儿童C1q肾病临床病理研究

目的 :探讨儿童C1q肾病临床病理特征及诊断与治疗。方法 :分析 8例C1q肾病患儿临床病理特点及激素或免疫抑制剂治疗效应 ,并与同期 77例原发性NS患儿作比较。结果 :8例C1q肾病患儿临床上大多表现为原发性NS(6例 ) ,仅 2例表现为肾炎综合征和单纯性血尿。LM主要包括MC(3例 )、MsPGN(2例 )、FSGS(2例 )和ECPGN(1例 )。IF显示明显的系膜区C1q沉积 ,伴或不伴有Ig和补体沉积。EM检查仅 1例有系膜区和内皮下电子致密物沉积。与原发性NS相比 ,6例表现为NS的C1q肾病患儿对泼尼松初次治疗产生耐药的相对危险度为 2 1(P <0 .0 0 1) ,但对免疫抑制剂治疗均敏感。结论 :儿童C1q肾病临床上以对激素耐药的NS为常见表现 ,IF是其主要诊断依据 ,使用免疫抑制剂治疗有效     

原发性肾病综合征免疫抑制剂治疗合并呼吸道感染的危险因素

目的了解原发性肾病综合征(primary nephrotic syndrome,PNS)患者并发呼吸道感染的临床特征与危险因素。方法选取2015年1月至2017年7月于徐州医科大学附属医院肾脏内科初诊初治的PNS患者203例,其中并发呼吸道感染者156例(76.8%),上呼吸道感染105例(51.7%),下呼吸道感染51例(25.1%),回顾性分析其临床特征及危险因素。结果 PNS患者免疫治疗过程中上呼吸道感染发病率18.2%～24.1%,下呼吸道感染发病率第2个月最高6.1%。各个组肾脏病理类型无明显差异。下呼吸道感染组较无呼吸道感染组年龄较大、心率较快、单日激素剂量较高,血淋巴细胞计数、单核细胞计数、血红蛋白、血清白蛋白、免疫球蛋白G (IgG)、估算肾小球滤过率(eGFR)水平较低;尿素、肌酐、尿免疫球蛋白G,C反应蛋白(CRP)、乳酸脱氢酶(lactate dehydrogenase,LDH)较高。低免疫球蛋白G、淋巴细胞、白蛋白水平,激素联合免疫抑制剂使用是下呼吸道感染独立危险因素。结论 PNS患者病程中上呼吸道感染发病率均较高,下呼吸道感染发病率在接受免疫治疗第2个月最高,合并低免疫球蛋白G、低淋巴细胞、白蛋白水平、使用激素联合免疫抑制剂患者易下呼吸道感染。     

膜型狼疮肾炎治疗方法的研究

目的 :探讨膜型狼疮肾炎并肾病综合征 (NephroticSyndromeNS)的最佳治疗方案。 方法 :分析了 2 7例膜型狼疮肾炎并NS患者的治疗效果。 12例患者单独用糖皮质激素治疗 (A组 ) ,而另外 15例患者用环磷酰胺(CTX)冲击及口服强的松和硫唑嘌呤治疗 (B组 )。结果 :在A组 :4例NS完全缓解 ,2例NS部分缓解 ;在平均 (12 1±5 1)月随访期内 ,11例患者发生一次或多次的NS复发。B组 :11例NS完全缓解 ,另外 4例NS部分缓解 ;随后约 (77± 30 )月随访期内 ,仅 1例患者出现NS复发。至随访期终 ,A、B两组患者均存活 ,但A组有 4例患者出现血肌酐增高1倍 ,1例呈持续性NS ,4例完全缓解 ,2例部分缓解。在B组 :1例患者发生毛细血管外增生性肾小球肾炎而在临床起病 15年后最终进入终末期肾衰竭 ,11例患者完全缓解 ,3例患者部分缓解。结论 :对膜型狼疮肾炎合并NS患者 ,用糖皮质激素联合环磷酰胺及硫唑嘌呤治疗 ,比单用糖皮质激素治疗 ,可更稳固地缓解NS ,更好地长期保护肾功能。     

