Primary focal segmental glomerulosclerosis in children: epidemiology, clinical presentation and prognosis

##plugins.themes.academic_pro.article.sidebar##

Published Aug 24, 2023
Download
PDF
Vol. 101 No. 3 (2023): Tunis Med mars 2023

##plugins.themes.academic_pro.article.main##

Abir Boussetta
Université Tunis el manar, faculté de médecine de tunis, service de pédiatrie hôpital Charles Nicolle";}
https://orcid.org/0000-0002-8034-2355
Khouloud Ben Njima
Faculté de médecine de Monastir
Manel Jellouli
Université Tunis el manar, faculté de médecine de tunis, service de pédiatrie hôpital Charles Nicolle
Taher Gargah
Université Tunis el manar, faculté de médecine de tunis, service de pédiatrie hôpital Charles Nicolle

Abstract

Introduction: Focal segmental glomerulosclerosis is a histopathological entity.


Aim: To analyze the epidemiological, clinical and histological profile of primary focal segmental glomerulosclerosis in children, as well as their prognostic factors.


Methods: This was a retrospective cross-sectional study over a period of 20 years (2001-2020), conducted in the Department of Pediatrics at Charles Nicolle Hospital in Tunis, which included children followed for primary focal segmental glomerulosclerosis.


Results: There were 35 children, 19 boys and 16 girls. The median age was 4.5 years. Nephrotic syndrome was seen in 88% of patients. Macroscopic hematuria was found in 4 cases, hypertension in 8 cases and renal failure in 7 cases at presentation. The most common variant was the not otherwise specified variant (77.1%). Steroid-sensitive nephrotic syndrome was observed in 71% of cases, and steroid-resistance in 29% of cases. Treatment with cyclosporine was indicated in 23 patients with complete remission rate of 56.5%. 42,8% children had progressed to chronic kidney disease, including an end-stage renal disease in 11.5% of cases. Only the presence of a family history of kidney disease was found as a predictive factor of progression to chronic kidney disease and end-stage renal disease. Renal survival rates were estimated at 100% at 3 years, 85% at 5 years and 73% at 10 years.


Conclusion: Identification of patients at high risk for chronic kidney disease progression, such as those with a family history of kidney disease or those who have failed to respond to corticosteroids, would allow therapeutic adjustments.

Keywords:

Primary focal segmental glomerulosclerosis, Nephrotic syndrome, Child, Renal failure, Prognosis

##plugins.themes.academic_pro.article.details##

Creative Commons License

This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.

