Araştırma Makalesi

Long-term outcomes in children with idiopathic nephrotic syndrome: a single center experience

Cilt: 48 Sayı: 2 2 Temmuz 2023
PDF İndir
TR EN

Long-term outcomes in children with idiopathic nephrotic syndrome: a single center experience

Abstract

Purpose: Idiopathic nephrotic syndrome (INS) is a common glomerular disease observed in children. Depending on their response to steroids, patients can be classified either as having steroid-sensitive nephrotic syndrome (SSNS) or steroid-resistant nephrotic syndrome (SRNS). Whereas SSNS patients respond favorably to steroid treatment and achieve a complete remission with normal renal functions, SRNS patients do not, and are at high risk of developing end-stage renal diseases (ESRD). The aim of this study was to analyze the long-term outcomes of patients with INS. Materials and Methods: In our medical center, records of children diagnosed with INS were evaluated. Demographic information, laboratory results, response to treatment, and clinical progression were analyzed. Result: Ninety-one children (64% male) with a mean age of 11.1±4.1 years (3.5-18) were included in the patient cohort, with a mean age of diagnosis of 5.2±3.8 years (1-16.2) and a mean follow-up period of 5.7±2.8 years (2-12). Sixty-eight (75%) patients had SSNS, and 23 (25%) patients had SRNS. Among the SSNS patients, 18 (31%) were steroid-dependent, 12 (20%) were frequently relapsing, and 29 (49%) were infrequently relapsing. Renal biopsy was performed on 29 (32%) patients, 59% had focal segmental glomerulosclerosis. The complete remission rate was 94% for all patients, with 100% for SSNS and 74% for SRNS. ESRD was developed for 9% of patients with SRNS. Conclusion: The response to steroid treatment serves as a valuable prognostic indicator for INS as it plays a pivotal role in mitigating the risk of progression toward end-stage renal failure.

Keywords

Nephrotic syndrome , steroid response , children

Kaynakça

  1. Eddy AA, Symons JM. Nephrotic syndrome in childhood. Lancet. 2003;362:629-39.
  2. Clark AG, Barratt TM. Steroid responsive nephrotic syndrome. Pediatric Nephrology, 4 th edition (Eds TM Barratt, ED Avner, WE Harmon): 731-45. Baltimore, Lippincott Williams& Wilkins, 1999.
  3. Noone DG, Iijima K, Parekh R. Idiopathic nephrotic syndrome in children Lancet. 2018;392:61-74.
  4. Hjorten R, Anwar Z, Reidy KJ. Long-term Outcomes of childhood onset nephrotic syndrome. Front Pediatr. 2016;4:53.
  5. Trautmann A, Schnaidt S, Lipska-Zietkiewicz BS, Bodria M, Ozaltın F, Emma F et al. Long-term outcome of steroid-resistant nephrotic syndrome in children. J Am Soc Nephrol. 2017;28:3055-65.
  6. Inaba A, Hamasaki Y, Ishikura K, Hamada R, Sakai T, Hataya H et al. Long-term outcome of idiopathic steroid-resistant nephrotic syndrome in children. Pediatr Nephrol. 2016;31:425-34.
  7. International Study of Kidney Disease in Children. Primary nephrotic syndrome in children: clinical significance of histopathologic variants of minimal change and of diffuse mesangial hypercellularity. A Report of the International Study of Kidney Disease in Children. Kidney Int. 1981;20:765-71.
  8. Hacıhamdioğlu DÖ, Kalman S, Gök F. Long-term results of children diagnosed with idiopathic nephrotic syndrome, single center experience. Türk Ped Arş. 2015;50:37-44.
  9. Mishra OP, Abhinay A, Mishra RN, Prasad R, Pohl M. Can we predict relapses in children with idiopathic steroid-sensitive nephrotic syndrome? J Trop Pediatr. 2013;59:343-9.
  10. Özlü SG, Demircin G, Tökmeci N, Yılmaz AÇ, Aydoğ Ö, Bülbül M et al. Long-term prognosis of idiopathic nephrotic syndrome in children. Ren Fail. 2015;37:672-7.

Kaynak Göster

MLA
Parmaksız, Gönül. “Long-term outcomes in children with idiopathic nephrotic syndrome: a single center experience”. Cukurova Medical Journal, c. 48, sy 2, Temmuz 2023, ss. 697-05, doi:10.17826/cumj.1243700.