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.     

激素抵抗型肾病环孢霉素受体的研究

目的探讨激素抵抗型肾病患儿血白细胞环孢素受体的表达和临床意义及介导免疫抑制的机理。方法采用逆转录聚合酶链反应(RT-PCR)测定23例激素抵抗型肾病患儿血白细胞中环孢素受体的表达,并与正常儿童作对照。结果不同病理类型激素抵抗型肾病患儿中,系膜增殖性肾小球肾炎血白细胞中环孢素受体呈高表达,微小病变型肾病、膜增殖性肾小球肾炎、局灶节段性肾小球硬化血白细胞中环孢素受体表达差异无显著性。激素抵抗型肾病患儿急性期血白细胞中环孢素亲和素(CyP)mRNA水平高于恢复期与对照组。激素抵抗型肾病患儿以环孢素5mg/(kg·d)口服治疗10～45d后,56.5%完全缓解,17.4%部分缓解。当环孢素血浓度在100～200μg/L范围、环孢素受体呈高水平表达时,激素抵抗型肾病患儿易引起临床缓解。结论激素抵抗型肾病患儿高水平环孢素受体表达时,可给予合适剂量的环孢素A(CyA)治疗;测定环孢素受体表达水平,对肾病的治疗有指导意义。     

Autoimmune thyroiditis in a child with steroid-dependent nephrotic syndrome

Autoimmune thyroiditis is rarely described in association with nephrotic syndrome. Herein we report a girl who developed autoimmune thyroiditis insidiously during the course of minimal change nephrotic syndrome. She was streroid-sensitive, but developed severe steroid dependency and did not respond to cyclophosphamide therapy. She went into stable remission with levamisole. Five months after introduction of levamisole a mild goiter was found on systematic examination at school. The diagnosis of autoimmune thyroiditis was established with typical ultrasound appearance of the thyroid gland along with significant titers of antithyroid antibodies. It is very unlikely that levamisole was responsible for thyroiditis because experimental animal administration of high doses of levamisole inhibited lymphocyte infiltration of the thyroid. Since levamisole has had a beneficial effect on the nephrotic syndrome in our patient we decided to continue the treatment. She has been receiving levamisole for 3 years, and no adverse effects have been observed during the treatment period.     

小儿肾病综合征的冲击疗法

研究目的探讨小儿肾病综合征的治疗方法。研究方法小儿肾病综合征患儿共６６例，随机分为两组：冲击组（３４例）和对照组（３２例）。冲击组用地塞米松（１．５－３）ｍｇ／（ｋｇ．ｄ）加入１０％ＧＳ溶液（１００－１５０）ｍｌ静滴，每日１次，连用３ｄ，第４日停用，第５日开始隔日１次，共用６次。次日开始口服强的松（１．５－２）ｍｇ／（ｋｇ．ｄ），共４周，后渐减量。对照组仅用强的松，用法用量同冲击组。结果冲击完全缓     

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??Abstract??Objective To investigate the clinical efficacy and safety of tacrolimus ??FK506?? therapy for children with steroid-dependent and steroid-resistant nephrotic syndrome ??NS??. Methods From Jun.2007 to Dec.2011 in Guangzhou Medical College Guangzhou First Municipal People's Hospital??42 children with primary nephrotic syndrome were enrolled??in which 32 cases were steroid-dependent NS ??SDNS?? and 10 cases were steroid-resistant NS ??the SRNS??. The indicators were tested before treatment and 12 weeks later??including ??1?? the 24h urinary protein excretion??blood urea nitrogen ??BUN????serum creatinine ??Scr????plasma albumin ??Alb????blood and urine ??2-microglobulin ????2-MG??????2?? lipid testing indicators??total cholesterol ??TC????triglyceride ??TG??????3??hypercoagulable state indicators??the prothrombin time ??PT????activated partial thromboplastin time ??APTT ????the plasma concentration of fibrinogen ??Fib?? and blood D-dimer ??D-dimer??. Results Tacrolimus combined with steroid therapy had significant effects.The clinical and biochemical indicators improved significantly after treatment ??P??0.05 or P??0.01????lipids and hypercoagulability-related indicators also improved significantly??proteinuria significantly reduced.Complete remission was achieved in 29 cases??partial remission in 10 cases??no remission in 4 cases.Eleven cases of simple type NS got complete remission??and 18 cases of nephritis type NS got complete remission??partial remission in 9 cases??no remission in 4 cases.Pathological type??5 cases of minimal change disease??complete remission????5 cases of focal segmental glomerulosclerosis??3 no remission??2 partial remission????six cases of mesangial proliferative glomerulonephritis??4 complete remission??2 partial remission??.Mild gastrointestinal reaction occurred in 4 cases and diarrhea in 2 cases.Conclusion The tacrolimus and steroid combination therapy for children with steroid-dependent and steroid-resistant NS can reduce proteinuria??reduce high cholesterol and improve the hypercoagulable state??the majority of children get complete remission??steroid-resistant nephrotic syndrome also has a significant improvement in the treatment.This combined treatment is safe and effective with fewer adverse reactions.     

