Prognostic factors and survivals of children with steroid-resistant nephrotic syndrome

  • Partini Pudjiastuti Trihono Department of Child Health, University of lndonesia Medical School
  • Nina Dwi Putri Department of Child Health, University of lndonesia Medical School
  • Aman B Pulungan Department of Child Health, University of lndonesia Medical School
Keywords: steroid-resistant nephrotic syndrome, end-stage renal disease, doubling of base creatinine levels, survival

Abstract

Background Children with steroid resistant nephrotic syndrome
(SRNS) generally survive, although during the course of disease
their kidney function may decrease, leading to end-stage renal
disease (ESRD). There have been few studies reporting on the
survivals of children with SRNS.
Objectives To determine patient and kidney survival rates in children
with SRNS at the first, second, third, fourth, and fifth years; and to
evaluate the effects of age at onset, initial kidney function, hypertension,
and type of resistance, on the survivals of children with SRNS.
Methods This retrospective cohort study was performed using
secondary data obtained from medical records of patients with SRNS in
Department of Child Health, Cipto Mangunkusumo Hospira~ between
2004-2011. The outcomes of kidney survivals were defined in two ways:
lack of doubling of base creatinine levels and lack of ESRD.
Results There were 45 children with SRNS in our study. Their median
duration ofillness was 24 (range 12-95) months. Twenty percent of the
subjects died, 31.1 % had a doublingofbase creatinine levels, and 13.4%
developed ESRD. Life survival rates of subjects at the first, second,
third, fourth, and fifth years after diagnosis were 93 %, 84%, 80"/ri, 7 2%
,and 61 %, respectively. Kidney survival rates determined by the lack
of doubling of base creatinine levels at the first, second, third, fourth
and fifth years were 92%, 72%, 56%, 42%, and 34%, respectively, while
kidney survival rates determined by the lack ofESRD were 97%, 88%,
81 %, 70"/o, and 58%, respectively. Age at onset, initial kidney function,
hypertension at onset, and type of resistance, did not significantly affect
the survivals of children with SRNS.
Conclusion Children with SRNS are prone to develop a doubling
of base creatinine levels and ESRD. Factors such as age, initial
kidney function, hypertension at onset, and type ofresistance, do
not significantly affect both, life and kidney survivals of children
with SRNS.

References

1. Roth KS, Amaker BH, Chan JCM. Nephrotic syndrome:
pathogenesis and management. Pediatr Rev. 2002;203:237-
48.
2. Wirya WION. Penelitian beberapa aspek klinis dan patologi
anatomis sindrom nefrotik primer pada anak di Indonesia
[dissertation]. Jakarta: Bagian Ilmu Kesehatan Anak FKUI;
1992.
3. Vidianty J, Pardede SO, Pudjiastuti P, Laksmi E, Alatas H,
Tambunan T. Gambaran antopometri pada anak dengan
sindrom nefrotik. In: Sadjimin T, Jufri M, Julia M, Wibowo T,
editors. Buku abstrak pertemuan ilmiah tahunan ilmu kesehatan
anak III. FK UGM; 2007 6-9 Mei; Yogyakarta, 2007.
4. Banerjee S. Steroid resistant nephrotic syndrome. Indian J
Pediatr. 2002;69:1065-9.
5. Niaudet P. Treatment of childhood steroid-resistant idiopathic
nephrosis with a combination of cyclosporine and prednisone.
J Pediatr. 1994;125:981-6.
6. Bagga A. Management of steroid resistant nephrotic
syndrome. Indian J Pediatr. 2009;46:35-47.
7. Mekahli D, Liutkus A, Ranchin B, Yu A, Bessenay L, Girardin
E, et al. Long-term outcome of idiopathic steroid-resistant
nephrotic syndrome: a multicenter study. Pediatr Nephrol.
2009;24:1525-32.
8. Alatas H, Tambunan T, Trihono PP, Pardede SO. Konsensus
tata laksana sindrom nefrotik idiopatik pada anak. Unit Kerja
Koordinasi N efrologi, Ikatan Dokter Anak Indonesia.Jakarta:
IDAI; 2005.
9. Oluwu WA, Adelusola KA, AdefehnitiA. Childhood idiopathic
steroid resistant nephrotic syndrome in Southwestern Nigeria.
Saudi] Kidney Dis Transpl. 2010;21:979-80.
10. Otukesh H, Otukesh S, Mojtahedzadeh M, Hoseini R,
Fereshtehnejad SM, Fard AR. Management and outcome
of steroid-resistant nephrotic syndrome in children. Iran J
Kidney Dis. 2009;3:210-7.
11. Hodson EM, Wilis NS, Craig JS. Interventions of idiopathic
steroid-resistant nephrotic syndrome in children (review).
Cochrane. 2010;11:1-87.
12. Kriz W, Hartmann I, Hosser H, Hahne! B, Kranzlin B. Tracer
studies in the rat demonstrate misdirected filtration and
peritubular filtrate spreading in nephrons with segmental
glomerulosclerosis. J Am Soc Nephrol. 2001; 12:496-506.
13. Goumenos DS, Tsagalis G, Nahas AM, Shortlan JR,
Davlouros P, Vlachojannis JG, et al. Immunosuppressive
treatment ofidiophatic focal segmental glomerulosclerosis: a
five-year follow up study. Nephron Clin Pract. 2006; 104:c75-
82.
14. Fomina S, Pavlenko T, Englund E, Bagdasarova I. Clinical
patterns and renal survival of nephrotic syndrome in
childhood: a single center study (1980-2006). The Open
Urology & Nephrology Journal. 2010;3:8-15.
15. Gulati S, Sengupta D, Sharma RK, Sharma A, Gupta RK,
Singh U, et al. Steroid resistant nephrotic syndrome: role of
histopathology. Indian Pediatr. 2006;43:55-60.
Published
2013-02-28
How to Cite
1.
Trihono P, Putri N, Pulungan A. Prognostic factors and survivals of children with steroid-resistant nephrotic syndrome. PI [Internet]. 28Feb.2013 [cited 24Nov.2024];53(1):42-. Available from: https://paediatricaindonesiana.org/index.php/paediatrica-indonesiana/article/view/247
Section
Articles
Received 2016-08-18
Accepted 2016-08-18
Published 2013-02-28