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Clinical course of 110 children and adolescents with primary focal segmental glomerulosclerosis

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Abstract

The purpose of this retrospective cohort study was to report the clinical course of children and adolescents with primary focal segmental glomerulosclerosis (FSGS). The records of 110 patients with biopsy-proven FSGS admitted between 1972 and 2004 were retrospectively reviewed. Demographic, clinical and laboratory data were recorded and histopathological data were reanalyzed by one pathologist who had no information about the outcome of the patients. Renal survival analysis was performed using the Kaplan-Meier method. Differences between subgroups (response to corticosteroids) were assessed by the two-sided log rank test. The median age at admission was 5 years (range: 1–15 years). Forty-two patients (38.2%) presented with hematuria at admission, and 55 (50%) presented blood pressure levels above the 95th percentile. Mean follow-up time was 10 years (SD 5.5). Twenty-four patients (21.8%) presented chronic kidney disease (CKD). It was estimated that the probability of CKD was 8% at 5 years, 17% at 10 years, and 32% at 15 years after diagnosis of nephrotic syndrome. In conclusion, on the basis of the clinical and histological characteristics observed, apparently our cohort of idiopathic FSGS is comparable with other published series. However, the long-term overall renal survival seems to be better in our cohort.

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References

  1. Korbet SM (1998) Primary focal segmental glomerulosclerosis. J Am Soc Nephrol 9:1333–1340

    PubMed  CAS  Google Scholar 

  2. Schnaper HW (2003) Idiopathic focal segmental glomerulosclerosis. Semin Nephrol 23:183–193

    Article  PubMed  Google Scholar 

  3. International Study of Kidney Disease in Children (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 13:159–165

    Article  Google Scholar 

  4. Southwest Pediatric Nephrology Study Group (1985) Focal segmental glomerulosclerosis in children with idiopathic nephrotic syndrome. A report of the Southwest Pediatric Nephrology Study Group. Kidney Int 27:442–449

    Article  Google Scholar 

  5. Srivastava T, Simon SD, Alon US (1999) High incidence of focal segmental glomerulosclerosis in nephrotic syndrome of childhood. Pediatr Nephrol 13:13–18

    Article  PubMed  CAS  Google Scholar 

  6. Ichikawa I, Fogo A (1996) Focal segmental glomerulosclerosis. Pediatr Nephrol 10:374–391

    PubMed  CAS  Google Scholar 

  7. Sorof JM, Hawkins EP, Brewer ED, Boydstun, II, Kale AS, Powell DR (1998) Age and ethnicity affect the risk and outcome of focal segmental glomerulosclerosis. Pediatr Nephrol 12:764–768

    Article  PubMed  CAS  Google Scholar 

  8. Task Force on Blood Pressure Control in Children (1987) Report of the second task force on blood pressure control in children. Pediatrics 79:1–25

    Google Scholar 

  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

    Article  Google Scholar 

  10. Mendoza SA, Reznik VM, Griswold WR, Krensky AM, Yorgin PD, Tune BM (1990) Treatment of steroid-resistant focal segmental glomerulosclerosis with pulse methylprednisolone and alkylating agents. Pediatr Nephrol 4:303–307

    Article  PubMed  CAS  Google Scholar 

  11. International Study of Kidney Disease in Children (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 

  12. 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 760:1299–1302

    Article  PubMed  CAS  Google Scholar 

  13. Gulati S, Elhence R, Kher V, Sharma RK, Jain M, Gupta A, Gupta RK (2000) Early versus late-onset idiopathic focal segmental glomerulosclerosis. Pediatr Nephrol 14:960–964

    Article  PubMed  CAS  Google Scholar 

  14. Schwartz GJ, Brion LP, Spitzer A (1987) The use of plasma creatinine concentration for estimating glomerular filtration rate in infants, children, and adolescents. Pediatr Clin North Am 34:571–590

    PubMed  CAS  Google Scholar 

  15. Cole TJ, Bellizzi MC, Flegal KM, Dietz WH (2000) Establishing a standard definition for child overweight and obesity worldwide: international survey. BMJ 320:1240–1243

    Article  PubMed  CAS  Google Scholar 

  16. D’Agati V (1994) The many masks of focal segmental glomerulosclerosis. Kidney Int 46:1223–1241

    Article  PubMed  CAS  Google Scholar 

  17. Haas M, Spargo BH, Coventry S (1995) Increasing incidence of focal-segmental glomerulosclerosis among adult nephropathies: a 20-year renal biopsy study. Am J Kidney Dis 26:740–750

    PubMed  CAS  Google Scholar 

  18. Bonilla-Felix M, Parra C, Dajani T, Ferris M, Swinford RD, Portman RJ, Verani R (1999) Changing patterns in the histopathology of idiopathic nephrotic syndrome in children. Kidney Int 55:1885–1890

    Article  PubMed  CAS  Google Scholar 

  19. Ellis D, Kapur S, Antonovych TT, Salcedo JR, Yunis EJ (1978) Focal glomerulosclerosis in children: correlation of histology with prognosis. J Pediatr 93:762–768

    Article  PubMed  CAS  Google Scholar 

  20. Cameron JS, Turner DR, Ogg CS, Chantler C, Williams DG (1978) The long-term prognosis of patients with focal segmental glomerulosclerosis. Clin Nephrol 10:213–218

    PubMed  CAS  Google Scholar 

  21. Arbus GS, Poucell S, Bacheyie GS, Baumal R (1982) Focal segmental glomerulosclerosis with idiopathic nephrotic syndrome: three types of clinical response. J Pediatr 101:40–45

