Long-term Treatment of Minimal-change Nephrotic Syndrome with Cyclosporin: A Control Biopsy Study

Seven patients with minimal-change nephrotic syndrome confirmedby renal biopsy were treated with cyclosporin (CsA). Four patientshad frequent relapses and three others had primary steroid resistantnephrotic syndrome. Corticosteroids were discontinued as soonas CsA whole blood trough values of 200–500 ng/ml (RIAmethod) were reached. A full remission, defined as completedisappearance of proteinuria, was achieved in five patientsunder this treatment. In the two other patients proteinuriawas reduced. Two patients experienced an acute episode of dose-dependentnephrotoxicity; however, overall renal function, as determinedby the creatinine clearance, was stable. Control biopsies infive patients after a mean treatment period of 10 months showedno significant vascular or interstitial toxicity.     

ACE Gene Polymorphism in Turkish Children with Nephrotic Syndrome

Since 1990, the role of angiotensin converting enzyme (ACE) gene polymorphism in various renal and cardiac diseases is still debated. This study comprised 71 pediatric patients with nephrotic syndrome, 47 males (66%) and 24 females (34%) with a mean age of 57.4 ± 37.6 months, and a control group of 83 healthy males (59%) and 57 healthy females (41%) with a mean age of 505 ± 160.5 months. The distribution of the ACE genotype in the control group was II, 11%; ID, 53%; and DD, 36%, and the nephrotic syndrome was II, 4%; ID, 78%; and DD, 18%. Angiotensin-converting enzyme genotypes were significantly different between patients and control groups (p<0.05). The study groups consisted of 52 (73%) with steroid-sensitive nephrotic syndrome (SNSS) and 19 (27%) with steroid-resistant nephrotic syndrome (SRNS). The distribution of the ACE genotype was II, 6%; ID, 75%; and DD, 19% in the SSNS population and ID, 84% and DD, 16% in the SRNS population. No statistically significant difference was found between steroid sensitivity and ACE genotypes (p=0.5). The results show that ACE I/D polymorphism does not contribute to the steroid resistance, even though this study indicates that the presence of the I/D genotype has a much higher risk—approximately 2.8 times—of having nephrotic syndrome. Further studies with a larger number of patients are needed.     

原发性肾病综合征患者IL-18的血浆水平及其在肾组织的表达

目的：初步探讨白细胞介素18(IL—18)在原发性肾病综合征(PNS)发生发展中的作用。方法：采用酶联免疫吸附(ELISA)法测定11例正常人及24例PNS患者血浆IL—18水平，同时用免疫组织化学方法检测6例正常肾组织和上述24例PNS患者肾组织IL—18的表达量。结果：PNS患者血浆IL—18水平与正常对照组IL较无统计学意义；而且各种病理类型间的差异也没有统计学意义(P均＞0．05)；而肾小球及肾小管—间质IL—18表达量却均显著高于正常对照组(P均＜0．01)。不同病理类型PNS肾小球区IL—18表达量存在差异，以膜增生性肾小球肾炎(MPGN)表达量为最高，其次为系膜增生性肾小球肾炎(MesPGN)，而膜性肾病(MD)、局灶节段性肾小球硬化(FSGS)和轻微病变(MCD)的表达量则相对较低，并且肾小球区IL—18表达量与24h尿蛋白排泄量(24h　UPQ)至正相关，与血浆白蛋白浓度(Alb)至负相关(r分别为0．669和－0．727，P均＜0．01)；肾小管—间质区IL—18表达量与小管—间质损害程度至正相关(r＝0．484，P＜0．05)。结论：肾组织IL—18高表达可能参与PNS的发病过程，而又可能以自分泌或/和旁分泌方式起作用。     

检测微小病变型肾病综合征患者腺苷脱氨酶的临床意义

目的：检测微小病变型肾病综合征（MCNS）患者血清及淋巴细胞内腺苷脱氨酶（S-ADA,L-ADA）活性的变化,以评估其细胞免疫状态,并探讨临床意义。方法：选取初发的MCNS患者30例,应用免疫学技术测定不同的病程阶段患者的S-ADA及L-ADA活性,比较ADA活性变化。结果：激素治疗前,激素敏感组（SS）、激素依赖组（SD）和激素抵抗组（SR）两两比较无统计学意义（P〉0.05）,经激素足量治疗4周后,SS组、SD组S-ADA和L-ADA活性均下降,SS组下降最明显（P〈0.01）,而SR组无变化（P〉0.05）;短期缓解期组S-ADA活性降至对照组水平,L-ADA活性也下降,但与对照组相比,其水平仍高（P〈0.05）;长期缓解期组S-ADA及L-ADA活性与对照组相比,无统计学差异（P〉0.05）;S-ADA及L-ADA活性有显著的相关性（r=0.811,P〈0.01）。结论：MCNS患者存在着细胞免疫的紊乱,检测S-ADA及L-ADA活性可作为判断临床治疗效果的指标之一。     