肾病综合征患者激素疗效与血栓素B_2浓度水平的关系

为观察原发性肾病综合征(PNS)患者激素疗效与血清血栓素B_2(TXB)水平变化的关系,我们测定了31例PNS患者激素治疗前后TXB_2浓度,并与15例健康人对照比较,现报告如下。 病例选择 31例PNS患者诊断符合第二届全国肾脏病会议“修订肾小球疾病临床分型意见”标准。男性21例,女性10例,年龄14～48岁,全部病例只接受强的松(45～dmg/d,观察期6～8周)和对症治疗,疗效判定以观察期24小时尿蛋白定量<1g为激素敏感;     

血管紧张素Ⅰ转换酶和血小板活化因子分解酶基因多态性在小儿激素敏感型肾病综合征复发中的意义

95%的原发性肾病综合征(PNS)患儿对激素治疗敏感,但40%～90%的患儿在激素减量或停药后容易复发,而目前仍然没有预测激素敏感型NS易复发的可靠指标。近年研究发现,血管紧张素(Ang)Ⅱ和血小板活化因子(PAF)是与多种肾脏疾病的发生和发展有关的两个重要介质。我们旨在探讨血管紧张素Ⅰ转换酶(ACE)基因及PAF分解酶(PAFAH)基因多态性与小儿激素敏感型NS复发的关系,为判断该病的复发情况提供预测指标。一、对象与方法1.对象(1)病例组:2001年1月至2002年3月我院住院的激素敏感型患儿42例,随访均超过1年,其中男37例,女5例;平均起病年龄(4.5…     

原发性肾病综合征并发急性肾衰竭56例报告

目的分析56例原发性肾病综合征（PNS）并发急性肾衰竭（ARF）患者临床特点、病理改变及疗效。方法回顾性分析56例PNS并发ARF患者临床和病理改变。结果PNS并发ARF发生率约2．9％左右,肾脏病理改变肾小球多以微小病变为主,肾小管上皮细胞浊肿、空泡变性及蛋白管型常见,但肾间质水肿改变并不明显。经及时有效治疗,91．1％患者肾功能可完全恢复正常,其中抗凝治疗和合适透析方式选择起到较为重要的作用。结论PNS并发ARF多发生于肾脏病变轻微患者,其发病机制有待进一步探讨,经及时有效的综合治疗,患者多预后良好。     

茶色素对肾病综合征血脂和血液流变学及肾功能的影响

目的:观察茶色素对肾病综合征(NS)患者的血脂、血液流变学及肾功能的影响。方法:将72例NS患者随机分成治疗组(38例)和对照组(34例)两组,对照组采用常规治疗,治疗组在常规治疗的同时,加用茶色素治疗,每日口服茶色素3次,每次240mg,共4周。对比观察两组治疗后血胆固醇(CH)、甘油三脂(TG)、高密度脂蛋白(HDL)、全血粘度高切变、中切变、低切变、血浆粘度、红细胞比积(Hct)、血纤维蛋白原(FI)、血小板最大聚集率(PMA)、血尿素氮(BUN)、血肌酐(Scr)的变化。结果:治疗4周后治疗组与对照组比较,CH、TG、LDL、PMA、Hct、FI、BUN、Scr、全血粘度低切变、血浆粘度较对照组均显著下降(P<0.05),HDL明显升高(P<0.05),而全血粘度高切变、中切变虽有所下降,但差别无显著性(P>0.05)。结论:茶色素对NS患者的血脂、血液流变学具有明显改善作用,对肾功能有一定的保护作用。     

难治性肾病综合征的病因及治疗研究进展

随着人类生活水平和健康意识的提高,各种慢性肾脏疾病(chronickidney diseases,CKD)的发病率也在不断上升,其发病率已达10%左右。临床上由于大量蛋白尿是导致肾功能恶化的独立危险因素,因此肾病综合征(NS)无论是原发性还是继发性,若大量蛋白尿得不到有效控制,将导致肾组织进行性纤维化,继而逐渐发展至终末期肾病。难治性NS(refractory ne-phritic syndrome,RNS)通常指常复发型、激素依赖、激素抵抗型或激素不耐受、激素使用禁忌的NS,     

