原发性肾病综合征合并颅内静脉窦血栓形成一例

患者,男,22岁,因颜面及双下肢浮肿4个月,加重伴头痛1周入院。患者因颜面及双下肢浮肿2个月余,于2004年7月26日入我科,诊断为肾病综合征;病理诊断：轻度系膜增生型IgA肾病（Lee分级：Ⅱ级）。采用甲基泼尼松龙0．5g,1次／d,连续3d,后予强的松50nag,1次／d,同时降脂治疗。3周后尿蛋白转阴。8月30日无明显诱因出现双下肢轻度浮肿,头枕部持续闷痛,无发热、呕吐。2004年9月6日再次入我科。     

中西医结合治疗原发性难治性肾病综合征的临床观察

目的 :观察中西医结合治疗原发性难治性肾病综合征 (ⅡNS)的临床疗效。方法 :6 2例ⅡNS患者随机分为治疗组和对照组。两组患者均给予激素及环磷酰胺治疗 ,治疗组 (31例 )辨证的基础上加用中药治疗。观察临床缓解率、副反应率及复发率。结果 :治疗组完全缓解率 (5 8.1% ) ,对照组 (4 5 .2 % ) ,两组比较无统计学差异 (P >0 .0 5 ) ;治疗组总缓解率 (87.1% ) ,对照组 (5 8.1% ) ,两组比较有统计学差异 (P <0 .0 1)。治疗组副反应率 (4 5 .2 % ) ,与对照组(83.9% )比较有统计学差异 (P <0 .0 1)。治疗组复发率 (2 2 .2 % )与对照组 (5 0 % )比较有统计学差异 (P <0 .0 1)。结论 :中西医结合治疗ⅡNS优于单纯西医治疗     

中西医结合治疗成人激素依赖性原发性肾病综合征40例疗效观察

目的 :探讨中西医结合治疗成人激素依赖性 (SD)原发性肾病综合征 (PNS)的临床疗效。方法 :4 0例成人SD -PNS予中西医结合治疗 (简称治疗组 ) ,对照组 4 2例单纯西医治疗 (简称对照组 ) ,观察两组治疗后缓解率、复发率、不良反应率。结果 :治疗组总缓解率为 77.2 % ,对照组为 35 .7% ,有统计学差异 (P <0 .0 1)。治疗组 6个月、12个月、4 8个月时复发率分别为 0 ,3.2 3% ,9.6 8% ;对照组为 13.3% ,2 0 % ,33.3% ,2组比较有统计学差异 (P <0 .0 5 ) ;治疗组不良发生率为 32 .7% ,对照组为 6 9.2 % ,治疗组显著低于对照组 (P <0 .0 1)。结论 :中西医结合治疗成人激素依赖性原发性肾病综合征疗效满意。     

中西医结合治疗原发性肾病综合征82例

目的观察中西医结合治疗成人原发性肾病综合征的疗效和不良反应。方法将中西医组42例和西医组40例对照观察。结果西医组总有效率55.0％,激素不良反应的发生率为50.0％。中西医组总有效率为85.7％,显著高于对照组(P＜0.01);使用激素的不良反应发生率为16.7％,显著低于对照组(P＜0.01)。结论用中药配合激素治疗原发性肾病综合征,能增加疗效,减少激素治疗过程中的不良反应。     

中西医结合治疗原发性肾病综合征疗效及激素副作用观察

目的 :探讨中西医结合方法治疗原发性肾病综合征的疗效及观察激素的副作用。方法 :将 6 9例首发住院患者随机分成两组 ,其中中西医结合治疗组 (中西组 ) 35例 ,西医治疗组 (西医组 ) 34例 ,分别观察其疗效、激素副作用及 2年内复发率。结果 :中西医组治疗有效率 91.4% ,西医组治疗有效率 76 .5 % (P <0 .0 5 )副作用中西医组 2 2 .9% ,西医组 5 0 .0 % (P <0 .0 5 ) ,完全缓解者 2年内复发率中西组 19.2 % ,西医组 45 .0 % (P <0 .0 5 )。结论 :中西结合治疗原发性肾病综合征疗效明显优于单纯西医治疗 ,且副作用少 ,复发率低     

