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2016 ; 8
(2
): 2039-47
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gab.com Text
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English Wikipedia
Immunosuppressive therapy in children with steroid-resistant,
frequently-relapsing, and steroid-dependent idiopathic nephrotic syndrome: a
single center experience
#MMPMID27054016
Moustafa BH
; Tolba OA
Electron Physician
2016[Feb]; 8
(2
): 2039-47
PMID27054016
show ga
INTRODUCTION: Immunosuppressive agents are recommended for the management of
children with steroid-resistant, frequently-relapsing, and steroid-dependent
idiopathic nephrotic syndrome, i.e., SRNS, FRNS, and SDNS. This study evaluated
the efficacy of immunosuppressive agents in these cases. METHODS: This is a
retrospective analysis of the records of 130 pediatric cases recruited from a
tertiary-care center over a period of two years. They were divided into two
groups, i.e., 51 patients with SRNS (Group I) and 79 cases with SDNS and FRNS
(Group II). They were treated with immunosuppressive agents in addition to
steroids, either as double- or triple-combination therapy. Complete or partial
remission was considered a good response. RESULTS: In group I, the proportions of
good response to cyclophosphamide, cyclosporine A, and mycophenolate mofetil were
48.6, 60, and 80%, respectively (p = 0.162). In group II, the resistance rate was
significantly higher with levamisole than with cyclophosphamide and azathioprine
(p = 0.046). Leukopenia was reported infrequently after the administration of
cyclophosphamide or azathioprine. The most serious adverse reaction was to
cyclosporine A, which induced nephrotoxicity (6.4%), while no adverse effects
were reported to be related to levamisole. Histopathological diagnoses were
available in only 39 patients. CONCLUSION: The high potency of cyclosporine with
steroids makes it useful in patients with idiopathic SRNS with a normal
glomerular filtration rate. Its efficacy is augmented when combined with
mycophenolate mofetil. Cyclophosphamide, orally or as intravenous boluses,
together with alternate-day steroids, could be a good option outside the
peripubertal age. The outcomes with FRNS and SDNS could be improved by
encouraging compliance with the use of levamisole.