陈以平教授以三焦论治老年肾病综合征验案三则

<正>我国老年慢性肾脏病(chronic kidney disease,CKD)的患病率高达30%~50%[1],肾病综合征(nephrotic syndrome,NS)是老年CKD的最主要临床表现,占33%~60%[2,3]。老年NS患者具有基础疾病多(高血压、糖尿病、动脉硬化、心肺功能不全等)、并发症明显(感染、急性肾损伤、栓塞、心脑血管疾病等)、易合并肾衰竭、肾穿刺接受度低、激素及免疫抑制剂疗效欠佳及副作用突出(如类固醇性糖尿病、感染、栓塞、诱发肿瘤、肝肾损害等)等特点[4~7],成为临床治疗难点。近年来越来越多学者主张对老年肾病患者制定激素及免疫抑制方案应持慎重、温和(避免过大剂量)及适可而止(疗程不易盲目延长)的态度,     

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.     

Immunoglobulins (IgG, IgA, IgM, IgE) and complement components (C3, C4) in nephrotic syndrome due to minimal change and other forms of glomerulonephritis, a clue for steroid therapy?

Serum IgG, IgA, IgM, IgE, C3 and C4 were measured in 13 patients with minimal change (MC) glomerulonephritis and 10 with the nephrotic syndrome (NS) due to other forms of glomerulonephritis. The tests were repeated in all patients with MC glomerulonephritis when they went into remission. Serum IgG was reduced, IgM, IgE and C3 were raised while serum IgA was within the normal range when the patients were nephrotic. Changes in serum immunoglobulins and complement components were not specific to MC glomerulonephritis and these parameters reverted towards normal when the NS went into remission. Elevated C3 levels probably reflected increased hepatic protein synthesis since C3 correlated significantly with serum cholesterol. There was a tendency for serum IgE concentrations to positively correlate with the total dose of prednisolone required to bring the NS to remission.     

Clinical study on the treatment of idiopathic membranous nephropathy with different dosage of corticosteroid combined with cyclosporine A

Objective To evaluate the efficacy and safety of different doses of prednisone combined with cyclosporine A(CSA) on the treatment of idiopathic membranous nephropathy (IMN). Methods The data of 64 patients with nephrotic syndrome (NS) diagnosed as IMN by renal biopsy were retrospectively analyzed. Median follow-up time was 10 (7, 19) months. The subjects were divided into 2 groups according to different prednisone dosage. Thirty-two cases were in the low-dose group: prednisone 0.15 mg?kg-1?d-1+CSA, and 32 cases in the moderate-dose group: prednisone 0.4-0.5 mg? kg-1?d-1 + CSA. Clinical and laboratory data were collected at baseline, 1, 3, and 6 months aftertreatment. During follow - up, cumulative recurrence rate and adverse reactions after treatment were recorded. Results Serum albumin (sALB) were significantly increased and 24 h urinary protein (24hUP) significantly decreased after treatment for 1, 3, 6 months compared with baseline data in the two groups. Serum creatine (Scr) increased after treatment with time. The elevation of sALB and the reduction of 24hUP in the moderate-dose group were higher than that of low-dose group at 6 months after treatment (P＜0.05). The effective rate of the low-dose and moderate-dose group was 65.6% and 87.5% at 6 months after treatment, respectively (χ2=4.267, P=0.039). Comparison of different doses of CSA in two groups at 6 months after treatment, in low-dose group: the effective rates of CSA＜3 mg? kg-1?d-1 and ＞3 mg?kg-1?d-1 subgroup were 76.5% and 53.3%, respectively (P=0.296); In moderate- dose group: the effective rates of CSA＜3 mg?kg-1?d-1 and ＞3 mg?kg-1?d-1 subgroup were 89.5% and 84.6%, respectively (P=0.077); there were similar effects in patients treated with different dose CSA in the two groups. About 20.4% of the total patients relapsed when followed up for 18 months (low dose group vs moderate - dose group: 9.5% vs 28.6% , P=0.136), which most occurred after prednisone withdrawal or during the reduction of cyclosporine. Renal function decreased in 57.8% patients (low dose group vs moderate-dose group: 50% vs 65.6%), mainly in the elderly (9/11) and the long course of treatment of CSA. There was no significant difference on adverse reactions between the two groups (P＞ 0.05). Renal function in patients with high Scr or high blood trough concentration of cyclosporine was difficult to fully recover. Conclusions Remission rate is lower in low-dose prednisone combined with cyclosporine than the moderate-dose group in the treatment of IMN for 6 months. The recurrence rate of IMN or the incidence of adverse reactions are similar between the two groups. Induction therapy of IMN with cyclosporin＜3 mg?kg-1?d-1 is safe and effective. The incidence of renal function reduction in the elderly is high, and the renal function is difficult to restore in patients with Scr exceeding normal upper limits.     

