Short versus long initial prednisone treatment in steroid-sensitive nephrotic syndrome in children

A total of 184 children aged, 13 months to 11 years, suffering from their first attack of steroid-responsive nephrotic syndrome were included in a randomized study. They were treated according to three treatment protocols. All children received l-2mg of prednisone/kg body weight/day (up to 80 mg daily) for 4 weeks, and thereafter 1 mg/kg body weight/48 h for the next 4 weeks. Treatment was discontinued at this point in 44 children (protocol A); in 68 (protocol B) the dose was reduced by 25% each week, tapering off to 0 at the end of the third month, while in 72 children (protocol C), after the first 2 months of initial treatment the dose was reduced by 25% each month and tapered off to 0 by the end of the sixth month. All patients completed a 2-year follow-up period after withdrawal of prednisone. Treatment results were expressed as: percentage of children relapse-free within the first 6 months and 2 years after withdrawal of treatment, and average number of relapses per patient per year. The best results were obtained in children who had been treated for 6 months: 65.3% of them remained relapse-free within the first 6 months and 50% over the entire 2-year follow-up period; the number of relapses per patient per year in this group was 0.49. The respective values for children treated 2 and 3 months were: 36.4% and 32.4% for the 6-month period; 27.3% and 20.6% for the 2-year period; the numbers of relapses per patient per year were 0.79 and 0.77, respectively. The frequency of corticosteroid side effects such as transient hypertension and cushingoid obesity during the initial treatment, and growth retardation or recurrent "respiratory tract" infections observed during the following 2-year follow-up period, did not increase after prolongation of the initial treatment. Initial treatment period, steroid-sensitive nephrotic syndrome
J Ksiek, Department of Nephrology, Child Health Centre, 04-736 Warsaw, Poland     

Alternate-day prednisone is more effective than intermittent prednisone in frequently relapsing nephrotic syndrome

In a multicenter cooperative study the effectiveness and side-effects of two most widely used regimens for prolonged interrupted prednisone treatment were compared in children with frequently relapsing nephrotic syndrome: i.e., alternate-day prednisone vs. intermittent prednisone. Sixty-four children were admitted to the study, 30 of whom were allocated to an alternate-day, 34 to an intermittent group. Sixteen patients did not complete the full trial, which left 48 children for final evaluation (23 alternate-day, 25 intermittent). The protocol consisted of two 6-month periods. During the first 6 months patients received maintenance prednisone (alternate-day = 35 mg/m²/48 h, intermittent = 40 mg/m² on 3 out of 7 days). During the second 6-month pericd no maintenance prednisone was administered unless a relapse occurred and was treated with a short course of prednisone. The alternate-day prednisone reduced the number of relapsers and the rate of relapses significantly as compared with the control period of the second 6 months. The intermittent prednisone, however, did not significantly lower the number of relapsers, but only the rate of relapses. In the alternate-day group the number of relapsers and the rate of relapses were significantly lower than in the intermittent group. Observation for toxic side-effects did not reveal any difference. It is concluded that an alternate-day regimen is preferable to the intermittent regimen, which should be abandoned in the treatment of children with the nephrotic syndrome.Supported by grants of VW FoundationParticipating centers: Basle, Switzerland: Kinderspital (F. Egli) Berlin-West: Universitaets-Kinderklinik Gesamthochschule (H. Olbing, H. J. Bachmann) Frankfurt a. M.: Universitaets-Kinderklinik (J. Dippell) Freiburg i. Br.: Universitaets-Kinderklinik (F. Schidera) Hamburg: Universitaets_Kinderklinik (F. Bläker, H. Altrogge) Hannover: Kinderklinik der Medizinischen Hochschule (J. Brodehl, H.-P. Krohn) und Kinderhielanstalt (J. Natzschka) Heidelberg: Universitaets-Kinderklinik (K. Schärer, D. Müller-Wiefel) Homburg: Universitaets-Kinderklinik (D. Krämer) Munich: Universitaets-Kinderklinik (R. Joppich) Münster: Universitaets-Kinderklinik (L. Diekmann) Stuttgart: Olgaspital (W. Hagge) Pathologist: W. Thoenes (Mainz) Statistician: B. Schneider (Hannover) Central office: Hannover, Kinderklinik der Medizinischen Hochschule (J.B)     

