu Y, Gipson DS (2009) Management patterns of childhood-onset nephrotic syndrome. Pediatr Nephrol 24:2193–2201PubMedCrossRefGoogle Scholar
  7. 7.
    Hodson EM, Willis NS, Craig JC (2008) Non-corticosteroid treatment for nephrotic syndrome in children. Cochrane Database Syst Rev (1):CD002290. doi: 10.1002/14651858.CD002290.pub3
  8. 8.
    Haute Autorité de Santé (2008) Syndrome néphrotique idiopathique de l’enfant. Protocol national de diagnostic et de soins pour une maladie rare. Haute Autorité de Santé, Saint-Denis La Plaine, pp 1–22. Available at:
  9. 9.
    Bagga A, Ali U, Banerjee S, Kanitkar M, Phadke KD, Senguttuvan P, Sethi S, Shah M (2008) Management of steroid sensitive nephrotic syndrome: revised guidelines. Indian Pediatr 45:203–214PubMedGoogle Scholar
  10. 10.
    Kidney Disease Improving Global Outcomes (KDIGO) Glomerulonephritis Work Group (2012) KDIGO clinical practice guideline for glomerulonephritis. Kidney Int Suppl 2:139–274CrossRefGoogle Scholar
  11. 11.
    Graham ID, Logan J, Harrison MB, Straus SE, Tetroe J, Caswell W, Robinson N (2006) Lost in knowledge translation: time for a map? J Contin Educ Health Prof 26:13–24PubMedCrossRefGoogle Scholar
  12. 12.
    Grol R (2001) Improving the quality of medical care: building bridges among professional pride, payer profit, and patient satisfaction. JAMA 286:2578–2585PubMedCrossRefGoogle Scholar
  13. 13.
    McGlynn EA, Asch SM, Adams J, Keesey J, Hicks J, DeCristofaro A, Kerr EA (2003) The quality of health care delivered to adults in the United States. N Engl J Med 348:2635–2645PubMedCrossRefGoogle Scholar
  14. 14.
    Straus SE, Tetroe J, Graham I (2009) Defining knowledge translation. CMAJ 181:165–168PubMedCrossRefGoogle Scholar
  15. 15.
    von Elm E, Altman DG, Egger M, Pocock SJ, Gotzsche PC, Vandenbroucke JP (2007) The Strengthening the Reporting of Observational Studies in Epidemiology (STROBE) statement: guidelines for reporting observational studies. Ann Intern Med 147:573–577CrossRefGoogle Scholar
  16. 16.
    Hodson EM, Willis NS, Craig JC (2007) Corticosteroid therapy for nephrotic syndrome in children. Cochrane Database Syst Rev (1):CD001533. doi: 10.1002/14651858.CD002290.pub3.
  17. 17.
    Hirano D, Nishizaki N, Kanai H, Hara S, Ohtomo Y, Umino D, Fujinaga S (2010) Long-term outcome of children treated with the ISKDC regimen for the first episode of INS. Nihon Jinzo Gakkai Shi 52:1029–1036PubMedGoogle Scholar
  18. 18.
    Hussain F, Mallik M, Marks SD, Watson AR (2010) Renal biopsies in children: current practice and audit of outcomes. Nephrol Dial Transplant 25:485–489PubMedCrossRefGoogle Scholar
  19. 19.
    Magnasco A, Ravani P, Edefonti A, Murer L, Ghio L, Belingheri M, Benetti E, Murtas C, Messina G, Massella L, Porcellini MG, Montagna M, Regazzi M, Scolari F, Ghiggeri GM (2012) Rituximab in children with resistant idiopathic nephrotic syndrome. J Am Soc Nephrol 23:1117–1124PubMedCrossRefGoogle Scholar
  20. 20.
    Eddy AA, Symons JM (2003) Nephrotic syndrome in childhood. Lancet 362:629–639PubMedCrossRefGoogle Scholar
  21. 21.
    Greenbaum LA, Benndorf R, Smoyer WE (2012) Childhood nephrotic syndrome–current and future therapies. Nat Rev Nephrol 8:445–458PubMedCrossRefGoogle Scholar
  22. 22.
    Cabana MD, Rand CS, Powe NR, Wu AW, Wilson MH, Abboud PA, Rubin HR (1999) Why don’t physicians follow clinical practice guidelines? A framework for improvement. JAMA 282:1458–1465PubMedCrossRefGoogle Scholar
  23. 23.
    Grimshaw JM, Shirran L, Thomas R, Mowatt G, Fraser C, Bero L, Grilli R, Harvey E, Oxman A, O’Brien MA (2001) Changing provider behavior: an overview of systematic reviews of interventions. Med Care 39:II2–45PubMedCrossRefGoogle Scholar
  24. 24.
    Campbell H, Hotchkiss R, Bradshaw N, Porteous M (1998) Integrated care pathways. BMJ 316:133–137PubMedCrossRefGoogle Scholar
  25. 25.
    Panella M, Marchisio S, Di Stanislao F (2003) Reducing clinical variations with clinical pathways: do pathways work? Int J Qual Health Care 15:509–521PubMedCrossRefGoogle Scholar
  26. 26.
    Rotter T, Kinsman L, James E, Machotta A, Gothe H, Willis J, Snow P, Kugler J (2010) Clinical pathways: effects on professional practice, patient outcomes, length of stay and hospital costs. Cochrane Database Syst Rev (3):CD006632. doi: 10.1002/14651858.CD006632.pub2

Copyright information

© IPNA 2013

Authors and Affiliations

  • Susan Samuel
    • 1
  • Catherine J. Morgan
    • 2
  • Martin Bitzan
    • 3
  • Cherry Mammen
    • 4
  • Allison B. Dart
    • 5
  • Braden J. Manns
    • 6
  • R. Todd Alexander
    • 2
  • Robin L. Erickson
    • 7
  • Silviu Grisaru
    • 1
  • Andrew W. Wade
    • 1
  • Tom Blydt-Hansen
    • 5
  • Janusz Feber
    • 8
  • Steven Arora
    • 9
  • Christoph Licht
    • 10
  • Michael Zappitelli
    • 3
    Email author
  1. 1.Department of Pediatrics, Division of NephrologyUniversity of Calgary and Alberta Children’s Hospital Research Institute for Child and Maternal HealthCalgaryCanada
  2. 2.Department of PediatricsUniversity of Alberta–Stollery Children’s HospitalEdmontonCanada
  3. 3.Department of Pediatrics, Division of NephrologyMcGill University Health Centre–Montreal Children’s HospitalMontrealCanada
  4. 4.Department of Pediatrics, Division of Pediatric NephrologyBritish Columbia Children’s HospitalVancouverCanada
  5. 5.Department of Pediatrics and Child Health, Section of NephrologyUniversity of ManitobaWinnipegCanada
  6. 6.Department of Medicine and Community Health SciencesUniversity of CalgaryCalgaryCanada
  7. 7.Department of Pediatrics, Division of Pediatric NephrologyUniversity of SaskatchewanSaskatoonCanada
  8. 8.Department of Pediatrics, Division of NephrologyUniversity of Ottawa–Children’s Hospital of Eastern OntarioOttawaCanada
  9. 9.Department of Pediatrics, Division of NephrologyMcMaster University–McMaster Children’s HospitalHamiltonCanada
  10. 10.Department of Pediatrics, Division of NephrologySickkids HospitalTorontoCanada

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