来氟米特治疗以肾病综合征为主要表现的IgA肾病的对照研究

目的探讨来氟米特治疗以肾病综合征为主要表现的IgA肾病患者的疗效及安全性。方法60例患者随机分为治疗组和对照组,每组30例。治疗组应用泼尼松联合来氟米特治疗;对照组应用泼尼松联合环磷酰胺冲击治疗。观察治疗前后24h尿蛋白定量,血浆白蛋白,肝、肾功能及治疗后药物的不良反应。结果治疗组与对照组的总有效率分别为86．7％和83．3％;不良反应总发生率分别为30．0％和73．3％。结论来氟米特联合泼尼松治疗以肾病综合征为主要表现的IgA肾病疗效可靠,不良反应发生少,为临床治疗提供的一种新的方法。     

Risk factors and outcome of focal and segmental glomerulosclerosis recurrence in adult renal transplant recipients.

BACKGROUND: Recurrence of nephrotic syndrome (NS) after renal transplantation for primary focal segmental glomerulosclerosis (FSGS) is a frequent and still unpredictable complication. However, risk factors for recurrence have not yet been clearly identified. METHODS: Data from 33 patients who underwent 35 renal transplantations for FSGS in two French centres are reported. RESULTS: Recurrent NS occurred in 12 transplant recipients (34%). A significantly higher number of patients in the group with recurrence (R group) compared with the group without recurrence (NR group) received cyclosporine for FSGS treatment before transplantation (83.3% vs 43.4%, P<0.02). Donors of R group recipients were significantly older than those of the non-NR group recipients (42.8 years vs 35 years, P<0.05). A higher number of patients from the R group required post-transplantation dialysis (33.3% vs 17.4%, P = 0.002). Surprisingly, acute rejection occurred more frequently in patients of the NR group compared with the R group, although the difference was not significant. Among the 12 patients with NS relapse, 9 were treated with plasmapheresis. Graft loss related to recurrence occurred in 6 cases. The 5-year graft survival was significantly lower in patients with recurrent NS compared with patients without recurrence (57% vs 82%, P<0.001). CONCLUSION: This study confirms the benefit to identify in the future clinical or biological predictive risk factors for NS recurrence after renal transplantation. It also indicates that donor age is a reliable risk factor for recurrence in adult recipients and suggests for the first time a possible opposite relationship between recurrent FSGS and acute rejection.     

