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Predictors of remission and relapse in idiopathic nephrotic syndrome: a prospective cohort study

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Abstract

Background

Although most children with idiopathic nephrotic syndrome will respond to corticosteroid therapy, 80–90 % suffer one or more relapses.

Methods

Using Cox proportional hazard models, we analyzed predictors of remission and relapse in 1-year follow-up data on children aged below 15 years with new-onset nephrotic syndrome.

Results

Of 129 children, 107 achieved remission with corticosteroid therapy and 86 subsequently relapsed. Boys achieved remission more often than girls (adjusted hazard ratio [AHR] 1.52, 95 % confidence interval (CI) 1.02–2.3). Boys relapsed significantly more frequently than girls (AHR 1.77, 95 % CI 1.11–2.83) and were more likely to have frequently relapsing disease (AHR 3.3, 95 % CI 1.18–9.23). The risk of first relapse increased with the number of days to first remission (AHR 1.02, 95 % CI 1.01–1.04). The risk for a frequently relapsing course increased with a shorter time from remission to first relapse (AHR 0.92, 95 % CI 0.87–0.97).

Conclusions

In idiopathic nephrotic syndrome, boys are more likely to respond initially, more likely to relapse, and to be classified as having frequently relapsing nephrotic syndrome. A decrease in time from remission to first relapse predicts for a frequently relapsing course.

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References

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

    Article  PubMed  Google Scholar 

  2. Koskimies O, Vilska J, Rapola J, Hallman N (1982) Long-term outcome of primary nephrotic syndrome. Arch Dis Child 57:544–548

    Article  PubMed Central  PubMed  CAS  Google Scholar 

  3. Tarshish P, Tobin JN, Bernstein J, Edelmann CM Jr (1997) 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 8:769–776

    PubMed  CAS  Google Scholar 

  4. Vivarelli M, Moscaritolo E, Tsalkidis A, Massella L, Emma F (2010) Time for initial response to steroids is a major prognostic factor in idiopathic nephrotic syndrome. J Pediatr 156:965–971

    Article  PubMed  Google Scholar 

  5. Yap HK, Han EJ, Heng CK, Gong WK (2001) Risk factors for steroid dependency in children with idiopathic nephrotic syndrome. Pediatr Nephrol 16:1049–1052

    Article  PubMed  CAS  Google Scholar 

  6. Abramowicz M, Barnett HL, Edelmann CM Jr, Greifer I, Kobayashi O, Arneil GC, Barron BA, Gordillo-P G, Hallman N, Tiddens HA (1970) Controlled trial of azathioprine in children with nephrotic syndrome. A report for the International Study of Kidney Disease in Children. Lancet 1:959–961

    Article  PubMed  CAS  Google Scholar 

  7. (1979) Alternate-day versus intermittent prednisone in frequently relapsing nephrotic syndrome. A report of “Arbetsgemeinschaft für Pädiatrische Nephrologie”. Lancet 1(8113):401–403

  8. (1982) Effect of cytotoxic drugs in frequently relapsing nephrotic syndrome with and without steroid dependence. A report of the “Arbetsgemeinschaft für Pädiatrische Nephrologie”. N Engl J Med 306(8):451–454

  9. National High Blood Pressure Education Program Working Group on High Blood Pressure in Children and Adolescents (2004) The fourth report on the diagnosis, evaluation, and treatment of high blood pressure in children and adolescents. Pediatrics 114:555–576

    Google Scholar 

  10. Roy LP, Tiller DJ, Jones DL (1984) The range of blood pressures in Australian children. Med J Aust 141:9–12

    PubMed  CAS  Google Scholar 

  11. (2001) Census of population and housing—(SEIFA) Socio-Economic Indexes for Areas. Australian Bureau of Statistics, Canberra

  12. (2001) Census of population and housing—Population by age and sex in Australian states and territories. Australian Bureau of Statistics, Canberra

  13. El Bakkali L, Rodrigues Pereira R, Kuik DJ, Ket JCF, van Wijk JAE (2011) Nephrotic syndrome in The Netherlands: a population-based cohort study and a review of the literature. Pediatr Nephrol 26:1241–1246

    Article  PubMed Central  PubMed  Google Scholar 

  14. Wong W (2007) 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 Paediatr Child Health 43:337–341

    Article  PubMed  Google Scholar 

  15. McKinney PA, Feltbower RG, Brocklebank JT, Fitzpatrick MM (2001) Time trends and ethnic patterns of childhood nephrotic syndrome in Yorkshire, UK. Pediatr Nephrol 16:1040–1044

