Article Text

TREATMENT OF STEROID-RESISTANT NEPHROTIC SYNDROME IN CHILDREN
  1. J A Kari1,
  2. M Halawani1
  1. 1Department of Pediatrics, King Abdul Aziz University Hospital, Jeddah, Saudi Arabia

Abstract

Objectives Achieving remission in children with steroid-resistant nephrotic syndrome (SRNS) could be difficult. We have studied the response of children with SRNS to variable modality of therapy.

Methods We reviewed the notes of all primary nephrotic children with SRNS, presented between 2002 and 2007. The inclusion criteria were SRNS, renal biopsy and follow-up duration of at least one year.

Results 31 children fulfilled the inclusion criteria, 23 girls and eight boys . Their mean age at presentation ± SD was 4.2 ± 3.2 years. The renal histopathology was compatible with FSGS in 55% (17 children).

Response to Treatment 20 children (65%) achieved partial (six children) or complete remission (14 children). 17 children were treated with cyclophosphamide either oral or intravenous. Only four children (23.5%) achieved remission. Seven children received oral chlorambucil, two of them (28.5%) achieved complete remission. Five children received cyclosporine, eight of them (53%) achieved remission. Six children developed gum hypertrophy and one had renal impairment, which was reversible after stopping ciclosporin. Mycophonelate mofetil (MMF) was used as the last option in five children. Two of them achieved complete remission. One child developed a systemic cytomegalovirus infection, which indicated stopping the MMF. 45% (14 children) needed more than one immunosuppressive therapy. Three children progressed to end-stage renal failure and required dialysis.

Conclusion SRNS in children is a difficult disease with significant morbidity. However, remission is achievable with ciclosporin and other immunosuppressive agents.

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