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2012, Number 5

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Bol Med Hosp Infant Mex 2012; 69 (5)

Steroid-resistant nephrotic syndrome: 15 years experience from the Hospital de Pediatría, Centro Médico Nacional Siglo XXI

Aguilar KMA, Zepeda MCC, Ibarra CMP, Sánchez BJL, Luna SRM, Mendoza GML, Diaz LK, Manuel UJ
Full text How to cite this article

Language: Spanish
References: 9
Page: 411-417
PDF size: 251. Kb.


Key words:

steroid-resistant nephrotic syndrome, children, outcome.

ABSTRACT

Background. In 1997, the Clinic for Nephrotic Syndrome was established at the Hospital de Pediatría, Centro Médico Nacional Siglo XXI, Instituto Mexicano del Seguro Social (Mexico City); 30-50% of children with steroid-resistant nephrotic syndrome develop chronic renal failure and 60-80% achieve remission with cyclosporine. The objective of the study was to report treatment response and prognosis using the described scheme in a group of patients with steroid-resistant nephrotic syndrome.
Methods. Retrospective study in children with steroid-resistant nephrotic syndrome was done. Remission frequency and renal survival were measured.
Results. One-hundred fifty seven patients were studied; 66.7% were male. Mean age at diagnosis was 5.9 ± 4.2 years. Biopsies showed 33 results (21.9%) with minimal changes (MC), 74 (49%) with diffuse mesangial proliferation (DMP) and 44 (29.1%) with focal segmental glomerulosclerosis (FSGS). Mean follow-up time was 59.3 months (minimum 3 months, maximum 178 months); 59% were on cyclosporine and 17.3% cyclophosphamide. Twenty six patients received both treatments and six patients received all three medications; 78.2% entered remission, 5.8% were on dialysis and 1.9% died. Five-year renal survival was 92.9% and 10-year survival was 80%. Remission in patients with MC and DMP was 79.8% and 86.5%, respectively and in FSGS was 59.1%. Chronic renal failure was found more often in FSGS (20.4%). Risk of developing renal failure with FSGS compared with DMP was 4.7 times and FSGS compared with MC risk was 8.7 times greater.
Conclusions. Similar rates of remission and better renal survival were found compared with the literature


REFERENCES

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  2. Niaudet P. Treatment of childhood steroid-resistant idiopathic nephrosis with combination of cyclosporine and prednisone. French Society of Pediatric Nephrology. J Pediatr 1994;125:981-986.

  3. Cattran DC, Alexopoulos E, Heering P, Hoyer PF, Johnston A, Meyrier A, et al. Cyclosporin in idiopathic glomerular disease associated with the nephrotic syndrome: workshop recommendations. Kidney Int 2007;72:1429-1447.

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

  5. Tune BM, Kirpekar RK, Sibley RK, Reznik VM, Griswold WR, Mendoza SA. Intravenous methylprednisolone and oral alkylating agent therapy of prednisone-resistant pediatric focal segmental glomerulosclerosis: a long term follow-up. Clin Nephrol 1995;43:84-88.

  6. Gargah TTG, Lakhoua MR. Mycophenolate mofetil in treatment of childhood steroid-resistant nephrotic syndrome. J Nephrol 2011;24:203-207.

  7. Hamasaki Y, Yoshikawa N, Hattori S, Sasaki S, Iijima K, Nakanishi K, et al. Cyclosporine and steroid therapy in children with steroid-resistant nephrotic syndrome. Pediatr Nephrol 2009;24:2177-2185.

  8. Hoyer PF, Vester U, Becker JU. Steroid-resistant nephrotic syndrome. En: Geary DF, Schaefer F, eds. Comprehensive Pediatric Nephrology. Philadelphia: Mosby Elsevier; 2008. pp. 265-272.

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Bol Med Hosp Infant Mex. 2012;69