Cyclophosphamide Treatment in Steroid-Sensitive Nephrotic Syndrome of Childhood

Barratt, T. M.; Osofsky, Stephen G.; Bercowsky, A.; Soothill, J. F.; Kay, Richard

doi:10.1016/s0140-6736(75)91068-5

Cited by 54 publications

(19 citation statements)

References 14 publications

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“…These results are similar to those cited in previous reports 10,11 and support the suggestion that patients with early steroid resistance, also called early nonresponders, should perhaps not undergo cyclophosphamide therapy.…”

Section: Discussionsupporting

confidence: 92%

“…This is a better response compared with the experiences described in some previous reports. 10,11 Results in our patients could be due to the age-related spontaneous decline in susceptibility, as about 60% of our patients were more than 6 years old at the time of therapy. The simultaneous use of prednisolone, which is known to prolong remission, 12 may also have contributed to our better results.…”

Section: Discussionmentioning

confidence: 76%

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Response to Cyclophosphamide Therapy in Saudi Children with Idiopathic Nephrotic Syndrome

1990

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Thirty-three children with idiopathic nephrotic syndrome received an eight-week course of 2.5 to 3.0 mg/kg of body weight of cyclophosphamide. Twenty-five (76%) were in remission 12 to 28 months later (mean, 21.8 months). Those in remission included 77% of the patients with mesangioproliferative glomerulonephritis, 70% of the patients with focal segmental glomerulosclerosis, and 78% of the patients with minimal change nephropathy. In terms of steroid sensitivity, 37.5% of the patients with early steroid resistance were in remission, compared with 100% of the patients with late steroid resistance and 86% of those who were steroid dependent and had frequent relapses. Cyclophosphamide therapy should be considered in patients who become resistant to corticosteroids after an initial response and in those who have frequent relapses and need a toxic dose of steroids to remain in remission. Response to such therapy correlates better with the patient's response to the preceding prednisolone therapy rather than to the renal histology. Children with idiopathic nephrotic syndrome are usually treated with corticosteroids. Some patients who are steroid resistant or have frequent relapses receive additional treatment with an alkylating agent such as cyclophosphamide. The drug is effective in inducing significant remission in steroid-resistant as well as frequent-relapsing steroidsensitive patients.1,2 Apprehension exists about its toxicity, particularly on the male gonads. However, based on the information available, this particular side effect is dose related, and careful use will prevent irreversible gonadal damage.3 Considering the relative risks of frequent relapses or high-dose steroid therapy, the potential benefits of cyclophosphamide therapy perhaps outnumber its possible side effects.In the Kingdom of Saudi Arabia, steroid toxicity is a common occurrence among children with steroid-resistant or frequent-relapsing, steroid-dependent nephrotic syndrome. 4 The purpose of this study was to assess the response of such patients to cyclophosphamide therapy and discuss the related issues.

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Section: Discussionsupporting

confidence: 92%

Section: Discussionmentioning

confidence: 76%

Response to Cyclophosphamide Therapy in Saudi Children with Idiopathic Nephrotic Syndrome

1990

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“…Some of these children are treated with immunosuppressive therapy without a histological diagnosis of their lesion [1][2][3]. Also a biopsy done at the onset or soon after, need not be representative of the morphological lesion present at the time of institution of immunosuppressive therapy since morphological transition has been documented [4].…”

Section: Introductionmentioning

confidence: 99%

Efficacy of Cyclophosphamide in Steroid-Sensitive Childhood Nephrotic Syndrome with Different Morphological Lesions

Tejani¹,

Phadke²,

Nicastri³

et al. 1985

Nephron

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We have reviewed the efficacy of cyclophosphamide in 39 steroid-sensitive frequently relapsing nephrotic children. Cyclophosphamide was used because of heavy steroid dependence and steroid toxicity. A percutaneous renal biopsy done prior to administration of cyclophosphamide showed the lesion to be minimal change in 7 children, IgM nephropathy in 17 children, and evolving from minimal change nephrotic syndrome to focal segmental sclerosis (FSGS) in 15 children. 100% of patients with minimal change nephrotic syndrome responded to cyclophosphamide, but only 58% of IgM patients responded (p < 0.05). Only 1 of 15 FSGS patients responded (p < 0.001 vs. minimal change nephrotic syndrome and p < 0.0l vs. IgM). In view of the failure of cyclophosphamide to produce a remission in FSGS and its potential for long-term impairment of suppressor T cell function, we suggest that it should not be used in patients whose disease has evolved from minimal change to FSGS.

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“…The beneficial effect of cytotoxic treatment in SRNS has long been established [2,3,[13][14][15]. Cyclophosphamide is still recommended for frequently relapsing and steroid-dependent nephrotic syndrome after the development of steroid toxicity [15][16][17].…”

Section: Discussionmentioning

confidence: 99%

“…Cyclophosphamide is still recommended for frequently relapsing and steroid-dependent nephrotic syndrome after the development of steroid toxicity [15][16][17]. Takeda et al [18] compared the long-term effects of three immunosuppressive agents, cyclophosphamide, chlorambucil and cyclosporin A, and concluded that sustained remission was only seen in cyclophosphamide-treated patients.…”

Section: Discussionmentioning

confidence: 99%

Influence of Prolonged Corticosteroid Therapy on the Outcome of Steroid-Responsive Nephrotic Syndrome

Matsukura

Inaba²,

Shinozaki³

et al. 2001

Am J Nephrol

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Eighty-six patients (59 males and 27 females) diagnosed with steroid-responsive nephrotic syndrome during childhood were identified. The patients were 20–40 years of age (mean 27.0 ± 5.0) with a mean follow-up period of 19.5 ± 5.9 years. All patients had been treated with a long-term tapering corticosteroid therapy. Thirty patients had also received a course of cyclophosphamide (2 mg/kg/day for 12 weeks). Sixty-six had achieved sustained remission off corticosteroids, while 20 were still receiving corticosteroids to maintain remission. None of the 86 patients had proteinuria or renal insufficiency at the time of the study. Mean final heights in males and females were similar (–0.51 ± 1.21 and –0.23 ± 1.16 standard deviation score). Mean final height of 20 steroid-dependent patients was significantly less than that of 66 in remission off corticosteroids (p < 0.005). Ten cyclophosphamide-treated patients got married and 9 had at least 1 healthy child. In children with steroid-responsive nephrotic syndrome, the need for corticosteroid therapy to maintain remission may be associated with decreased adult height. Patients who received a 12-week course of cyclophosphamide are likely to be normally fertile as adults.

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Cyclophosphamide Treatment in Steroid-Sensitive Nephrotic Syndrome of Childhood

Cited by 54 publications

References 14 publications

Response to Cyclophosphamide Therapy in Saudi Children with Idiopathic Nephrotic Syndrome

Response to Cyclophosphamide Therapy in Saudi Children with Idiopathic Nephrotic Syndrome

Efficacy of Cyclophosphamide in Steroid-Sensitive Childhood Nephrotic Syndrome with Different Morphological Lesions

Influence of Prolonged Corticosteroid Therapy on the Outcome of Steroid-Responsive Nephrotic Syndrome

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