    Article  PubMed  CAS  Google Scholar 

  16. Nakanishi K, Iijima K, Ishikura K, Hataya H, Sasaki S, Honda M, Yoshikawa N (2013) Two-year outcome of the ISKDC regimen and frequently relapsing risk in children with idiopathic nephrotic syndrome. Clin J Am Soc Nephrol 8:787–796

    Article  CAS  Google Scholar 

  17. (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(4):561–564

  18. Gulati S, Sural S, Sharma RK, Gupta A, Gupta RK (2001) Spectrum of adolescent-onset nephrotic syndrome in Indian children. Pediatr Nephrol 16:1045–1048

    Article  PubMed  CAS  Google Scholar 

  19. Kim JS, Bellew CA, Silverstein DM, Aviles DH, Boineau FG, Vehaskari VM (2005) High incidence of initial and late steroid resistance in childhood nephrotic syndrome. Kidney Int 68:1275–1281

    Article  PubMed  CAS  Google Scholar 

  20. White RH, Glasgow EF, Mills RJ (1970) Clinicopathological study of nephrotic syndrome in childhood. Lancet 1:1353–1359

    Article  PubMed  CAS  Google Scholar 

  21. (1978) Nephrotic syndrome in children: prediction of histopathology from clinical and laboratory characteristics at time of diagnosis. A report of the International Study of Kidney Disease in Children. Kidney Int 13(2):159–165

  22. Takeda A, Matsutani H, Niimura F, Ohgushi H (1996) Risk factors for relapse in childhood nephrotic syndrome. Pediatr Nephrol 10:740–741

    Article  PubMed  CAS  Google Scholar 

  23. Andersen RF, Thrane N, Noergaard K, Rytter L, Jespersen B, Rittig S (2010) Early age at debut is a predictor of steroid-dependent and frequent relapsing nephrotic syndrome. Pediatr Nephrol 25:1299–1304

    Article  PubMed  Google Scholar 

  24. Constantinescu AR, Shah HB, Foote EF, Weiss LS (2000) Predicting first-year relapses in children with nephrotic syndrome. Pediatrics 105:492–495

    Article  PubMed  CAS  Google Scholar 

  25. Kabuki N, Okugawa T, Hayakawa H, Tomizawa S, Kasahara T, Uchiyama M (1998) Influence of age at onset on the outcome of steroid-sensitive nephrotic syndrome. Pediatr Nephrol 12:467–470

    Article  PubMed  CAS  Google Scholar 

  26. Takeda A, Takimoto H, Mizusawa Y, Simoda M (2001) Prediction of subsequent relapse in children with steroid-sensitive nephrotic syndrome. Pediatr Nephrol 16:888–893

    Article  PubMed  CAS  Google Scholar 

  27. Harambat J, Godron A, Ernould S, Rigothier C, Llanas B, Leroy S (2013) Prediction of steroid-sparing agent use in childhood idiopathic nephrotic syndrome. Pediatr Nephrol 28:631–638

    Article  PubMed  Google Scholar 

  28. Lewis MA, Baildom EM, Davis N, Houston IB, Postlethwaite RJ (1989) Nephrotic syndrome: from toddlers to twenties. Lancet 1:255–259

    Article  PubMed  CAS  Google Scholar 

  29. (1982) Early identification of frequent relapsers among children with minimal change nephrotic syndrome. A report of the International Study of Kidney Disease in Children. J Pediatr 101(4):514–518

  30. Letavernier B, Letavernier E, Leroy S, Baudet-Bonneville V, Bensman A, Ulinski T (2008) Prediction of high-degree steroid dependency in pediatric idiopathic nephrotic syndrome. Pediatr Nephrol 23:2221–2226

    Article  PubMed  Google Scholar 

  31. Noer MS (2005) Predictors of relapse in steroid-sensitive nephrotic syndrome. Southeast Asian J Trop Med Public Health 36:1313–1320

    PubMed  Google Scholar 

  32. Trompeter RS, Lloyd BW, Hicks J, White RH, Cameron JS (1985) Long-term outcome for children with minimal-change nephrotic syndrome. Lancet 1:368–370