绞股蓝刺五加与激素治疗小儿原发性肾病综合征疗效的探讨

小儿原发性肾病综合征 (简称ＮＳ) ,是一种难治性常见病。目前其治疗仍以激素为首选药物 ,但因其易出现尿蛋白反复或对激素依赖 ,目前已成为国内外学者关注的难题。近年来我们采用绞股蓝、刺五加与激素联合治疗本病 ,临床应用疗效好 ,副作用少 ,比单纯应用激素治疗为优 ,现将较完整的 40例临床资料总结如下。资料与方法1　病例选择　本组病例选自 1997年～ 1999年儿科住院病人 40例 ,其中男 2 4例 ,女 16例 ;2岁～ 5岁 19例 ,6岁～ 9岁 13例 ,10岁～ 13岁 8例。病程 1～ 2 8个月 ,平均9.8个月。全部病例均符合 1981年小儿肾脏病科研协作组第…     

成人原发性肾病综合征治疗的探讨

报告成人原发性肾病综合征（ＰＮＳ）１００例，采用８周大剂量强的松治疗后，病情未获显著缓解者，至小剂量强的松（０．５ｍｇ·ｋｇ￣－１／ｄ）治疗时，维持８个月或更长一些时间，总疗程在１年或更长一些时间，病情往往获得显著缓解。与过去常规大剂量激素治疗成人ＰＮＳ１００例比较，两者有效率分别为７１％和５４％，有显著性差异（Ｐ＜０．０５）。本文还观察了各种病理类型的激素疗效。     

中西医结合治疗原发性肾病综合征180例观察

目的：为了探讨原发性肾病综合征（PNS）的治疗措施,提高其疗效,方法：将1995年3月－2000年3月PNS患随机分组,进行分析研究,结果：（1）中西医结合组完全缓解63．89％,部分缓解24．44％,总有效率为88．33％,显高于单纯西药组的42．15％,19．01％和61．16％（P＜0．01）。（2）中西医结合组复发率及激素不良反应发生率分别为17．39％和18．33％,明显低于单纯西药组的68．63％和78．51％（P＜0．01）。结论：中西医结合治疗PNS疗效满意。     

激素依赖性肾病综合征患儿并发垂体性侏儒症一例

小儿激素依赖性肾病综合征（SDNS）长期接受糖皮质激素治疗可导致患儿生长发育迟缓，但这种生长发育迟缓一般仅较正常同龄儿童平均水平略低，且肾病综合征（NS）缓解停用激素后会有一追赶期，成年后基本无差异。本文报道的SDNS患者因病情控制不佳，长期应用糖皮质激素治疗而导致严重的生长发育障碍，表现为垂体性侏儒症。     

激素治疗难治性肾病综合征对蛋白尿及肾脏高灌状态的加重作用

激素治疗难治性肾病综合征对蛋白尿及肾脏高灌状态的加重作用崔世维，邵亚男，钱桐荪ＡＣＣＥＬＥＲＡＴＥＩ，ＥＦＦＥＣＴＳＯＦＧＬＵＣＯＳ－ＴＥＲＯＩＤＳＯＮＰＲＯＴＥＩＮＵＲＩＡＡＮＤＨＹＰＥＲＦＩＬＴＲＡ－ＴＩＯＮＳＴＡＴＥＩＮＰＡＴＩＥＮＴＳＷＩＴＨ...     

Cyclosporine and steroid therapy in children with steroid-resistant nephrotic syndrome