    Article  PubMed  CAS  Google Scholar 

  22. Yoshikawa N, Ito H, Akamatsu R, Matsuyama S, Hasegawa O, Nakahara C, Matsuo T (1986) Focal segmental glomerulosclerosis with and without nephrotic syndrome in children. J Pediatr 109:65–70

    Article  PubMed  CAS  Google Scholar 

  23. Tufro-McReddie A, Alvarez E, Arrizurieta E, Repetto H (1992) Focal glomerulosclerosis in children: an Argentinian experience. Pediatr Nephrol 6:158–161

    Article  PubMed  CAS  Google Scholar 

  24. Mongeau JG, Robitaille PO, Clermont MJ, Merouani A, Russo P (1993) Focal segmental glomerulosclerosis (FSG) 20 years later. From toddler to grown up. Clin Nephrol 40:1–6

    PubMed  CAS  Google Scholar 

  25. Cattran DC, Rao P (1998) Long-term outcome in children and adults with classic focal segmental glomerulosclerosis. Am J Kidney Dis 32:72–79

    PubMed  CAS  Google Scholar 

  26. Martinelli R, Okumura AS, Pereira LJ, Rocha H (2001) Primary focal segmental glomerulosclerosis in children: prognostic factors. Pediatr Nephrol 16:658–661

    Article  PubMed  CAS  Google Scholar 

  27. Tejani A, Nicastri AD, Sen D, Chen CK, Phadke K, Adamson O, Butt KM (1983) Long-term evaluation of children with nephrotic syndrome and focal segmental glomerular sclerosis. Nephron 35:225–231

    PubMed  CAS  Google Scholar 

  28. Korbet SM, Schwartz MM, Lewis EJ (1994) Primary focal segmental glomerulosclerosis: clinical course and response to therapy. Am J Kidney Dis 23:773–783

    PubMed  CAS  Google Scholar 

  29. Mongeau JG, Corneille L, Robitaille P, O’Regan S, Pelletier M (1981) Primary nephrosis in childhood associated with focal glomerular sclerosis: is long-term prognosis that severe? Kidney Int 20:743–746

    Article  PubMed  CAS  Google Scholar 

  30. Ingulli E, Tejani A (1991) Racial differences in the incidence and renal outcome of idiopathic focal segmental glomerulosclerosis in children. Pediatr Nephrol 5:393–397

    Article  PubMed  CAS  Google Scholar 

  31. Parra FC, Amado RC, Lambertucci JR, Rocha J, Antunes CM, Pena SD (2003) Color and genomic ancestry in Brazilians. Proc Natl Acad Sci USA 100:177–182

    Article  PubMed  CAS  Google Scholar 

  32. Alexopoulos E, Stangou M, Papagianni A, Pantzaki A, Papadimitriou M (2000) Factors influencing the course and the response to treatment in primary focal segmental glomerulosclerosis. Nephrol Dial Transplant 15:1348–1356

    Article  PubMed  CAS  Google Scholar 

  33. Chun MJ, Korbet SM, Schwartz MM, Lewis EJ (2004) Focal segmental glomerulosclerosis in nephrotic adults: presentation, prognosis, and response to therapy of the histologic variants. J Am Soc Nephrol 15:2169–2177

    Article  PubMed  Google Scholar 

  34. Valeri A, Barisoni L, Appel GB, Seigle R, D’Agati V (1996) Idiopathic collapsing focal segmental glomerulosclerosis: a clinicopathologic study. Kidney Int 50:1734–1746

    Article  PubMed  CAS  Google Scholar 

  35. Schwartz MM, Evans J, Bain R, Korbet SM (1999) Focal segmental glomerulosclerosis: prognostic implications of the cellular lesion. J Am Soc Nephrol 10:1900–1907

    PubMed  CAS  Google Scholar 

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

    Article  PubMed  Google Scholar 

  37. Donatti TL, Koch VH, Fujimura MD, Okay Y (2003) Growth in steroid-responsive nephrotic syndrome: a study of 85 pediatric patients. Pediatr Nephrol 18:789–795

    Article  PubMed  Google Scholar 

  38. Ponticelli C, Edefonti A, Ghio L, Rizzoni G, Rinaldi S, Gusmano R, Lama G, Zacchello G, Confalonieri R, Altieri P et al (1993) Cyclosporin versus cyclophosphamide for patients with steroid-dependent and frequently relapsing idiopathic nephrotic syndrome: a multicentre randomized controlled trial. Nephrol Dial Transplant 8:1326–1332

    PubMed  CAS  Google Scholar 

  39. Ruf RG, Lichtenberger A, Karle SM, Haas JP, Anacleto FE, Schultheiss M, Zalewski I, Imm A, Ruf EM, Mucha B, Bagga A, Neuhaus T, Fuchshuber A, Bakkaloglu A, Hildebrandt F (2004) Patients with mutations in NPHS2 (podocin) do not respond to standard steroid treatment of nephrotic syndrome. J Am Soc Nephrol 15:722–732

    Article  PubMed  Google Scholar 

Download references

Acknowledgements

This study was partially supported by CNPq (Brazilian National Research Council), Pró-Reitoria de Pesquisa (UFMG), and FAPEMIG.

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Correspondence to Eduardo A. Oliveira.

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Abrantes, M.M., Cardoso, L.S.B., Lima, E.M. et al. Clinical course of 110 children and adolescents with primary focal segmental glomerulosclerosis. Pediatr Nephrol 21, 482–489 (2006). https://doi.org/10.1007/s00467-006-0019-4

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  • DOI: https://doi.org/10.1007/s00467-006-0019-4

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