Cyclophosphamide Therapy for Children with Steroid Dependent and Frequently Relapsing Nephrotic Syndrome

Abstract

ABSTRACT
Background: Nephrotic syndrome is primarily a paediatric disorder with an incidence of
2-3/100000 children per year. Cyclophosphamide could be used as adjuvant to steroid or
as alternative therapy to prolong the duration of remission and reduce the number of
relapses in children with frequently relapsing and steroid dependent nephrotic syndrome.
Objectives: To evaluate the cyclophosphamide therapy in children with steroid-dependent
and frequently relapsing nephrotic syndrome.
Methods: Forty three patients with frequently relapsing and steroid dependent nephrotic
syndrome recorded in Paediatric Nephrology
Consultation Department at both Al-Kadhymia Teaching Hospital and Central Teaching
Hospital of Pediatric were studied prospectively from the first of January 2004 to 31 of
December 2007 after giving single course of oral cyclophosphamide for 8-12 w and
followed strictly for 2-3 years during and after therapy. Thirty one were males and 12 were
females.
Results: A major portion of patients, 23(53.5%) were given oral cyclophosphamide in a
dose of 2-3 mg/kg/day and this is the usual dose range in most centres which associated
with better response in term of longer duration of remission and lower adverse effect.
The relation of cyclophosphamide adverse effects to the duration of treatment is not
clearly defined from this study since all patients taking it for 12 w developed the short term
adverse effects of the drug. The adverse effects of cyclophosphamide were frequent, so
that 16 patients (37%) develop one or more of the known adverse effects with neutropenia
being the most common (28%). All adverse effects of cyclophosphamide were more
frequent in males than females (42% versus 25%). There is close relationship between
the dose of the drug and the long term response to therapy. Females show relatively
longer remission periods than males. In relation to age, remission periods was longer in
those received the drug in younger age.
Conclusion: Cyclophosphamide is a relatively safe drug in nephrotic children provided
that it is given in a proper dose. Cyclophosphamide is an effective adjuvant to steroid
therapy in maintaining remission for 1-2 years and minimizing the adverse effects of
steroid.