We conducted a prospective, multicenter trial to evaluate the efficacy and safety of a 12-month course of cyclosporine in children with steroid-resistant nephrotic syndrome (SRNS). Thirty-five patients were enrolled, of whom 28 had minimal change or diffuse mesangial proliferation (MC/DMP), and seven had focal segmental glomerulosclerosis (FSGS). All patients received cyclosporine and prednisolone; patients with FSGS additionally received methylprednisolone pulse therapy (MPT). The dose of cyclosporine was adjusted to maintain a trough level of 120–150 ng/ml during the initial 3 months of treatment, followed by 80–100 ng/ml during months 4–12. The primary end point was the remission rate at month 12. Remission was achieved in 23 of 28 (82.1%) patients in the MC/DMP group and in six of the seven (85.7%) patients in the FSGS group. Follow-up renal biopsies were performed in 26 patients (nine at month 12, 17 at month 24), and cyclosporine-related nephrotoxicity was detected in one (3.8%). Major adverse events comprised severe bacterial infections (two patients) and posterior reversible encephalopathy syndrome (one patient). In conclusion, a high remission rate was achieved in our patient cohort using a combined cyclosporine/prednisolone treatment regimen in children with SRNS who had MC/DMP and a combined cyclosporine/prednisolone plus MPT regimen in children who had FSGS.     

Value of serum IgG combined with IgE in predicting steroid therapy response in children with primary nephrotic syndrome

Objective To observe the value of serum IgG combined with IgE in the diagnosis of steroid-sensitive nephrotic syndrome (SSNS) children. Methods Children with primary nephrotic syndrome diagnosed in Beijing Children's Hospital affiliated to Capital Medical University from January 2018 to March 2019 were enrolled. According to the 4 weeks steroid therapy, the patients were divided into steroid sensitive group and steroid resistance group. The receiver operating characteristic curve (ROC) was used to analyze the diagnostic value of serum IgG combined with IgE for SSNS. Results There were no significant differences in age, gender, course of the disease, hemoglobin, platelet, urea nitrogen, creatinine, albumin, globulin, 24 h urine protein, IgA level, IgM level and hematuria between steroid sensitive group and steroid resistance group (all P＞0.05). There were significant differences in C3, IgG and IgE levels (all P＜0.05). ROC curve analysis showed that the cut-off values of IgG and IgE in the diagnosis of SSNS were 2.57 g/L (AUC=0.717, 95%CI 0.623-0.812, P＜0.001) and 174.3 IU/ml (AUC=0.828, 95%CI 0.751-0.905, P＜0.001). The area under the ROC curve for IgG＜2.57 g/L in combination with IgE＞174.3 IU/ml in the diagnosis of SSNS was 0.904 (95%CI 0.834-0.953, P＜0.001). Logistic regression showed that children who met both IgG＜2.57 g/L and IgE＞174.3 IU/ml were 51.00 times more likely to have SSNS than those who did not (P＜0.001). Conclusion IgG combined with IgE can be used as a simple and practical clinical marker for predicting SSNS.     

小儿激素依赖性肾病综合征长期激素治疗对生长发育的影响

目的探讨难治性肾病综合征及糖皮质激素治疗对小儿生长发育的影响，增强对小儿肾病综合征生长发育迟缓的防治意识。方法针对1例病情长期未得到良好控制的激素依赖性肾病综合征并发垂体性侏儒症的患儿成功诊治经验，回顾分析激素依赖性肾病综合征对患儿生长发育影响的相关文献。结果肾病综合征疾病本身及糖皮质激素长期应用都会对患儿生长发育造成影响，具体机制还不清楚。结论在治疗小儿肾病综合征时，应尽可能对初发病例采用较短疗程的治疗方案。对此患儿应避免长期使用激素，可换用其它免疫抑制剂，并注意监测身高和生长速度，必要时可加用毕长激索治疗．     

A case of nephrotic syndrome with diabetes mellitus and primary aldosteronism]

A 65-year-old man had been followed by a family doctor for the treatment of hypertension and chronic hepatitis (type C) for about 20 years. Although he was pointed out to have impaired glucose tolerance and primary aldosteronism in 1995, he refused an adrenal tumor operation. He was admitted to our hospital on December, 1997 for further evaluation of general malaise, pitting edema of the legs, and positive urinary protein. A diagnosis of nephrotic syndrome was made on admission and a renal biopsy was performed. Histological findings indicated that he was at the early phase of diabetic nephropathy and hypertensive renal sclerosis. It is commonly believed that diabetic nephropathy develops after ten years of diabetic history and under poor control conditions. The diabetic history of this patient was only several years and the disease was under good control. In contrast to blood glucose, hypertension was not well-controlled with any antihypertensive drug, because he had a primary aldosteronism. Unfortunately, he could not take a spironolactone because of side effects. After removal of his adrenal tumor, his blood pressure was normalized gradually, and concomitantly his urinary protein was reduced and plasma protein and albumin were restored. Hypokalemia also disappeared. These findings suggest that uncontrolled hypertension may have accelerated the condition of diabetic nephropathy. The data indicates that the control of hypertension is important for inhibiting the progression of diabetic nephropathy.     