    Article  PubMed  CAS  Google Scholar 

  33. Fujinaga S, Hirano D, Nishizaki N (2011) Early identification of steroid dependency in Japanese children with steroid-sensitive nephrotic syndrome undergoing short-term initial steroid therapy. Pediatr Nephrol 26:485–486

    Article  PubMed  Google Scholar 

  34. He S, Zurynski YA, Elliott EJ (2009) Evaluation of a national resource to identify and study rare diseases: the Australian Paediatric Surveillance Unit. J Paediatr Child Health 45:498–504

    Article  PubMed  Google Scholar 

  35. (1994) Consensus statement on management and audit potential for steroid responsive nephrotic syndrome. Report of a Workshop by the British Association for Paediatric Nephrology and Research Unit, Royal College of Physicians. Arch Dis Child 70(2):151–157

  36. Hogg RJ, Portman RJ, Milliner D, Lemley KV, Eddy A, Ingelfinger J (2000) Evaluation and management of proteinuria and nephrotic syndrome in children: recommendations from a pediatric nephrology panel established at the National Kidney Foundation conference on proteinuria, albuminuria, risk, assessment, detection, and elimination (PARADE). Pediatrics 105:1242–1249

    Article  PubMed  CAS  Google Scholar 

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

    Article  PubMed  Google Scholar 

  38. MacHardy N, Miles PV, Massengill SF, Smoyer WE, Mahan JD, Greenbaum L, Massie S, Yao L, Nagaraj S, Lin J-J, Wigfall D, Trachtman H, Hu Y, Gipson DS (2009) Management patterns of childhood-onset nephrotic syndrome. Pediatr Nephrol 24:2193–2201

    Article  PubMed  Google Scholar 

  39. Samuel S, Morgan CJ, Bitzan N, Mammen C, Dart AB, Manns BJ, Alexander RT, Erickson RL, Grisaru S, Wade AW, Blydt-Hansen T, Feber J, Arora S, Licht C, Zappitelli M (2013) Substantial practice variation exists in the management of childhood nephrotic syndrome. Pediatr Nephrol 29:2289–2298

    Article  Google Scholar 

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Acknowledgments

This study was performed using case ascertainment through the Australian Paediatric Surveillance Unit (APSU). The activities of the APSU are supported by the National Health and Medical Research Council of Australia (Enabling Grant no. 402784 and Practitioner Fellowship No 1021480: E. Elliott); the Australian Government Department of Health and Ageing; the Sydney Medical School, The University of Sydney and the Royal Australasian College of Physicians. The authors would like to thank all Australian pediatricians who participated in APSU surveillance, reported cases, and completed questionnaires on their patients. The study was funded in part by Kidney Health Australia. Kidney Health Australia had no involvement in the design, data collection, data analysis or writing of the manuscript.

Parts of this study were presented at the 13th Congress of the International Pediatric Nephrology Association, August 29 to September 2 2004, Adelaide, Australia, and were published in abstract form.

The authors would like to thank Mrs. Sandra Puckeridge and Dr. Jeffrey Fletcher for their assistance with data collection and for the preliminary analyses of the data. The authors would also like to thank the Australian members of the Australia and New Zealand Paediatric Nephrology Association, who reviewed the protocol and questionnaires for this study.

Disclosures

None.

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Authors and Affiliations

  1. Centre for Kidney Research, The Children’s Hospital at Westmead, Locked Bag 4001, Westmead, Sydney, NSW 2145, Australia

    Premala Sureshkumar, Elisabeth M. Hodson, Narelle S. Willis & Jonathan C. Craig

  2. Department of Psychology, Macquarie University, Sydney, Australia

    Premala Sureshkumar

  3. Sydney School of Public Health, University of Sydney, Sydney, Australia

    Elisabeth M. Hodson, Narelle S. Willis & Jonathan C. Craig

  4. The George Institute for Global Health, Sydney, Australia

    Federica Barzi

Authors
  1. Premala Sureshkumar
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  2. Elisabeth M. Hodson
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  3. Narelle S. Willis
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  4. Federica Barzi
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  5. Jonathan C. Craig
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Correspondence to Elisabeth M. Hodson.

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Sureshkumar, P., Hodson, E.M., Willis, N.S. et al. Predictors of remission and relapse in idiopathic nephrotic syndrome: a prospective cohort study. Pediatr Nephrol 29, 1039–1046 (2014). https://doi.org/10.1007/s00467-013-2736-9

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