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Primary focal segmental glomerular sclerosis in children: clinical course and prognosis

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Abstract

To review the clinical course and identify prognostic factors, we retrospectively analyzed 92 children with steroid-resistant primary focal segmental glomerulosclerosis (FSGS). The mean age of onset was 80.4±42.4 months. The mean follow-up duration was 98.2±63.3 months. Eighty-five patients presented with nephrotic syndrome and seven presented with asymptomatic proteinuria. Thirty-three patients were initial responders to steroid treatment (late non-responders) and 59 were initial nonresponders. At last follow-up, 36 patients (39.1%) were in complete remission, and 29 (31.5%) progressed to chronic renal failure (CRF). Renal survival rates at 5, 10, and 15 years were 84, 64, and 53%, respectively. By morphological classification, there were tip variants (6.1%), collapsing variants (10.6%), cellular variants (1.5%), perihilar variants (9.1%), and NOS (not otherwise specified, 72.7%). Among the variants, there were no significant differences in age of onset, degree of proteinuria, response to treatment, or progression to CRF. Poor prognostic factors for CRF included: asymptomatic proteinuria at presentation, initial renal insufficiency, higher segmental sclerosis (%), severe tubulointerstitial change, initial nonresponse, and absence of remission. In the multivariate analysis, an increase in the initial serum creatinine and resistance to treatment were independent risk factors for CRF. A more prolonged use of corticosteroid therapy and early introduction of cyclosporin A (CsA) may improve the prognosis for primary FSGS in patients with initial steroid nonresponsiveness.

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References

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

    CAS  PubMed  Google Scholar 

  2. Habib R, Kleinknecht C (1971) The primary nephrotic syndrome of childhood. Classification and clinicopathologic study of 406 cases. Pathol Annu 6:417–474

    CAS  PubMed  Google Scholar 

  3. (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

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

    CAS  PubMed  Google Scholar 

  5. Korbet SM (1999) Clinical picture and outcome of primary focal segmental glomerulosclerosis. Nephrol Dial Transplant 14(Suppl 3):68–73

    PubMed  Google Scholar 

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

    Article  CAS  PubMed  Google Scholar 

  7. Newman WJ, Tisher CC, McCoy RC, Gunnells JC, Krueger RP, Clapp JR, Robinson RR (1976) Focal glomerular sclerosis: contrasting clinical patterns in children and adults. Medicine (Baltimore) 55:67–87

    CAS  Google Scholar 

  8. 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

    CAS  PubMed  Google Scholar 

  9. (1985) Focal segmental glomerulosclerosis in children with idiopathic nephrotic syndrome. A report of the Southwest Pediatric Nephrology Study Group. Kidney Int 27:442–449

  10. Pei Y, Cattran D, Delmore T, Katz A, Lang A, Rance P (1987) Evidence suggesting under-treatment in adults with idiopathic focal segmental glomerulosclerosis. Regional Glomerulonephritis Registry Study. Am J Med 82:938–944

    CAS  PubMed  Google Scholar 

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

    CAS  PubMed  Google Scholar 

  12. Beaufils H, Alphonse JC, Guedon J, Legrain M (1978) Focal glomerulosclerosis: natural history and treatment. A report of 70 cases. Nephron 21:75–85

    CAS  PubMed  Google Scholar 

  13. 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

    CAS  PubMed  Google Scholar 

  14. 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  CAS  PubMed  Google Scholar 

  15. 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

    CAS  PubMed  Google Scholar 

  16. Cameron JS (1996) The enigma of focal segmental glomerulosclerosis. Kidney Int(Suppl 57):S119–S131

    CAS  PubMed  Google Scholar 

  17. D’Agati VD, Fogo AB, Bruijn JA, Jennette JC (2004) Pathologic classification of focal segmental glomerulosclerosis: a working proposal. Am J Kidney Dis 43:368–382

    PubMed  Google Scholar 

  18. Schwartz GJ, Haycock GB, Edelmann CM, Jr, Spitzer A (1976) A simple estimate of glomerular filtration rate in children derived from body length and plasma creatinine. Pediatrics 58:259–263

    CAS  PubMed  Google Scholar 

  19. Burritt MF, Slockbower JM, Forsman RW, Offord KP, Bergstralh EJ, Smithson WA (1990) Pediatric reference intervals for 19 biologic variables in healthy children. Mayo Clin Proc 65:329–336

    CAS  PubMed  Google Scholar 

  20. Cherian AG, Hill JG (1978) Percentile estimates of reference values for fourteen chemical constituents in sera of children and adolescents. Am J Clin Pathol 69:24–31

    CAS  PubMed  Google Scholar 

  21. 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

    CAS  PubMed  Google Scholar 

  22. 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

    CAS  PubMed  Google Scholar 

  23. 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

    CAS  PubMed  Google Scholar 

  24. 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

    CAS  PubMed  Google Scholar 

  25. 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

    CAS  PubMed  Google Scholar 

  26. 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

    CAS  PubMed  Google Scholar 

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

    CAS  PubMed  Google Scholar 

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

    CAS  PubMed  Google Scholar 

  29. Abrantes MM, Cardoso LS, Lima EM, Silva JM, Diniz JS, Bambirra EA, Oliveira EA (2006) Clinical course of 110 children and adolescents with primary focal segmental glomerulosclerosis. Pediatr Nephrol 21:482–489