黄芪对肾病综合征患者氧化应激影响的研究

目的:探讨原发性肾病综合征(PNS)患者氧化应激状态以及中药黄芪的抗氧化作用.方法:47例PNS患者随机分为A、B两组,分别给予激素和激素加黄芪治疗,于治疗前后测定血浆及红细胞丙二醛(MDA)浓度、红细胞超氧化物歧化酶(SOD)、全血谷胱甘肽过氧化物酶(GSH-px)的活性、维生素E(Vit E)的含量和主要生化指标.结果:与正常对照组比较,PNS患者MDA浓度增高, SOD 、GSH-px活性和Vit E含量显著降低;与治疗前比较,A组氧化应激指标无显著性改变,B组患者MDA浓度降低,SOD 、GSH-px活性增加(P<0.05).结论:PNS患者氧化应激增强,中药黄芪有一定的抗氧化作用.     

中西医结合治疗老年人肾病综合征98例疗效观察

目的 :比较中西医结合和单纯西医治疗老年人原发性肾病综合征 (PNS)的临床疗效。方法 :98例老年人PNS随机分为治疗组和对照组。治疗组 (4 9例 )用强的松、环磷酰胺 (CTX)并分阶段辨证配合中药治疗。对照组(4 9例 )单用西药治疗。观察临床缓解率、不良反应率、复发率及缓解时间。结果 :治疗组完全缓解率 (5 9.2 % )和总缓解率 (85 .7% )均显著高于对照组 (38.7%、6 3.2 % ,P <0 .0 5 )。治疗组不良反应率 (36 .7% )明显低于对照组 (80 .1% ,P <0 .0 1)。随防 (4 2± 11.6 4 )月后 ,治疗组复发率 (10 .3% )与对照组 (2 1% )比较无统计学差异 (P >0 .0 5 )。但治疗组平均缓解期为 (36 .7± 4 .6 )月 ,较对照组 (15 .2± 4 .4 )月显著延长 (P <0 .0 1)。结论 :中西医结合治疗老年人肾病综合征优于单纯西医治疗。     

红曲提取物对肾病综合征血清脂谱和肾小球硬化的影响

目的:探讨红曲提取物对肾病综合征(NS)大鼠降血脂的肾脏保护作用。方法:采用嘌呤霉素引起的NS模型。实验分正常组、肾病组和治疗组(红曲提取物0.8g·kg~(-1)·d~(-1))。检测各组血清脂谱,以Northern杂交检测肾脏转化生长因子β(TGF-β)纤维连接蛋白(FN)mRNA表达。结果:肾病大鼠呈高脂蛋白血症,该组肾脏TGF-β和FNmRNA表达较正常对照组和治疗组均明显上调(P<0.01,~#P<0.01);红曲提取物治疗组,血清总胆固醇、甘油三脂、低密度脂蛋白,低密度脂蛋白和载脂蛋白B低于肾病对照组,而高密度脂蛋白和载脂蛋白A_1高于肾病组(~#P<0.05),同时治疗组较肾病组血浆白蛋白升高,尿蛋白减少。结论:红曲提取物能显著降低NS大鼠血脂水平,减轻蛋白尿,升高血浆白蛋白。并通过下调肾脏TGF-β和FNmRNA的表达防止和减轻肾小球硬化。     

Nephrotic Syndrome and AA Amyloidosis Revealing Adult-Onset Cryopyrin-Associated Periodic Syndrome

Cryopyrin-associated periodic syndrome (CAPS) is due to gain-of-function mutations in the cryopyrin gene, which determines an overactive inflammatory response. AA amyloidosis is a complication of this syndrome.

A 53-year-old man was referred to us because of lower limb edema. Past history: at the age of 20, he complained of arthralgia/arthritis and bilateral hypoacusis. At the age of 35, he presented posterior uveitis, several episodes of conjunctivitis, and progressive loss of visual acuity. Laboratory tests disclosed nephrotic syndrome, and renal biopsy showed AA amyloidosis. He was given anakinra with improvement of arthritis. A genetic study revealed the p.D303N mutation in the cryopyrin gene, and he was diagnosed as having AA amyloidosis due to CAPS. Twenty-one months after starting anakinra, the arthritis has disappeared, although nephrotic-range proteinuria persisted.

It is important to be aware of cryopyrin-associated periodic syndrome because it can cause irreversible complications, and there is effective therapy.     