Recent advances in minimal change nephrotic syndrome

The etiology of MCNS remains an enigma. Increase in the permeability of the glomerular capillary filter to plasma proteins results in massive proteinuria and hypoalbuminemia which are the hallmarks of this disease. The cause of this increased permeability remains to be ascertained. Research in pursuit of this goal has led to the accumulation of fragmentary data on the morphological abnormalities, immunological dysfunction and metabolic changes that accompany this disease. In our experience, immunology dysfunctions, abnormalities of lipid profile and the deposition of IgM in the mesangium as detected by immunofluorescent microscopy are some of the unusual features of MCNS. The therapeutic implications of these findings and some of the other recent advances are discussed in this article.     

Mycophenolate mofetil therapy for children with intractable nephrotic syndrome

BACKGROUND: Cyclosporin A (CyA) can suppress relapses and reduce proteinuria in frequent-relapse nephrotic syndrome (FRNS) and steroid-resistant nephrotic syndrome (SRNS). However, some patients remain resistant to CyA therapy. The purpose of the present paper was to evaluate mycophenolate mofetil (MMF) treatment in pediatric patients with CyA-resistant intractable nephrotic syndrome. METHODS: MMF therapy was given to 11 patients with FRNS who had relapse despite CyA therapy, and one patient with SRNS who had been receiving combined therapy using steroid and CyA until immediately before the start of MMF. MMF was administered at a daily dose of 750-1000 mg/m(2) in two divided doses. RESULTS: Ten of the 11 patients with FRNS were able to maintain remission. Among them, seven patients remained relapse free for 1 year, and two patients had a decrease in the frequency of relapse after initiation of MMF therapy. One patient, however, had repeated cycles of remission and relapse, and was considered resistant to MMF therapy. The total prednisolone dose during the period from month 6 to month 12 after the start of MMF therapy was significantly lower than that during the 6 month period before the start of MMF therapy. The patient with SRNS, who had not achieved remission despite CyA administration, had complete remission on MMF. No serious adverse effects were seen in any of the present patients. CONCLUSION: MMF could be useful in CyA-treatment-refractory FRNS and CyA-resistant SRNS.     

Characteristic phenotype in congenital nephrotic syndrome

We present three children with congenital nephrotic syndrome and previously undescribed facial features. These include: (1) small mouth (intercommissural distance mouth (intercommissural distance <3rd percentile); (2) tented upper lip; (3) small nose (interalar distance –2 SD below mean); (4) fullness of cheeks giving a jowly appearance; (5) overhanging outer thirds of upper eyelids. These features may reflect prolonged tissue oedema and protein malnutrition in utero and early life.Conclusion Characteristic facial features in congenital nephrotic syndrome may reflect intra-uterine malnutrition.     