    PubMed  Google Scholar 

  30. Korbet SM, Schwartz MM, Lewis EJ (1986) The prognosis of focal segmental glomerular sclerosis of adulthood. Medicine (Baltimore) 65:304–311

    CAS  Google Scholar 

  31. Detwiler RK, Falk RJ, Hogan SL, Jennette JC (1994) Collapsing glomerulopathy: a clinically and pathologically distinct variant of focal segmental glomerulosclerosis. Kidney Int 45:1416–1424

    CAS  PubMed  Google Scholar 

  32. 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

    CAS  PubMed  Google Scholar 

  33. Schwartz MM (2005) The blind men and the elephant. Kidney Int 68:1894–1895

    PubMed  Google Scholar 

  34. Saint-Hillier Y, Morel-Maroger L, Woodrow D, Richet G (1975) Focal and segmental hyalinosis. Adv Nephrol Necker Hosp 5:67–88

    CAS  PubMed  Google Scholar 

  35. Velosa JA, Holley KE, Torres VE, Offord KP (1983) Significance of proteinuria on the outcome of renal function in patients with focal segmental glomerulosclerosis. Mayo Clin Proc 58:568–577

    CAS  PubMed  Google Scholar 

  36. McAdams AJ, Valentini RP, Welch TR (1997) The nonspecificity of focal segmental glomerulosclerosis. The defining characteristics of primary focal glomerulosclerosis, mesangial proliferation, and minimal change. Medicine (Baltimore) 76:42–52

    CAS  Google Scholar 

  37. Habib R, Gubler MC (1973) Focal sclerosing glomerulonephritis. Perspect Nephrol Hypertens 1 Pt 1:263–278

    CAS  PubMed  Google Scholar 

  38. Chan PC, Chan KW, Cheng IK, Chan MK (1991) Focal sclerosing glomerulopathy. Risk factors of progression and optimal mode of treatment. Int Urol Nephrol 23:619–629

    CAS  PubMed  Google Scholar 

  39. Banfi G, Moriggi M, Sabadini E, Fellin G, D’Amico G, Ponticelli C (1991) The impact of prolonged immunosuppression on the outcome of idiopathic focal-segmental glomerulosclerosis with nephrotic syndrome in adults. A collaborative retrospective study. Clin Nephrol 36:53–59

    CAS  PubMed  Google Scholar 

  40. Miyata J, Takebayashi S, Taguchi T, Naito S, Harada T (1986) Evaluation and correlation of clinical and histological features of focal segmental glomerulosclerosis. Nephron 44:115–120

    CAS  PubMed  Google Scholar 

  41. Guillot AP, Kim MS (1993) Pulse steroid therapy does not alter the course of focal segmental glomerulosclerosis (abstract). J Am Soc Nephrol 4:276

    Google Scholar 

  42. Waldo FB, Benfield MR, Kohaut EC (1992) Methylprednisolone treatment of patients with steroid-resistant nephrotic syndrome. Pediatr Nephrol 6:503–505

    CAS  PubMed  Google Scholar 

  43. Hari P, Bagga A, Jindal N, Srivastava RN (2001) Treatment of focal glomerulosclerosis with pulse steroids and oral cyclophosphamide. Pediatr Nephrol 16:901–905

    CAS  PubMed  Google Scholar 

  44. 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

    CAS  PubMed  Google Scholar 

  45. Passerini P, Ponticelli C (2001) Treatment of focal segmental glomerulosclerosis. Curr Opin Nephrol Hypertens 10:189–193

    CAS  PubMed  Google Scholar 

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

    CAS  PubMed  Google Scholar 

  47. Ponticelli C (1995) Focal segmental glomerular sclerosis. To treat or not to treat? 1. It is worthwhile to give the adult patient with nephrotic syndrome the benefit of an adequate therapeutic trial? Nephrol Dial Transplant 10:2351–2354

    CAS  PubMed  Google Scholar 

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Authors and Affiliations

  1. Department of Pediatrics, Seoul National University Children’s Hospital, 28 Yongon-Dong, Chongno-Gu, Seoul, 110-744, South Korea

    Bum Hee Lee, Hee Yeon Cho, Hee Gyung Kang, Il Soo Ha, Hae Il Cheong & Yong Choi

  2. Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, 50 Il-Won Dong, Gang-Nam Gu, 135-710, Seoul, South Korea

    Kyung Hoon Paik & Dong-Kyu Jin

  3. Department of Pathology, Seoul National University Hospital, 28 Yongon-Dong, Chongno-Gu, Seoul, 110-744, South Korea

    Kyung Chul Moon

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  1. Kyung Hoon Paik
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  2. Bum Hee Lee
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Correspondence to Yong Choi.

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Paik, K.H., Lee, B.H., Cho, H.Y. et al. Primary focal segmental glomerular sclerosis in children: clinical course and prognosis. Pediatr Nephrol 22, 389–395 (2007). https://doi.org/10.1007/s00467-006-0301-5

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