References

  1. Korbet SM. Treatment of primary focal segmental glomerulosclerosis. Kidney Int. 2002; 62(6):2301 10.
  2. Thomas DB, Franceschini N, Hogan SL, ten Holder S, Jennette CE, Falk RJ, et al. Clinical and pathologic characteristics of focal segmental glomerulosclerosis pathologic variants. Kidney Int. 2006;69(5):920 6.
  3. Shabaka A, Tato Ribera A, Fernandez Juarez G. Focal segmental glomerulosclerosis: state of the art and clinical perspective. Nephron. 2020;144(9):413-27.
  4. Filler G, Young E, Geier P, Carpenter B, Drukker A, Feber J. Is there really an increase in non-minimal change nephrotic syndrome in children. Am J Kidney Dis. 2003;42(6):1107 13.
  5. Boyer O, Moulder JK, Somers MJG. Focal and segmental glomerulosclerosis in children: a longitudinal assessment. Pediatr Nephrol. 2007;22(8):1159 66.
  6. Smith JM, Stablein DM, Munoz R, Hebert D, McDonald RA. Contributions of the Transplant Registry: The 2006 Annual Report of the North American Pediatric Renal Trials and Collaborative Studies (NAPRTCS). Pediatr Transplant. 2007;11(4):366 73.
  7. Rosenberg AZ, Kopp JB. Focal segmental glomerulosclerosis. Clin J Am Soc Nephrol. 2017;12(3):502-17.
  8. Eddy AA, Symons JM. Nephrotic syndrome in childhood. The Lancet. 2003 Aug;326(9384):629-39.
  9. André J-L. Hypertension artérielle chez l’enfant et l’adolescent. EMC - Cardiol-Angéiologie. 2005;2(4):478 90.
  10. Schwartz GJ, Muñoz A, Schneider MF, Mak RH, Kaskel F, Warady BA, et al. New Equations to Estimate GFR in Children with CKD. J Am Soc Nephrol. 2009;20(3):629 37.
  11. National Kidney Foundation. KDOQI clinical practice guidelines for chronic kidney disease: evaluation, classification and stratification. Am J Kidney Dis Off J Natl Kidney Found. 2002;39(2 Suppl 1):S1-266.
  12. D’Agati VD, Fogo AB, Bruijn JA, Jennette JC. Pathologic classification of focal segmental glomerulosclerosis: a working proposal. Am J Kidney Dis. 2004;43(2):368 82.
  13. Bérard É, Broyer M, Dehennault M, Dumas R, Eckart P, Fischbach M, et al. Syndrome néphrotique pur (ou néphrose) corticosensible de l’enfant. Néphrologie Thérapeutique. 2005;1(3):150 6.
  14. Smith JM, Stablein DM, Munoz R, Hebert D, McDonald RA. Contributions of the Transplant Registry: The 2006 Annual Report of the North American Pediatric Renal Trials and Collaborative Studies (NAPRTCS). Pediatr Transplant. 2007;11(4):366 73.
  15. Korbet SM. Treatment of Primary FSGS in Adults. J Am Soc Nephrol. 2012 Nov;23(11):1769 76.
  16. Tizki S, Lasry F, Khalifa HH, Itri M. Primary focal segmental glomerular sclerosis in children: epidemiology and prognosis. Nephrol Ther. 2013;9(6):433 7.
  17. Abrantes MM, Cardoso LSB, Lima EM, Silva JMP, Diniz JS, Bambirra EA, et al. Clinical course of 110 children and adolescents with primary focal segmental glomerulosclerosis. Pediatr Nephrol. 2006;21(4):482 9.
  18. Paik KH, Lee BH, Cho HY, Kang HG, Ha IS, Cheong HI, et al. Primary focal segmental glomerular sclerosis in children: clinical course and prognosis. Pediatr Nephrol. 2007;22(3):389 95.
  19. Beşbaş N, Ozaltin F, Emre S, Anarat A, Alpay H, Bakkaloğlu A, et al. Clinical course of primary focal segmental glomerulosclerosis (FSGS) in Turkish children: a report from the Turkish Pediatric Nephrology FSGS Study Group. Turk J Pediatr. 2010;52(3):255 61.
  20. Shakeel S, Mubarak M, Kazi JI. Frequency and clinicopathological correlations of histopathological variants of pediatric idiopathic focal segmental glomerulosclerosis. Indian J Nephrol. 2014;24(3):148 53.
  21. El-Refaey AM, Bakr A, Hammad A, Elmougy A, El-Houseeny F, Abdelrahman A, et al. Primary focal segmental glomerulosclerosis in Egyptian children: a 10-year single-centre experience. Pediatr Nephrol Berl Ger. 2010;25(7):1369 73.
  22. Sozeri B, Mir S, Mutlubas F, Sen S. The long-term results of pediatric patients with primary focal and segmental glomerulosclerosis. Saudi J Kidney Dis Transplant Off Publ Saudi Cent Organ Transplant Saudi Arab. 2010;21(1):87 92.
  23. Silverstein DM, Craver R. Presenting Features and Short-Term Outcome According to Pathologic Variant in Childhood Primary Focal Segmental Glomerulosclerosis. Clin J Am Soc Nephrol. 2007;2(4):700 7.
  24. Tizki S, Lasry F, Khalifa HH, Itri M. Primary focal segmental glomerular sclerosis in children: epidemiology and prognosis. Nephrol Ther. 2013;9(6):433 7.
  25. Cattran DC, Appel GB, Hebert LA, Hunsicker LG, Pohl MA, Hoy WE, et al. A randomized trial of cyclosporine in patients with steroid-resistant focal segmental glomerulosclerosis. North America Nephrotic Syndrome Study Group. Kidney Int. 1999;56(6):2220-6.
  26. Gipson DS, Gibson K, Gipson PE, Watkins S, Moxey-Mims M. Therapeutic approach to FSGS in children. Pediatr Nephrol. 2007;22(1):28-36.
  27. Lieberman KV, Tejani A. A randomized double-blind placebo-controlled trial of cyclosporine in steroid-resistant idiopathic focal segmental glomerulosclerosis in children. J Am Soc Nephrol JASN. 1996 Jan;7(1):56-63.
  28. Hogg R, Middleton J, Vehaskari VM. Focal segmental glomerulosclerosis: epidemiology aspects in children and adults. Pediatr Nephrol Berl Ger. 2007;22(2):183 6.
  29. Martinelli R, Okumura AS, Pereira LJ, Rocha H. Primary focal segmental glomerulosclerosis in children: prognostic factors. Pediatr Nephrol Berl Ger. 2001;16(8):658-61.
  30. Gipson DS, Gibson K, Gipson PE, Watkins S, Moxey-Mims M. Therapeutic approach to FSGS in children. Pediatr Nephrol. 2007;22(1):28-36.
  31. D’Agati VD, Alster JM, Jennette JC, Thomas DB, Pullman J, Savino DA, et al. Association of histologic variants in FSGS clinical trial with presenting features and outcomes. Clin J Am Soc Nephrol CJASN. 2013;8(3):399 406.
  32. Abrantes MM, Cardoso LSB, Lima EM, Penido Silva JM, Diniz JS, Bambirra EA, et al. Predictive factors of chronic kidney disease in primary focal segmental glomerulosclerosis. Pediatr Nephrol Berl Ger. 2006;21(7):1003 12.