Comparison of different regimens of prednisone therapy in frequently relapsing nephrotic syndrome

The long-term results of four different regimens of prednisone therapy were compared in 32 children with steroid sensitive, frequently relapsing idiopathic nephrotic syndrome with minimal glomerular lesions on renal biopsy. Prednisone was administered according to the following dosage schedules: 1) long-term daily, 2) standard intermittent, 3) standard alternate-day, and 4) short-term daily. Over a mean observation period of 7 years patients without steroid dependency received 19 mg/m2/day. Relapse free intervals were the longest with long-term daily prednisone therapy compared to the other three regimens. In frequently relapsing patients without steroid dependency the relapse free intervals were similar with either intermittent or alternate-day prednisone therapy (median 75d); however, they were significantly shorter with short-term prednisone therapy (median 33d). In frequently relapsing patients with steroid dependency the time of remission was generally shorter than in patients without steroid dependency (median 25d vs. 69d) with no benefit of any of the different forms of short-term treatment.     

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??Objective To observe the effect of mizoribine??MZR?? in the treatment of children with frequently relapsing primary nephrotic syndrome. Methods Totally 21 children with frequently relapsing nephrotic syndrome were treated with mizoribine?? and another 24 children were included as the control group. Observe the changes of relapse number??dosage of prednisone?? glomerular filtration rate?? serum albumin?? urine protein?? uric acid and other indicators. Results After the treatment with mizoribine??the recurrence rate and the effective dosage of prednisone decreased?? serum albumin level rose?? and primary urinary protein decreased?? but there were no significant changes in glomerular filtration rate and uric acid level. Conclusion The mizoribine is effective in treatment of children with frequently relapsing primery nephrotic syndrome .     

Efficacy of levamisole in children with frequently relapsing and steroid-dependent nephrotic syndrome

Objectives

To assess the efficacy of levamisole in frequently relapsing nephrotic syndrome and steroid-dependent nephrotic syndrome.

Study Design

Retrospective analysis of hospital case records.

Setting

Pediatric nephrology department of a tertiary referral pediatric hospital.

Participants

62 children with frequently relapsing nephrotic syndrome and 35 children with steroid-dependent nephrotic syndrome.

Methods

Case records of children who were diagnosed as steroid-dependant or frequently-relapsing nephrotic syndrome from June 2004 to June 2011, were reviewed. Levamisole was given daily (2 mg/kg/d) along with tapering doses of alternate day steroids after remission on daily steroids.

Results

Levamisole was effective in 77.3% children with a better (80.6%) efficacy in frequently relapsing nephrotic syndrome. A total of 34 children completed 1 year follow-up post levamisole therapy. The cumulative mean (SD) steroid dose 1-year before therapy was 4109(1154) mg/m² and 1-year post therapy was 661 (11) mg/m² (P<0.001). The relapses were also less during the period of post-levamisole therapy.

Conclusion

Levamisole is an effective alternative therapy in frequently relapsing and steroid-dependent nephrotic syndrome.     

Persistent hypercholesterolaemia in frequently relapsing steroid-responsive nephrotic syndrome

Objective: To investigate long-term changes of serum cholesterol levels in children with frequently relapsing steroid-responsive nephrotic syndrome (NS).
Methodology: Serum cholesterol values just before and during or immediately after 'relapse' were reviewed and the incidence of hypercholesterolaemia (≥200 mg/dL) was determined in eight patients (M:F, 6:2).
Results: The patients with frequently relapsing NS usually showed hypercholesterolaemia (mean incidence, 81%) just before 'relapse' during clinical remission, as well as in relapse (mean incidence, 96%). A high incidence of steroid therapy was also found in each case (mean, 89%) just before relapse.
Conclusions: Our results demonstrate that children with frequently relapsing NS have prolonged periods of hypercholesterolaemia, even during clinical remission. It is suggested that serum lipid profiles be monitored carefully in such patients.     