98例特发性局灶节段性肾小球硬化的临床病理分析

目的：探讨温州地区特发性局灶节段性肾小球硬化（FSGS）的临床病理及流行病学特点。方法：对1993年2月～2007年9月间经我院肾内科病理室诊断的98例特发性FSGS进行回顾性总结,分析其临床表现、肾活检组织形态学及流行病学特点,进行临床与病理联系分析。结果：（1）98例特发性FSGS以20岁～45岁为发病高峰年龄（占59.1%）,占同期原发性肾小球疾病的3.6%及原发性肾病综合征的4.3%。（2）临床表现以肾病综合征（NS）最常见,占43例（43.9%）,发病时常并发高血压（49.0%）和慢性肾衰竭（35.7%）。肾衰竭组的肾病范围蛋白尿发生率、高血压的发生率及血尿酸水平明显高于肾功能正常组（P〈0.05）。（3）主要病理特征：76.5%患者伴有不同程度的肾小球球性硬化,其中球性硬化比例≥25%者占36.7%;82.7%患者伴不同程度的慢性肾小管间质病变,其中Ⅱ～Ⅲ级占15.3%。肾衰竭者Ⅱ～Ⅲ级肾小管间质病变比例较肾功能正常者高（28.6%vs10.0%,P〈0.05）。（4）肾小球球性硬化的比例与血清肌酐值、病程及年龄呈正相关（P〈0.05）,肾小管间质病变程度与Ccr呈负相关（P〈0.05）。球性硬化比例与肾小管间质病变程度呈正相关（P〈0.01）。结论：特发性FSGS在占本地区同期肾活检原发性肾小球疾病的3.6%,该病常并发高血压和慢性肾衰竭,病理上常见明显的肾小球球性硬化及慢性肾小管间质损害,慢性肾小管间质病变是影响患者预后的重要因素。     

263例成人原发性局灶节段肾小球硬化性肾小球肾炎临床病理分析

目的 了解我国南方地区原发性局灶节段肾小球硬化性（FSGS）肾小球肾炎的发病情况及探讨FSGS临床与病理特征的关系。方法 收集1988年7月至2005年7月经我院肾活检确诊为原发性FSGS的263例成人患者的临床及肾脏病理资料并进行分析。 结果 （1）原发性FSGS占同期成人原发性肾小球疾病 7.02%,占成人原发性肾病综合征（NS）6.33%，其构成比近年有逐渐升高的趋势。青壮年为成人FSGS的主要患病人群，临床以不同程度的蛋白尿为特征，以NS为主要临床表现的有133例，占50.6%。（2）主要病理特征： 48.4%患者肾小球硬化比率≥25%，肾小球硬化并伴有肾小管间质病变者占88.6%，其中伴严重肾小管间质病变占25.2%。（3）肾小球硬化程度及小管间质病变程度与Ccr呈负相关 (P ＜ 0.01），并与Scr水平呈正相关(P ＜ 0.05）。肾小球硬化程度与小管间质病变程度呈正相关(P ＜ 0.01），与血浆白蛋白水平呈正相关(P ＜ 0.05）。肾小管间质病变是FSGS患者出现肾功能不全的重要影响因素。结论 原发性FSGS是成人肾病综合征的主要病理类型之一，就诊时肾小球硬化及肾小管间质纤维化已损害明显，并与肾功能损害密切相关。早期诊断和及时治疗，从而延缓FSGS的进展仍是广大肾脏病工作者探索的重要课题。     

High incidence of focal segmental glomerulosclerosis in nephrotic syndrome of childhood

In recent adult literature, there have been reports of an increasing incidence of focal segmental glomerulosclerosis (FSGS) among patients with nephrotic syndrome. To examine whether this observation is also relevant to the pediatric population we utilized our hospital computerized database to analyze the data on children with primary nephrotic syndrome seen first between the years 1984 and 1995. A questionnaire was also sent to all metropolitan Kansas City pediatricians to identify possible patients outside the database. The inclusion criteria were clinical nephrotic syndrome or proteinuria with a kidney biopsy. A total of 148 patients (group A) were identified; 86 of them from metropolitan Kansas City (group B). In group A the incidence of minimal change disease (MCD) and FSGS was 52.7% [95% confidence interval (CI) 44%-60%] and 23.0% (95% CI 16-29%), respectively and in group B 54.7% (95% CI 44%-65%) and 24.5% (95% CI 15%-33%), respectively. Those numbers were significantly different from the International Study of Kidney Disease in Children (IS-KDC) reported incidence of 76.4% for MCD and 6.9% for FSGS. Similar to the ISKDC, in our population children over 6 years had a higher incidence of FSGS than younger children (32.8% vs. 16.7%, P = 0.028). The annual incidence rate for nephrotic syndrome in group B was 2.2 cases/10(5) children per year, of which MCD comprised 1.22 cases/10(5) children per year and FSGS 0.5 cases/10(5) children per year. The annual incidence rates of both primary nephrotic syndrome (3.6) and FSGS (1.6) were significantly higher in African-Americans, than Caucasians (1.8 and 0.3 cases/10(5) children per year, respectively). Our study indicates nearly no change in the annual incidence of pediatric primary nephrotic syndrome, but a higher incidence of FSGS with reciprocal decline in the incidence of MCD. The possibility of primary nephrotic syndrome being caused by a non-MCD entity is further raised among African-American and in children over 6 years. We conclude that our perception of primary nephrotic syndrome of childhood as a benign condition has to be carefully reexamined and a more-guarded prognostic approach adopted in our geographic area.     