中西医结合治疗原发性肾病综合征的临床研究

目的 :探讨中西医结合治疗原发性肾病综合征 (PNS)临床意义。方法 :2 6 6例PNS患者随机分为治疗组 (中药 强的松 )和对照组 (强的松 ) ,观察两组治疗前后的临床表现 ,2 4h尿蛋白定量、血浆白蛋白、血脂、肾功能及尿纤维蛋白降解产物 (FDP) ,副作用和并发症及复发情况。结果 :治疗前两组各项生化指标 ,尿FDP均明显异常 ,治疗后治疗组各项生化指标及尿FDP明显改善 ,治疗前后比较 (P <0 .0 1或 <0 .0 5 )。治疗组总有效率 (97.2 % )高于对照组 (72 .2 % ) ,对照组副作用和并发症发生率 (5 9.5 % )高于治疗组 (2 1.8% ) ,治疗组复发率 (2 .8% )低于对照组(13.5 % )。结论 :中西医结合治疗具有调节PNS免疫紊乱的作用。能提高疗效 ,减少副作用和并发症。对预防感染 ,巩固疗效 ,减少复发有一定帮助。     

Klinefelter's Syndrome and Mediastinal Teratoma

In a patient with Klinefelter's syndrome and HCG secreting mediastinal teratoma an interpretation is given of the hormonal changes in the pre- and postoperative period.
Ten cases of KS and mediastinal germ cell tumors were previously published. A karyotype could be advocated in all cases of mediastinal germ cell tumors.
Klinefelter-Syndrom und Mediastinum-Teratom

Zusammenfassung

Bei einem 17-Jährigen mit Klinefelter-Syndrom (47, XXY) und einem HCG-produzierenden Teratom des Mediastinums wird eine Interpretation für die hormonalen Veränderungen in der prae- und postoperativen Periode gegeben. Bisher sind 10 Patienten mit Klinefelter-Syndrom und mediastinalen Keimzelltumoren in der Literatur beschrieben worden. Es wird ausdrücklich darauf gedrungen, bei allen mediastinalen Keimzelltumoren grundsätzlich auch den Karyotypus zu untersuchen.     

Disease Severity Correlates with Thrombotic Capacity in Experimental Nephrotic Syndrome

Thrombotic disease, a major life–threatening complication of nephrotic syndrome, has been associated with proteinuria and hypoalbuminemia severity. However, it is not fully understood how disease severity correlates with severity of the acquired hypercoagulopathy of nephrotic syndrome. Without this knowledge, the utility of proteinuria and/or hypoalbuminemia as biomarkers of thrombotic risk remains limited. Here, we show that two well established ex vivo hypercoagulopathy assays, thrombin generation and rotational thromboelastometry, are highly correlated with proteinuria and hypoalbuminemia in the puromycin aminonucleoside and adriamycin rat models of nephrotic syndrome. Notably, in the puromycin aminonucleoside model, hyperfibrinogenemia and antithrombin deficiency were also correlated with proteinuria severity, consistent with reports in human nephrotic syndrome. Importantly, although coagulation was not spontaneously activated in vivo with increasing proteinuria, vascular injury induced a more robust thrombotic response in nephrotic animals. In conclusion, hypercoagulopathy is highly correlated with nephrotic disease severity, but overt thrombosis may require an initiating insult, such as vascular injury. Our results suggest that proteinuria and/or hypoalbuminemia could be developed as clinically meaningful surrogate biomarkers of hypercoagulopathy to identify patients with nephrotic syndrome at highest risk for thrombotic disease and potentially target them for anticoagulant pharmacoprophylaxis.     

A Case with Acute Renal Failure and Subsequent Nephrotic Syndrome

Nephrotic syndrome due to secondary amyloidosis is not so common, and the prognosis depends on primary disease. We report a case of secondary amyloidosis caused by Takayasu's arteritis. Sustained high fever and acute renal failure proceeded to the occurrence of nephrotic syndrome. Secondary amyloidosis was diagnosed by renal biopsy before the diagnosis of primary disease. She was completely recovered from nephrotic syndrome after two years' treatment with prednisolone, aspirin, and dimethyl sulfoxide. This rare case provides meaningful suggestions for the diagnosis and treatment of acute renal failure and nephrotic syndrome caused by secondary amyloidosis.     

Steroid Responsiveness of Children with Idiopathic Nephrotic Syndrome in Southeastern Region of Turkey

Background. Our aim was to determine the prognostic factors effective in the response to steroid treatment and relapse frequency. Patients and Methods. In this study, we evaluated 84 children with idiopathic nephrotic syndrome followed-up from 1997–2002. The variables were analyzed with respect to medical history, physical examination, laboratory findings, response to treatment, and factors associated with remissions and relapses. Our study group consisted of 62 children with minimal change nephrotic syndrome (MCNS), 11 children with focal segmental glomerulosclerosis (FSGS), and 11 children with diffuse mesangial proliferation (DMP). Results. According to response to steroids; 57.1% were steroid-sensitive with infrequent relapses, 22.6% were steroid-dependent with frequent relapses, and 20.2% were steroid-non-responders. Significantly high non-responder ratios to steroids were found in children with initial hypertension and hematuria (p < 0.05). Although patients older than six years were found to be associated with steroid non-response (p < 0.05), the number of relapses were found to be increased with an increasing number of infections (p < 0.05). The time period for the first relapse was found to be statistically correlated with relapse numbers of the first 6 (p = 0.001) and 12 (p = 0.01) months. Conclusion. The time span between initial presentation and remission and the number of infections were significant for relapse frequency. The existence of hematuria and hypertension and age greater than 6 years at initial presentation were associated with steroid non-responsiveness. The likelihood of developing resistance to the treatment should be emphasized early to the parents of patients bearing these risk factors, and hence the possible disappointment in the family should be prevented.     