Immunoregulation with levamisole in children with frequently relapsing steroid responsive nephrotic syndrome

The immunological and clinical effects of levamisole were studied in 10 children with frequently relapsing steroid responsive nephrotic syndrome (SRNS). The efficacy of the drug was tested during remission of the disease with all patients on alternate day steroid therapy. The lymphocyte proliferative response to phytohemagglutinin (PHA), concanavalin-A (Con-A) and pokeweed mitogen (PWM) were normal. The Con-A induced suppressor T-lymphocyte activity of 7 patients was low before treatment with levamisole 8 +/- 3.7% and increased to normal values during therapy 34 +/- 6%; p less than 0.001 (control 32 +/- 5%). In these 7 children prednisolone dosage could be decreased significantly or discontinued altogether (44.1 +/- 5.3%). Patients without immunoregulatory abnormalities did not respond to levamisole. In 3 out of 4 children tested the percentage of OKT8+ cells rose during levamisole therapy from 19.7 +/- 2.1 to 37 +/- 2.3 (p less than 0.001), thus correcting the elevated pre-treatment OKT4+/OKT8+ ratio from 3.1 +/- 0.2 to 1.5 +/- 0.2; p less than 0.001 (control 1.47 +/- 0.2). These data support the hypothesis that abnormal immunoregulation may play a role in the pathogenesis of SRNS. Treatment with levamisole can be useful in some patients with the frequently relapsing form of the disease.     

Mycophenolate sodium for children with frequently relapsing or steroid dependent nephrotic syndrome

In this retrospective study, patients with idiopathic frequentlyrelapsing nephrotic syndrome (FRNS) (n=27) and steroid dependent nephrotic syndrome (SDNS) (n=13) who received enteric coated mycophenolate sodium (ECMS) for at least 6 months, were included for analysis. Primary outcome was response to ECMS, which was defined as complete if there were no relapses, partial response if there was 1 relapse and no response if there were 2 or more relapses within 6 months of initiation. The mean (SD) dose of ECMS was 985.24 (190.82) mg/m²/day. Thirty patients(75%) had complete response, eight (20%) had partial and two (5%) patients did not respond at 6 months. ECMS seems to be a safe and effective as steroid sparing agent in children with FRNS/SDNS.     

Final height in children with steroid-sensitive nephrotic syndrome

BACKGROUND: Growth retardation following steroid treatment in children is a major problem. Reduction of steroid dose has been tried using immunosuppressive agents such as cyclosporine A or mizoribine in children with frequently relapsing nephrotic syndrome. Few reports concerning final height in steroid-sensitive nephrotic syndrome (SSNS) are available. METHOD: Patients who developed SSNS before 15 years of age and reached their final height were retrospectively studied by standard deviation score (SDS) of height and target height calculated by their parental height. RESULTS: A total of 34 patients were evaluated for their final height. The mean age at onset of SSNS was 8.0 years and the mean age at last follow up was 21.6 years. In total, 22 patients had frequent relapses and were treated with cyclophosphamide, mizoribine or cyclosporin A. All patients had normal renal function at the last evaluation. The mean final height was 168 cm in males and 155 cm in females. The mean height SDS was 0.37 at the time of onset and was -0.43 when they reached their final height (P = 0.0001). The final height was a mean of 2.5 cm below target height and was significantly lower than their siblings (P = 0.007). Final height of two boys who continued to have frequent relapses during puberty and were not treated with cyclosporin A was 146 and 150 cm. CONCLUSION: Final height in children with SSNS was slightly affected by steroid treatment and two patients had severe growth retardation.     