环磷酰胺静脉冲击治疗儿童激素依赖性肾病综合征32例疗效分析

目的　了解大剂量环磷酰胺静脉冲击疗法 (CTX PT)治疗儿童激素依赖性肾病的近期疗效以及影响疗效的相关因素。方法　总结 1991年 9月至 2 0 0 0年 6月接受CTX PT治疗的激素依赖性肾病 3 2例 ,并就其疾病类型、临床观察指标等与疗效的关系进行了分析。结果　①用CTX PT治疗 2 3例有效 (有效率 71 9% )。②影响疗效的因素主要有疾病的临床、病理类型以及血清肌酐 (SCr)水平。单纯性肾病疗效明显好于肾炎性肾病(有效率分别为 85 0 %和 5 0 0 % ,P<0 0 5 ) ;病理表现为微小病变 (MCNS)者明显好于非MCNS ;SCr水平在治疗前较高者提示可能疗效也差。CTX PT疗效与性别、发病年龄、血清白蛋白、球蛋白、血胆固醇、免疫球蛋白、2 4h尿蛋白排泄量等无关 (P >0 0 5 )。③毒副作用 :11例 (3 4 4% )未见明显CTX毒副作用 ;2 1例 (65 6% )出现不同程度的副作用 ,主要为急性胃肠道反应占 40 6% (3d内缓解 ) ,其它副作用少见 ,而且这些毒副作用均为一过性并很快恢复正常。结论　CTX PT治疗儿童激素依赖性肾病有效率为 71 9% ,影响疗效的因素主要有疾病的临床、病理类型以及SCr水平等。     

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).     

Congenital nephrotic syndrome with clinical hypothyroidism

A 15 month old boy with typical features of congenital nephrotic syndrome (CNS) is reported, who in addition to the renal pathology had an associated clinical hypothyroidism with low levels of total and free thyroxine and triiodothyronine and an elevated serum TSH. Improvement in the physical parameters and mental status from thyroid hormone replacement therapy is documented.     

Childhood idiopathic nephrotic syndrome in Turkey

BACKGROUND: It has been reported that there are racial and regional differences in peak incidence age, histopathological features and response to steroid therapy in childhood idiopathic nephrotic syndrome. METHODS: One hundred and thirty-eight patients with a diagnosis of idiopathic nephrotic syndrome, followed up in 1994-2000, were assessed retrospectively. The aim of this study was to assess the patients' response pattern to steroid therapy, to determine whether the duration of the initial steroid therapy alters the steroid response pattern of the disease and to assess renal biopsy results. RESULTS: One hundred and fourteen patients who initially received only steroid therapy and were followed up regularly were classified according to response pattern. Of the 114 patients, 30 children had an initial response, 25 children had infrequent relapse, 19 had frequent relapse, 25 had steroid dependence and 15 children had steroid resistance. The 99 patients with steroid responsive nephrotic syndrome were divided into two groups with respect to duration of the initial steroid therapy. There was no statistically significant difference between standard and short therapy groups with respect to the steroid response patterns. Percutaneous renal biopsy was performed in 43 of the 138 patients. Mesengioproliferative glomerulonephritis was the most common histopathological lesion, followed by membranoproliferative glomerulonephritis. The proportions of membranous glomerulonephritis, focal segmental glomerulosclerosis and minimal change nephrotic syndrome were low in our group. CONCLUSIONS: Our study group is similar to one reported from Saudi Arabia with respect to the steroid response pattern and to Saudi Arabian and Nigerian reports with respect to the histopathology. Although it has been reported that short initial steroid therapy was followed by a higher rate of relapses, there was no statistically significant difference between standard and short therapy groups with respect to the relapse rate in our study group.     

