Management and outcome of steroid-resistant nephrotic syndrome in children.
BACKGROUND
Steroid-resistant nephrotic syndrome (SRNS) is uncommon in children, but often leads to ESRD. We report our experience with SRNS and its treatments and outcomes.


METHODS
We assessed 73 children with SRNS admitted to Ali Asghar Children Hospital in Tehran, Iran. Their clinical presentations, treatment, and disease courses were reviewed. The mean follow-up duration was 6.0 +/- 4.2 years. Moreover, survival times were calculated and the Cox regression method was used to determine variables able to predict survival of the kidneys.


RESULTS
Age at the onset of the disease, sex, and hematuria were not predictive of the response to treatment with immunosuppressive drugs in the children with SRNS. The type of resistance (early or late) was associated with the responsiveness to immunosuppressives. Response to any of the immunosuppressive drugs determined the responsiveness to other immunosuppressive drugs. Cyclosporine was more effective than cyclophosphamide as initial therapy. The mean kidney survival time was 11.62 years. Kidney survival rates were 94.6%, 70.0%, 56.0%, and 34.0% at 1, 5, 10, and 15 years, respectively, in patients with initial resistance to steroid, while these were 100%, 100%, 83.0%, and 83.0% in those with late resistance, respectively (P = .03).


CONCLUSIONS
We showed that patients with late steroid resistance had better response to immunosuppressive drugs than patients with early resistance. We also showed that resistance to immunosuppressive therapies increased the risk of resistance to other immunosuppressive drugs. Achievement of complete or partial remission with any therapy reduced the risk of ESRD.
Department of Nephrology, Ali Asghar Children Hospital, Iran University of Medical Sciences, Tehran, Iran. hasanotukesh@yahoo.com
Hassan
H
Otukesh
Salman
S
Otukesh
Mona
M
Mojtahedzadeh
Rozita
R
Hoseini
Seyed-Mohammad
SM
Fereshtehnejad
Azam
A
Riahi Fard
Nader
N
Sadigh
Ashkan
A
Heshmatzade Behzadi
Ramila
R
Javadi
Nakissa
N
Hooman
Mitra
M
Mehrazma
D000367:Age Factors; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D004351:Drug Resistance; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D007492:Iran; D007676:Kidney Failure, Chronic / Q000175:diagnosis / Q000453:epidemiology* / Q000517:prevention & control; D008297:Male; D009404:Nephrotic Syndrome / Q000150:complications / Q000175:diagnosis* / Q000628:therapy*; D012189:Retrospective Studies; D016019:Survival Analysis; D016896:Treatment Outcome
D005938:Glucocorticoids; D007166:Immunosuppressive Agents