腹腔镜下卵巢打孔术治疗多囊卵巢综合征不孕症51例临床分析

目的探讨腹腔镜下卵巢打孔术对多囊卵巢综合征不孕症的治疗价值。方法对51例女性多囊卵巢综合征合并不孕症患者行腹腔镜下卵巢打孔术治疗,单极电凝打孔,直径一般为2-4 mm,深度4-6 mm,两孔间隔约10 mm。手术前后监测血清促卵泡生成素（FSH）、黄体生成素（LH）、睾酮（T）、雌二醇（E2）,术后监测排卵情况并观察妊娠率。结果术后1个月血清FSH升高,LH、LH/FSH、T下降（P〈0.05）。术后自然排卵率84.3%（43/51）,术后2年内妊娠39例,术后累积妊娠率76.5%（39/51）。结论腹腔镜卵巢打孔术为多囊卵巢综合征合并不孕症的有效快捷的治疗方案,并且创伤小,术后粘连少,多胎妊娠率低,卵巢过度刺激综合征少。     

环孢素A与吗替麦考酚酯治疗难治性肾病综合征疗效对比

目的：通过检测临床指标的变化,观察对比环孢素A和吗替麦考酚酯治疗难治性肾病综合征的疗效差异。方法：将本院自2008年1月以来收治的56例原发性难治性肾病综合征患者随机分为CsA、MMF两组,在口服泼尼松0.5mg.kg-1.d-1治疗的基础上,CsA组口服环孢素4mg·kg-1.d-1,MMF组口服吗替麦考酚酯0.75mg2/d,进行1年的随访,在治疗前、治疗3、6、12个月分别记录尿常规、24h尿蛋白定量、肝功能（AST、ALT、Alb）、肾功能（CRE,UA,GLU）、血脂以及环孢素血药浓度等指标的变化,并记录不良反应。结果：从治疗1个月开始,部分患者的尿蛋白定量减轻,血浆白蛋白水平开始有所恢复,前后结果差异有统计学意义（P〈0.05）,两组患者之间差异无统计学意义（P〉0.05）。结论：环孢素A、吗替麦考酚酯均可以有效的治疗难治性肾病综合征,治疗同期两者差异不明显。     

Divergent Effects of Glucocorticoids on Cortical and Trabecular Compartment BMD in Childhood Nephrotic Syndrome

Glucocorticoid (GC) effects on skeletal development have not been established. The objective of this pQCT study was to assess volumetric BMD (vBMD) and cortical dimensions in childhood steroid‐sensitive nephrotic syndrome (SSNS), a disorder with minimal independent deleterious skeletal effects. Tibia pQCT was used to assess trabecular and cortical vBMD, cortical dimensions, and muscle area in 55 SSNS (age, 5–19 yr) and >650 control participants. Race‐, sex‐, and age‐, or tibia length‐specific Z‐scores were generated for pQCT outcomes. Bone biomarkers included bone‐specific alkaline phosphatase and urinary deoxypyridinoline. SSNS participants had lower height Z‐scores (p < 0.0001) compared with controls. In SSNS, Z‐scores for cortical area were greater (+0.37; 95% CI = 0.09, 0.66; p = 0.01), for cortical vBMD were greater (+1.17; 95% CI = 0.89, 1.45; p < 0.0001), and for trabecular vBMD were lower (?0.60; 95% CI, = ?0.89, ?0.31; p < 0.0001) compared with controls. Muscle area (+0.34; 95% CI = 0.08, 0.61; p = 0.01) and fat area (+0.56; 95% CI = 0.27, 0.84; p < 0.001) Z‐scores were greater in SSNS, and adjustment for muscle area eliminated the greater cortical area in SSNS. Bone formation and resorption biomarkers were significantly and inversely associated with cortical vBMD in SSNS and controls and were significantly lower in the 34 SSNS participants taking GCs at the time of the study compared with controls. In conclusion, GCs in SSNS were associated with significantly greater cortical vBMD and cortical area and lower trabecular vBMD, with evidence of low bone turnover. Lower bone biomarkers were associated with greater cortical vBMD. Studies are needed to determine the fracture implications of these varied effects.