Primary nephrotic syndrome during childhood in Turkey

BACKGROUND: Minimal change nephrotic syndrome (MCNS) is the most common cause of primary nephrotic syndrome (NS) during childhood. However, recent studies from different countries have reported an increasing incidence of focal segmental glomerulosclerosis (FSGS) in children. METHODS: This is a retrospective study in which 392 Turkish children who were diagnosed with NS during the last 10 years and were followed for at least 2 years, were evaluated. Mean age of the study group was 4.6 +/- 3.4 years (range 0.9-16 years) and 232 were male and 160 were female. RESULTS: In total, 280 patients were diagnosed as MCNS with their initial presentations, laboratory features, and clinical course. Kidney biopsy was performed in the remaining 112 children according to current recommendations. The results showed that membranoproliferative glomerulonephritis (MPGN) was the most common histopathologic diagnosis, 38 (34%) of the 112 patients were found to have MPGN. The number diagnosed as FSGS was 26 (23%). A significant difference was found between the age groups for both MPGN and FSGS, the former being more common in children >6 years of age and the latter more frequent in children 相似文献   

先天性肾病综合征研究进展

先天性肾病综合征的发病率尽管不高,却是导致儿童终末期肾病的常见病因,该病的诊断与治疗甚为棘手.近年来,随着分子生物学等技术的快速发展,该病的病因、诊断和治疗等均取得了一定进展.该文详细阐述了先天性肾病综合征的最新分型和诊治方法.     

Renal effects of cyclosporin A in children treated for idiopathic nephrotic syndrome

Little data have been published on tubular renal function during cyclosporin A treatment in children without transplants. We studied 12 young subjects (mean age 10 years) with steroid-responsive idiopathic nephrotic syndrome and with signs of steroid toxicity. After achieving remission with prednisone 60 mg/m², 8 children started cyclosporin A therapy (6 mg/kg/day) (group A) and 4 children were given cyclophosphamide 2.5 mg/kg/day (group B). The latter were considered as controls together with 10 other children with idiopathic nephrotic syndrome in complete remission and off therapy (group C). We monitored creatinine clearance and tubular handling of β₂-microglobulin, sodium, phosphorus and uric acid for one year. Cyclosporin A induced a decrease in creatinine clearance with a decrease in fractional excretion of β₂-microglobulin; sodium excretion was similar in the two treated groups and a transient decrease in fractional excretion of uric acid was seen only in patients receiving cyclosporin A. Both groups showed an increased renal threshold phosphate concentration. Our results suggest that in children, cyclosporin A therapy induces a decrease in glomerular filtration rate associated with increased reabsorption activity of proximal tubular cells.     

甲基泼尼松龙冲击疗法治疗小儿肾病综合征复发疗效观察

目的 初步分析甲基泼尼松龙冲击治疗激素敏感的原发性肾病综合征的疗效和不良反应。方法 对2000～2004年应用甲基泼尼松龙冲击治疗的激素敏感的11例原发性肾病综合征住院患儿临床资料进行回顾性分析,并与既往相似情况下口服足量泼尼松治疗的效果进行自身对照分析。结果 11例中男9例,女2例;甲基泼尼松龙冲击时年龄3～12岁,平均（7．5±3．1）岁;病程3～38个月,平均（15．1±10．4）个月;3例为不频复发,8例为频复发。甲基泼尼松龙冲击治疗1疗程后11例中尿蛋白转阴10例,尿蛋白转阴时间为1～18d,平均（7．4±4．4）d;其中7例较口服足量泼尼松治疗尿蛋白转阴的时间缩短,但对前后2次复发时病情基本相同的6例患儿尿蛋白转阴时间进行配对样本t检验,差异无统计学意义。甲基泼尼松龙冲击治疗期间,出现不良反应6例。结论 与口服足量泼尼松治疗相比,甲基泼尼松龙冲击治疗对小儿激素敏感的原发性肾病综合征的疗效尚待定论,且治疗期间可能出现不良反应,需慎重使用,其有效性及安全性有待进一步作前瞻性随机对照研究证实。     

肾病综合征患儿的腹痛问题

腹痛是肾病综合征患儿经常主诉的症状之一,多数情况下主要是由于肾病导致迅速产生大量腹水及肠壁水肿引起,但有些患儿腹痛明显,很可能是某些严重并发症的临床表现。该文就几种引起肾病患儿严重腹痛的病因如低血容量发作、原发性腹膜炎、血栓栓塞及胃肠道并发症进行简述,以加强对肾病患儿腹痛的重视,从而达到早期诊断、早期治疗,改善预后。