频复发/激素依赖肾病综合征儿童利妥昔单抗治疗1年以上随访情况的系统评价/Meta分析

背景：权威指南和共识均推荐将利妥昔单抗（RTX）应用于儿童激素敏感型肾病综合征（SSNS）中频复发/激素依赖肾病综合征（FRNS/SDNS）的治疗,但仍存在临床适应证不统一、治疗和随访方案多样等问题。目的：了解RTX首疗程治疗缓解期FRNS/SDNS随访1年以上复发和激素使用情况结局。设计：系统评价/Meta分析。方法：检索PubMed、Embase、Cochrane、Scopus和中国生物医学文献服务系统数据库,从建库至2022年6月26日,以SSNS、FRNS、SDNS和 RTX构建中英文数据库检索式。同一篇文献初筛、全文筛选和证据提取均由2人完成,有争议和不确定的文献由第3人复核审查。纳入至少1组干预措施使用RTX治疗1~22岁SSNS患儿的研究。主要结局指标:RTX干预后随访≥1年的复发率、首次复发时间,激素累积剂量和停用比例。结果:符合本文临床结局的文献26篇（RCT 8篇、非随机对照试验 1篇、队列研究8篇、病例系列报告9篇）,中文文献1篇,英文文献25篇。基于FRNS/SDNS病例的随访≥1年复发率的9项研究的Meta分析显示,RTX较对照组复发率下降了78%(OR=0.22,95%CI:0.09~0.53),在FRNS/SDNS+（RTX干预前已使用其他免疫抑制剂）亚组病例中,RTX较对照组复发率下降了67%(OR=0.33,95%CI:0.12~0.94),在FRNS/SDNS-（RTX干预前未使用其他免疫抑制剂）亚组病例中,RTX-（不联用其他并免疫抑制剂）较对照组复发率下降了85%(OR=0.15,95%CI:0.03~0.68)。基于20项研究的Meta分析显示,RTX复发率42% (95%CI:32%~53%)。基于FRNS/SDNS+随访≥1年首次复发时间的9项研究的Meta分析显示,首次复发时间9.89（95%CI: 7.14~12.65)月。基于FRNS/SDNS-开始干预至随访≥1年中位首次复发时间的3项研究的 Meta分析显示,RTX（1~2剂）较对照组中位首次复发时间长20 d,中位生存比（MSR）为0.69(95%CI:0.52~0.87）。基于FRNS/SDNS的12个月激素累积剂量减少结局的4项研究的Meta分析显示,RTX较对照组年激素累积剂量减少明显,差异有统计学意义(SMD=-1.12,95%CI:-1.49~-0.74)。基于FRNS/SDNS的随访3个月激素停用率的2项研究的Meta分析显示,RTX是对照组（CNI或CTX）随访3个月激素停用率的14.6倍 (OR=14.62,95%CI:5.43~39.39)。基于FRNS/SDNS+的RTX治疗6个月停用激素率的3项研究的Meta分析显示,停用激素率68%（95%CI:56%~79%）。结论:与对照组相比,RTX从随访1年的首次复发时间中获益有限,可从激素减量中获益但不能从停用激素率中获益。RTX治疗FRNS/SDNS随访12个月较安慰剂治疗或空白对照至少可降低88%的复发率,FRNS/SDNS接受RTX治疗随访1年复发率43%。RTX治疗FRNS/SDNS+可获得10个月的无复发生存时间。     

Bone mineral density in children with steroid-sensitive nephrotic syndrome

Objective  

To observe the influence of prednisolone treatment on bone mineral density (BMD) in children with idiopathic nephrotic syndrome.     

