Children with Steroid-resistant Nephrotic Syndrome: Long-term Outcomes of Sequential Steroid Therapy.
OBJECTIVE
This study aimed to investigate the long-term outcomes in children with steroid-resistant nephrotic syndrome (SRNS), who received methylprednisolone pulse therapy (MPT)-based sequential steroid therapy. In particular, we aimed to observe whether these patients had a high risk of adverse events.


METHODS
We conducted a retrospective study over a 5-year period. The long-term outcomes for children with SRNS receiving sequential therapy were observed.


RESULTS
Sixty-three children were diagnosed with SRNS and underwent MPT-based sequential steroid therapy. Thirty-five (55.6%) achieved complete or partial remission, 19 (30.2%) of whom were in remission even after treatment cessation at last review. The mean time to initial remission after MPT was 24.3±13.1 days. Forty-nine children (77.8%) experienced relapses, of whom 31 (49.2%) demonstrated a frequent relapsing course. Adverse effects relevant to MPT were generally mild and infrequent. Five patients (7.9%) complained of vomiting or nausea during MPT infusion; 25 (39.7%) experienced excessive weight gain and developed an obvious Cushingoid appearance; and 26 (41.3%) had poor growth associated with long-term steroid use. Twenty-eight patients (44.4%) failed to respond to MPT, of whom 21 (33.3%) achieved complete or partial remission with immunosuppressive agents.


CONCLUSIONS
MPT-based sequential steroid therapy appears to be a safe and effective method for inducing rapid remission in childhood SRNS. Further clinical studies are needed to comprehensively evaluate this therapy.
The department of Pediatrics, West China Second University Hospital of Sichuan University, Chengdu 610041, Sichuan, China.
Hui
H
Zhang
The department of Pediatrics, West China Second University Hospital of Sichuan University, Chengdu 610041, Sichuan, China; Key Laboratory of Birth Defects and Related Diseases of Women and Children (Sichuan University), Ministry of Education, Chengdu 610041, Sichuan, China.
Zheng
Z
Wang
The department of Pediatrics, West China Second University Hospital of Sichuan University, Chengdu 610041, Sichuan, China.
Li Qun
LQ
Dong
The department of Pediatrics, West China Second University Hospital of Sichuan University, Chengdu 610041, Sichuan, China; Key Laboratory of Birth Defects and Related Diseases of Women and Children (Sichuan University), Ministry of Education, Chengdu 610041, Sichuan, China.
Yan Nan
YN
Guo
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D004351:Drug Resistance; D005260:Female; D006801:Humans; D008297:Male; D008775:Methylprednisolone / Q000008:administration & dosage* / Q000009:adverse effects; D009404:Nephrotic Syndrome / Q000188:drug therapy*; D012189:Retrospective Studies; D013997:Time Factors; D016896:Treatment Outcome
D008775:Methylprednisolone
Clinical outcome; Focal segmental glomerulosclerosis; Methylprednisolone; Minimal change disease; Steroid resistance