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Focal glomerulosclerosis in children: an argentinian experience

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Abstract

Twenty-six children presenting with idiopathic nephrotic syndrome and a histological diagnosis of focal glomerulosclerosis were studied retrospectively to evaluate their response to treatment, outcome and clinicopathological correlations. Twenty-two patients (84.6%) were steroid resistant; of these, 8 of the 19 with focal segmental glomerulosclerosis and 2 of the 3 with focal global within 16 weeks of starting therapy. Seven patients relapsed after a CY-induced remission, but 5 of them became steroid responsive. After an average follow-up of 83 months, 17 patients are in remission with normal renal function, 3 patients have persistent nephrotic range proteinuria and 6 patients are in chronic renal failure. Persistence of proteinuria, a high percentage of segmentally selerotic glomeruli and diffuse mesangial proliferation were indicators of poor prognosis. We believe longer courses of CY therapy than those traditionally utilized are responsible for the relatively good results in our patients.

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References

  1. Habib R, Kleinknecht C (1971) The primarynephrotic syndrome of childhood: classification and clinicopathological study of 406 cases. Pathol Annu 6:417–474

    Google Scholar 

  2. Churg J, Habib R, White R (1970) Pathology of the nephrotic syndrome in children. Lancet I:1299–1302

    Google Scholar 

  3. Habib R (1973) Focal glomerular sclerosis (editorial). Kidney Int 4:355–361

    Google Scholar 

  4. Habib R, Gubler M (1975) Focal glomerularsclerosis associated with nephrotic syndrome. In: Rubin MI, Barrat TM (eds) Pediatric nephrology. Williams & Wilkins, Baltimore, pp 559–574

    Google Scholar 

  5. ISKDC report (1981) Primary nephrotic syndrome in children. Identification of patients with minimal change nephrotic syndrome from initial response to prednisone. J Pediatr 98:561–564

    Google Scholar 

  6. Nash M, Greifer I, Obling H, Bernstein J, Bennet B, Spitzer A (1976) The significance of focal sclerotic glomeruli in children. J Pediatr 88:806–813

    Google Scholar 

  7. Ellis D, Kapur S, Antonovich T, Salcedo J, Yunis E (1978) Focal glomerulosclerosis in children: correlation of histology with prognosis. J Pediatr 93:762–768

    Google Scholar 

  8. Cameron J, Turner D, Ogg C, Chantler C (1978) The long term prognosis of patients with focal segmental glomerulosclerosis. Clin Nephrol 10:213–218

    Google Scholar 

  9. Hyman L, Burkholder P (1973) Focal sclerosing glomerulopathy with segmental hyalinosis. Lab Invest 28:533–544

    Google Scholar 

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

    Google Scholar 

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

    Google Scholar 

  12. Velosa J, Donadio J, Holley K (1975) Focal sclerosing glomerulopathy, a clinicopathologic study. Mayo Clin Proc 50:121–132

    Google Scholar 

  13. schoeneman M, Bennet B, Greifer I (1978) The natural history of focal segmental glomerulosclerosis with and without mesangial hypercellularity in children. Clin Nephrol 9:45–54

    Google Scholar 

  14. Walherr R, Gubler M, Levy M, Broyer M, Habib R (1978) The significance of pure diffuse mesangial proliferation in idiopathic nephrotic syndrome. Clin Nephrol 10:171–179

    Google Scholar 

  15. Gubler M, Waldherr R, Levy M, Broyer M, Habib R (1979) Idiopathic nephrotic syndrome with focal and segmental sclerosis and/or hyalinosis: clinical course, response to therapy and long term outcome. In: Strauss J (ed) Pediatric nephrology. Garland STPM Press, New York, pp 193–212

    Google Scholar 

  16. Kohaut E, Singer D, Hill LL (1976) The significance of focal glomerularsclerosis in children who have nephrotic syndrome. Am J Clin Pathol 66:545–550