环孢素A治疗儿童不同病理类型肾病综合征83例的疗效观察

目的　研究环孢素A(CyA)治疗儿童不同病理类型肾病综合征的临床疗效及意义。方法　 83例肾病综合征患儿入院后逐渐减用激素 ,给予口服CyA ,剂量 5mg/ (kg·d) ,疗程 3～ 6个月 ,并监测血浓度调整CyA的剂量。结果　 83例患儿经治疗后 ,尿蛋白转阴者 4 5例 (完全缓解率 5 4 % ) ,尿蛋白减少者 2 3例 (部分缓解率 2 8% ) ,未缓解 15例 (18% ) ;总有效率达 82 %。不同病理类型治疗反应 :微小病变型肾病有效率为 86 % ,系膜增殖性肾小球肾炎为 84 % ,膜增殖性肾小球肾炎为 3/ 5 ,局灶节段性肾小球硬化为 2 / 4。显效时间为 7～ 4 5d ,其效应多出现于用药 1个月内。服药后分别于 1周和 2周末 ,测定CyA的血药浓度 ,有效血浓度维持在 10 0～ 2 0 0 μg/L ,可使大部分患儿病情顺利缓解 ,疗程一般在 3～ 6个月。 83例患儿都进行了随访 ,其中 6 8例经CyA治疗缓解后的 17例在减量或停药后出现复发 ,复发率为 2 5 % ,复发的患儿重新服用CyA仍然有效。治疗过程中 5例患儿出现一过性尿肌酐的增加 ,8例尿N 乙酰 β D 氨基葡萄糖苷酶轻微增加 ,一般减量或停药后可逆转。 结论　CyA是替代皮质激素治疗难治性肾病的较好方法之一 ,能有效而快速达到治疗难治性肾病的目的 ,其治疗效果与有效的血药浓度和病理类型有关     

Distribution of memory B cell subsets in peripheral blood of children with frequently relapsing nephrotic syndrome北大核心CSCD

目的观察记忆B细胞在频复发肾病综合征(frequently relapsing nephrotic syndrome,FRNS)患儿病程中分布变化。方法前瞻性选择2020年10月—2021年10月就诊于徐州医科大学附属医院儿科的原发肾病综合征(primary nephrotic syndrome,PNS)患儿35例,根据其糖皮质激素(glucocorticoid,GC)治疗后的反应及复发频次,分为FRNS组、非频复发肾病综合征(non-frequently relapsing nephrotic syndrome,NFRNS)组;选择同期15例体检儿童为健康对照组。比较各组GC治疗前后记忆B细胞变化,并与临床指标作相关性分析。结果治疗前,FRNS组、NFRNS组总B细胞、总记忆B细胞、IgD^(+)记忆B细胞、IgE^(+)记忆B细胞比例均较健康对照组增高,且FRNS组较NFRNS组增高明显(P<0.05);FRNS组类别转换记忆B细胞比例较NFRNS组及健康对照组降低(P<0.05);治疗后,FRNS组、NFRNS组总B细胞、总记忆B细胞、IgM^(+)IgD^(+)记忆B细胞、IgM^(+)记忆B细胞、IgE^(+)记忆B细胞、IgD^(+)记忆B细胞、IgG^(+)记忆B细胞比例较治疗前降低(P<0.05);类别转换记忆B细胞比例较治疗前增高(P<0.05)。FRNS组尿蛋白定量高于NFRNS组及健康对照组(P<0.05),FRNS组白蛋白水平低于健康对照组(P<0.05);FRNS组尿蛋白定量与类别转换记忆B细胞比例呈负相关,与IgE^(+)记忆B细胞比例呈正相关(P<0.05)。结论FRNS患儿存在记忆B细胞亚群的分布异常;而IgE^(+)记忆B细胞和类别转换记忆B细胞比例可作为FRNS患儿在GC治疗后复发的正相关和负相关因素。     

Multiple drug-resistant gene 1 in children with steroid-sensitive nephrotic syndrome