Yellow nails and minimal change nephrotic syndrome.

We report a case of a 38-year-old man showing the yellow nail syndrome in association with minimal change nephrotic syndrome. Treatment with prednisone and vitamin E resulted in complete resolution of the nephrotic syndrome and slow improvement of the yellow nails, respectively. Although the rare yellow nail syndrome has been described in association with renal disease, this is the first report of the association of this syndrome with minimal change nephrotic syndrome.     

成人肾病综合征激素治疗的中医症候证型分析

目的 :探讨成人原发性肾病综合征使用激素治疗期间的中医症侯、证型特征及变化规律。方法 :观察并分析 2 2 0例成人原发性肾病综合症患者使用激素治疗期间的临床症候 ,辨证类型的变化。结果 :成人原发性肾病综合征患者使用激素治疗期间 ,本证方面 ,在大剂量时 ,辨证属阴虚 (73% )比使用激素前 (12 % )明显增多 ,属阳虚 (9% )和阴阳两虚 (19% )比使用激素前 (5 8%和 30 % )明显减少 ;小剂量时 ,辨证属阴虚 (5 0 % ) ,虽减少却也占 1/ 2 ,属阳虚(10 % )与大剂量时 (9% )相近 ,而阴阳两虚 (40 % )明显增加 ;在维持量时 ,阴虚证明显减少 (16 % ) ,阳虚和阴阳两虚证有所增加 (2 6 %和 4 6 % )。标证方面 ,在大、小剂量时 ,属热毒 (6 1%和 35 % )、湿热 (49%和 4 5 % ) ,比使用激素前 (15 %和 13% )明显增多 ,在整个激素使用期间 ,属水湿比用药前明显减低 ,而血瘀、气滞均比用药前增加。结论 :治疗成人原发性肾病综合征使用激素后 ,中医症候、证型有明显变化 ,其变化有一定的特点 ,本证的变化规律由阳虚或阴阳两虚→阴虚→阴阳两虚或阳虚转化 ;标证发病率由高至低为水湿、湿热、热毒→热毒、湿热、血瘀、气滞→血瘀、气滞转化 ,这种变化与用药剂量有关 ,正确辨证论治 ,有助于提高激素效果 ,减少不良反应     

Changes of physico-chemical characteristics of red blood cells in children with nephrotic syndrome

Changes of milieu interieur in nephrotic syndrome (NS) have many consequences in various organs. We have measured the electrical charge of erythrocytes (Ery) with binding of alcian blue (AB) in 18 children with relapse of NS (12 minimal changes, 3 membranous and 3 mesangioproliferative glomerulonephritis) and 15 healthy children. The most important finding was that the binding of AB to Ery in patients with minimal change nephrotic syndrome (MCNS) and other aetiologies of NS was significantly less than that in the control group (p<0.05). In addition, we have studied the thermal denaturation of the Ery membranes by differential scanning microcalorimetry. In some children with NS we have seen the splitting of B transition. We suppose that these phenomena occur as the result of structural change, which may involve lipoprotein components of the cytoskeletal network.