    Google Scholar 

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

    Google Scholar 

  18. Brown C, Cameron J, Turner D, Chantler C, Ogg C, William D, Bewick M (1978) Focal segmental glomerulosclerosis with rapid decline in renal function (“malignant FSGS”). Clin Nephrol 10:51–61

    Google Scholar 

  19. Mongeau J, Corneille L, Robitaille P, O'Regan S, Pelletier M (1981) Primary nephrosis in childhood associated with focal glomerularsclerosis: is long term prognosis that severe? Kidney Int 20:743–746

    Google Scholar 

  20. Report of the Task Force on blood pressure control in children (1977) Pediatrics [Suppl]: 59:797–801

    Google Scholar 

  21. Yoshikawa N, Hiroshi I, 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

    Google Scholar 

  22. Geary DF, Farine M, Thorner P, Baumal R (1984) Response to cyclophosphamide in steroid resistant focal segmental glomerularsclerosis: a reappraisal. Clin Nephrol 22:109–113

    Google Scholar 

  23. Beny P, Hawkins E (1984) Multicenter collaborative clinicopathologic study of focal segmental glomerulosclerosis in children with nephrotic syndrome (abstract). Kidney Int 24:154

    Google Scholar 

  24. Moncrieff M, White R, Ogg C, Cameron G (1969) Cyclophosphamide therapy in the nephrotic syndrome in childhood. BMJ 1:666–670

    Google Scholar 

  25. ISKDC report (1974) Prospective, controlled trial of cyclophosphamide therapy in children with nephrotic syndrome. Lancet II:423–427

    Google Scholar 

  26. Schein P, Winokur S (1975) Immunosuppressive and cytotoxic chemotherapy: long term complications. Ann Intern Med 82:84–95

    Google Scholar 

  27. Meadow S, Weller R, Archibald E (1969) Fatal systemic measles in a child receiving cyclophosphamide for nephrotic syndrome. Lancet II:876–878

    Google Scholar 

  28. Feehally J, Beattie T, Brenchley P, Coupes B, Houston I, Mallick N, Postletwaite R (1984) Modulation of cellular immune function by cyclophosphamide in children with minimal change nephropathy. N Engl J Med 310:415–420

    Google Scholar 

  29. Wall C, Clansen K (1975) Carcinoma of the urinary bladder in patients receiving cyclophosphamide. N Engl J Med 293:271–273

    Google Scholar 

  30. Grunwald HW, Rosner F (1979) Acute leukemia and immunosuppressive drug use. Arch Intern Med 139:461–466

    Google Scholar 

  31. Etteldorf JN, West CD, Pitcock JA, Williams DL (1976) Gonadal function, testicular histology, and meiosis following cyclophosphamide therapy in patients with nephrotic syndrome. J Pediatr 88:206–212

    Google Scholar 

  32. Velosa J, Holley K, Torres V, Offord K (1983) Significance of proteinuria on the outcome of renal function in patients with focal segmental glomerulosclerosis. Mayo Clin Proc 58:568–577

    Google Scholar 

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Authors and Affiliations

  1. Paediatric Nephrology Section, Hospital Professor Alejandro Posadas, Buenos Aires, Argentina

    Horacio Repetto

  2. Department of Pathology, Hospital Professor Alejandro Posadas, Buenos Aires, Argentina

    Estela Alvarez

  3. Instituto Alfredo Lanari, Buenos Aires, Argentina

    Alda Tufro-McReddie & Elvira Arrizurieta

Authors
  1. Alda Tufro-McReddie
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  2. Estela Alvarez
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  3. Elvira Arrizurieta
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  4. Horacio Repetto
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Additional information

Present address: University of Virginia, Department of Pediatrics/Nephrology, MR4 Building, Room 2001, Charlottesville, VA 22908, USA

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Tufro-McReddie, A., Alvarez, E., Arrizurieta, E. et al. Focal glomerulosclerosis in children: an argentinian experience. Pediatr Nephrol 6, 158–161 (1992). https://doi.org/10.1007/BF00866298

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  • DOI: https://doi.org/10.1007/BF00866298

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