Steroid-Responsive Nephrotic Syndrome of Childhood: A Long-Term Study of Clinical Course, Histopathology, Efficacy of Cyclophosphamide Therapy, and Effects on Growth

Berns, Jeffrey S.; Gaudio, Karen M.; Krassner, Leonard S.; Anderson, Frederic P.; Durante, Dennis; McDonald, Bruce M.; Siegel, Norman J.

doi:10.1016/s0272-6386(87)80087-2

Cited by 42 publications

(19 citation statements)

References 27 publications

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“…Patients with MCN have been reported to respond better compared to ones with MesPGN or FSGS, 13 but no such difference was seen among our patients. The reason for this variation is not known and longer follow-up is necessary before any conclusions can be drawn.…”

Section: Discussioncontrasting

confidence: 79%

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: Discussioncontrasting

confidence: 79%

Response to Cyclophosphamide Therapy in Saudi Children with Idiopathic Nephrotic Syndrome

1990

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“…Our use of cyclophosphamide is based on the classic approach to minimal-change nephrotic syndrome, where cyclophosphamide has been shown to have a potent immunomodulating effect, especially in steroid-sensitive patients [23][24][25]. Cyclophosphamide has also been used with success in the treatment of several other forms of crescentic glomerulonephritis, particularly those characterized by altered autoimmunity, including Wegener's granulomatosis and systemic lupus erythematosus [22,26,27].…”

Section: Discussionmentioning

confidence: 99%

Treatment of Henoch-Schönlein Purpura Glomerulonephritis in Children with High-Dose Corticosteroids plus Oral Cyclophosphamide

et al. 2001

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Background: Henoch-Schönlein Purpura (HSP) is a common childhood vasculitis with manifestations in numerous organ systems, including glomerulonephritis. Patients with more severe HSP-associated glomerulonephritis may develop chronic renal failure. Currently, no widely accepted treatment protocols exist for patients with significant renal involvement. Methods: We retrospectively reviewed the clinical courses of 12 children (mean age 9 years) with HSP glomerulonephritis treated with high-dose corticosteroids plus oral cyclophosphamide. All patients had nephrotic-range proteinuria, and all had significant histopathologic changes on biopsy, including crescentic nephritis in 10 patients. Treatment consisted of either intravenous pulse methylprednisolone or oral prednisone followed by oral cyclophosphamide (2 mg/kg/day) for 12 weeks, along with either daily or alternate-day oral prednisone. Prednisone was tapered following completion of cyclophsophamide. Results: Serum albumin rose significantly after treatment from 2.8 ± (SD) 0.5 to 3.7 ± 0.4 g/dl (p < 0.001), and there was a concurrent reduction in proteinuria, as reflected by decreasing serial protein-to-creatinine ratios: from 6.3 ± 4.4 to 0.8 ± 0.8 (p = 0.002). Renal function remained normal in all patients. Hypertension developed during treatment in 10 patients, all but 1 of whom were normotensive at last follow-up, 35 ± 17 months following biopsy. Conclusions: We conclude that treatment of children with HSP nephritis with high-dose corticosteroids plus oral cyclophosphamide is safe and, as in nephrotic syndrome, appears to significantly reduce proteinuria which is a known risk factor for the development of renal insufficiency in HSP. Further studies with larger numbers of patients should be conducted to confirm this finding.

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“…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%

“…In contrast, unfavorable responses to cyclophosphamide treatment in steroiddependent nephrotic syndrome have also been reported (sustained remission rate of 25 and 30%) [19,20]. Berns et al [16] showed that the response to cyclophosphamide was strongly dependent on histologic lesions, while Webb et al [21] stressed that response to cytotoxic therapy was better correlated with initial steroid sensitivity than histology. The natural history of SRNS is for relapses to become less frequent with age [21,22].…”

Section: Discussionmentioning

confidence: 99%

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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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Steroid-Responsive Nephrotic Syndrome of Childhood: A Long-Term Study of Clinical Course, Histopathology, Efficacy of Cyclophosphamide Therapy, and Effects on Growth

Cited by 42 publications

References 27 publications

Response to Cyclophosphamide Therapy in Saudi Children with Idiopathic Nephrotic Syndrome

Response to Cyclophosphamide Therapy in Saudi Children with Idiopathic Nephrotic Syndrome

Treatment of Henoch-Schönlein Purpura Glomerulonephritis in Children with High-Dose Corticosteroids plus Oral Cyclophosphamide

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

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