Growth in boys with idiopathic nephrotic syndrome on long-term cyclosporin and steroid treatment

Although steroid-free remission can usually be achieved with cyclosporin A (CsA) in patients with steroid-dependent nephrotic syndrome (SDNS), some CsA-treated patients require long-term steroid therapy. Data on growth in these patients are scarce. Sixty-four boys with SDNS receiving long-term CsA and steroid therapy were retrospectively analyzed. During the 10-year follow-up period, height standard deviation score (HSDS) remained in the normal range in 47 patients but was below −2 SD in 17 patients. The occurrence of growth retardation was influenced by height at diagnosis and the number of relapses. Thirty patients were followed for at least 3 years before and after age 12. The decrease in HSDS per year of disease in patients older than 12 years was twice that observed in children younger than 12. However, adult height was ≤ −2 SD in only two of the 14 patients reaching adult height, reflecting potential catch-up growth during late puberty. Careful monitoring of growth is recommended, given than up to 25% of patients experienced severe growth retardation during the course of their disease.     

Nitrogen mustard therapy in children with frequent-relapsing nephrotic syndrome and steroid toxicity

Nitrogen mustard (HN-2) is an alkylating agent known to be effective in inducing and prolonging remissions of nephrotic syndrome in children. The fact that the drug is administered intravenously eliminates the problem of compliance, and the fact that the treatment needs to be given only for a few days may lower the incidence of side-effects observed with other cytotoxic agents. We administered HN-2 to 12 children with the steroid-sensitive frequent-relapsing form of nephrotic syndrome, who had evidence of steroid toxicity. Life table analysis reveals a 46% sustained remission rate after 27 mo of follow-up. Complications of HN-2 treatment included only mild thrombophlebitis (2 patients) and mild local paresthesia (1 patient).     

原发性胆汁性肝硬化并发肾病综合征一例

女,31岁,双下肢水肿伴尿检异常1 d于2009年11月24日入院.患者劳累后出现双下肢水肿,来我院检查,尿常规蛋白3+,多项检查异常,为进一步治疗,以慢性肾炎收治入院.5年前因分娩时,在当地医院查肝功能,ALT及AST均升高,后去南京市第一人民医院查抗线粒体抗体阳性,诊断原发性胆汁性肝硬化Ⅱ期.     

Effect of intrauterine growth retardation on the progression of nephrotic syndrome

BACKGROUND/AIM: Neonates with intrauterine growth retardation (IUGR) experience higher morbidity and mortality rates than appropriate-for-gestational-age (AGA) neonates. The purpose of our study was to clarify whether IUGR has any influences on the progression of nephrotic syndrome in children. METHODS: We performed a retrospective review of 56 children with nephrotic syndrome. IUGR was defined as a birth weight less than the tenth percentile for gestational age. Among 56 patients having nephrotic syndrome, 8 had IUGR, and 48 were AGA. RESULTS: The 24-hour urinary protein level in children with IUGR was significantly higher than that in children who were AGA (7.61 +/- 6.75 vs. 3.92 +/- 3.70 g/day, p < 0.05). There was a statistically significant difference in the incidence of steroid resistance (62.5 vs. 10.4%, p < 0.05) and in the time to remission (median 60 vs. 13 days, p < 0.05) between the children with IUGR and those being AGA. Also, there was a significant difference in the incidences of treatment with cytotoxic agents (75 vs. 29.2%, p < 0.05) and complications such as hypertension. CONCLUSIONS: Our report indicates that IUGR predicts an unfavorable progression of nephrotic syndrome. So, it is important for nephrologists to pay attention to the clinical course of nephrotic syndrome in neonates with IUGR.