Background: A full dose of corticosteroid is required to induce complete remission (CR) in steroid‐sensitive nephrotic syndrome (SSNS), unless it is possible to taper and discontinue along with the course after CR. But the mechanism of this change in steroid sensitivity remains unknown. P‐glycoprotein (PGP) can eliminate given corticosteroids from cytoplasm, which results in corticosteroid resistance. Therefore, drug delivery was analyzed using real‐time polymerase chain reaction (PCR) of multiple drug‐resistant gene 1 (MDR1; encoding PGP) mRNA expression. Methods: Fourteen patients with SSNS (male/female: 14/0; age: 1–23 years; mean 10.4 years) were enrolled in the study. MDR1 mRNA gene expression of peripheral blood nucleated cells (PBNC), before and after CR (19 sets of blood samples), was quantified using real‐time PCR. Results: The MDR1 mRNA levels before CR were variable in each patient, but there was an apparent decrease in the MDR1 gene expression of PBNC after CR (P < 0.003). Conclusion: PGP may play a role in the tapering of corticosteroids after CR in SSNS.     

Cyclophosphamide therapy in frequently relapsing nephrotic syndrome with and without steroid dependence

An 8-week therapy with cyclophosphamide (CP) and alternate-day prednisone was given to 65 patients having steroid-sensitive, frequently relapsing nephrotic syndrome, including 17 with steroid dependence. It induced remissions of less than 6 months in 18 patients, 6 to 36 months in 21 and over 3 years in 26. Considering a remission of 6 months as significant, certain clinical variables were compared in patients without such a response with those getting longer remissions. In the group with frequent relapses a higher proportion of patients aged above 8 years had remissions of over 6 months as well as over 3 years, than those who were younger at CP therapy. Among patients with frequent relapses as well as those with steroid dependence, a post-CP remission of more than 6 months was associated with a better long-term course. The response to CP in both groups was similar regarding the duration of remissions, but a greater proportion of steroid-dependent patients subsequently again showed steroid dependence or frequent relapses. Our findings suggest that a higher age at CP therapy and an ensuing remission of over 6 months are predictors of a better response, and steroid dependence of a less favorable outcome.     

Chlorambucil dosage in frequently relapsing nephrotic syndrome: a controlled clinical trial

A controlled clinical trial was performed using two dosage regimens of chlorambucil to treat children with frequently relapsing nephrotic syndrome. All children concurrently received prednisone (60 mg/m2 on alternate days). Ten children (Group I) were given chlorambucil as a stable dose (0.2 mg/kg/day) for 56 to 60 days, and 11 children (Group II) received increasing doses (0.2 to 0.63 mg/kg/day) for 42 to 77 days. Two children in each group subsequently relapsed. Follow-up averaged 28.6 and 27.2 months in Groups I and II, respectively. Three children in Group II developed infectious complications. The data indicate that a stable dosage regimen for chlorambucil is as effective as an increasing dose regimen in achieving long-term remission of frequently relapsing nephrotic syndrome.     

Evaluation and management of steroid-sensitive nephrotic syndrome

PURPOSE OF REVIEW: This review examines new literature published in 2006 and 2007 on steroid-sensitive nephrotic syndrome. RECENT FINDINGS: Steroid-sensitive nephrotic syndrome has long been thought to be due to lymphocyte-derived circulating factors leading to podocyte injury with subsequent proteinuria. New studies support this mechanism and implicate the T helper 2 cytokine IL-13. In addition a genetic mutation in familial nephrotic syndrome has been reported in a child, who responded to corticosteroid therapy. There are new clinical trial data supporting the efficacy of levamisole in steroid-sensitive nephrotic syndrome and preliminary trial data on mycophenolate mofetil supporting its efficacy as a steroid-sparing agent. Case reports support the use of the B cell-depleting antibody rituximab in steroid-sensitive nephrotic syndrome. Finally there is a meta-analysis of six studies suggesting an increase in the incidence of focal and segmental glomerulosclerosis in steroid-sensitive nephrotic syndrome over the last 20 years. SUMMARY: Progress has been made towards elucidating the cause of steroid-sensitive nephrotic syndrome. Data from adequately powered randomized controlled trials are still required to evaluate therapies for frequently relapsing and steroid-dependent steroid-sensitive nephrotic syndrome.     

Long versus standard prednisone therapy for initial treatment of idiopathic nephrotic syndrome in children

Two regimens of steroid treatment for the initial attack of idiopathic nephrotic syndrome (NS) in children were compared in a controlled prospective multicentre study. Long prednisone therapy consisted of 60 mg/m² per 24 h for 6 weeks, followed by alternate day 40 mg/m² per 48 h for 6 weeks. The standard prednisone therapy was 60 mg/m² per 24 h for 4 weeks, followed by 40 mg/m² per 48 h for 4 weeks. A total of 71 children with an initial attack of idiopathic NS were allocated at random to the two groups. The cumulative rate of patients with sustained remissions after 2 years was significantly higher after the long course than after the standard treatment (49% vs 19%,P=0.0079). The mean relapse rate per patient at intervals of 3, 6 and 12 months was lower in the long-course prednisone group than in the standard prednisone group, and the proportion of children with frequent relapses during any subsequent 6 months period was lower in the long-course group than in the standard group (29% vs 57%,P=0.03). Mild side-effects of corticosteroid therapy were observed more frequently after long-course prednisone treatment. It is concluded that long-course prednisone therapy of the initial attack of steroid responsive NS is preferable to the standard regimen because it reduces the rate of subsequent relapses without increasing the risk for severe steroidal side-effects. Contributing investigators and centres were: Prof. F. R. Egli (Basel, Switzerland); Prof. G. Mau, Dr. J. Zimmermann (Braunschweig, Germany); Dr. W. Marg (Bremen, Germany); Dr. R. Mallmann (Bonn, Germany); Dr. K. Witzel (Düsseldorf, Germany); Prof. D. Michalk (Erlangen, Germany); Prof. H. Olbing (Essen, Germany); Dr. E. Bopp (Flensburg, Germany); Prof. J. Dippel (Frankfurt, Germany); Dr. H. Zappel (Göttingen, Germany); Dr. D. Schwarke (Hamburg, Germany) Prof. J. Brodehl (Hannover, Germany); Prof. K. Schärer (Heidelberg, Germany); Prof. F. Schindera (Karlsruhe, Germany); Dr. M. Kirschstein (Lübeck, Germany); Prof. H. P. Weber (Lüdenscheid, Germany); Prof. M. Brandis (Marburg, Germany); Prof. R. Eife (München, Germany); Dr. F. K. Hübner (München, Germany); Dr. K. Gellissen (Neuwied, Germany); Prof. W. Rauh (Trier, Germany).     

Cyclosporin therapy in steroid-dependent nephrotic syndrome

Abstract Five children with multiple relapsing steroid-dependent nephrotic syndrome were treated with continuous cyclosporin for periods ranging from 18 to 48 months. Renal biopsy showed mild mesangial proliferation in three of the children and minimal change in two. All children previously had been treated with cyclophosphamide. Cyclosporin was started during remission at 5 mg/kg per day. If a relapse occurred the dose was increased until a trough blood level of 100–250 ng/mL (HPLC) was achieved. In the initial 12 months of treatment, the mean number of relapses decreased from 6.4±0.54(s.d.) per annum to 1.6±1.3 per annum (P<0.01). Cyclosporin was effective in maintaining long-term remission in four of the five patients. Side effects included hypertrichosis (5) and gum hyperplasia (1). The mean creatinine clearance decreased from 126±16 to 97=22 mL/min per 1.73 m² (P = NS). A renal biopsy in all five patients after 12 months therapy showed no nephrotoxicity. A further biopsy in one patient after 4 years therapy showed interstitial fibrosis. Cyclosporin should be considered in children with steroid-dependent nephrotic syndrome who show signs of steroid toxicity and have only a short remission period after cyclophosphamide. Serial renal biopsies are recommended if prolonged therapy is used.