局灶节段性肾小球硬化诊治的进展

局灶节段性肾小球硬化 (ＦＳＧＳ)国外报道近年有增多趋势 ,国内因肾穿刺近年有较广泛的开展 ,使本症得以诊断 ,再兼治疗困难、预后较严重 (10年时30 %～ 4 0 %进入肾功能衰竭 )故引起儿科同道重视。今摘临床诊治中的某些进展述之如下。一、病因本症是一病理形态学诊断 ,指累及部分肾小球(<5 0 % )、部分毛细血管袢的硬化性改变 (肾小球毛细血管袢塌陷、基质增加 )。其始发因素尚不详 ,从临床角度除原发者外 ,尚可继发或伴发于多种情况(表 1)。表 1　ＦＳＧＳ的多种病因[1 3]原发性ＦＳＧＳ继发性ＦＳＧＳ1.继发于肾单位数量减少 :如一侧肾…     

儿童局灶节段性肾小球硬化

局灶节段性肾小球硬化（FSGS）近年来有增多趋势,FSGS不仅是一种形态学描述,而被视为一种临床病理综合征,表现为蛋白尿,常为肾病水平蛋白尿,并有局灶节段分布的肾小球硬化和足突融合。FSGS可为原发性（特发性）、继发性和遗传家族性。最近FSGS被区分为5种变异型,提示其不同的发病机制和预后,这5型包括特异FSGS、门周型、细胞型、顶端病变和塌陷型。该文还就FSCS的治疗和预后进行了讨论。     

局灶节段性肾小球硬化的研究进展

局灶节段性肾小球硬化的研究进展张敬京杨霁云局灶节段性肾小球硬化（ｆｏｃａｌｓｅｇｍｅｎｔａｌｇｌｏｍｅｒｕｌｏｓｃｌｅｒｏｓｉｓ，ＦＳＧＳ）是儿童及成人多种肾脏疾病持续进展至终末期肾病最多见的病理改变，也是肾移植后复发最常见的原因。本病虽早在１９２...     

局灶节段性肾小球硬化诊治现状

局灶节段性肾小球硬化(FSGS)临床主要表现为蛋白尿和肾病综合征,病理以局灶节段分布的肾小球硬化及足细胞的足突融合为特征,是导致终末期肾脏病的主要原因之一。由于FSGS病因复杂,发病机制也尚未明确,其诊断及治疗还面临很多困难。文章综述近年来FSGS的诊治现状。     

家族性局灶节段性肾小球硬化

局灶节段性肾小球硬化(focal segmental glomerulosclerosis,FSGS)是一种肾脏病理改变,以蛋白尿、肾病综合征和进行性肾功能损害为特征,是终末期肾病的常见原因.近年来,常染色体显性遗传和隐性遗传家族性局灶节段性肾小球硬化(familial FSGS)陆续报道,家族性FSGS基因的发现将有助于了解原发性FSGS的发病机制、分子基础和病理生理.文章总结了近年来有关疾病的临床特征、诊断、发病机制、治疗及预后的研究进展.     

原发性局灶节段性肾小球硬化的诊断与治疗策略

儿童局灶节段性肾小球硬化(FSGS)近年来有增多趋势,FSGS不仅是一种形态学描述,而且被视为一种临床病理综合征,多表现为肾病综合征,同时伴血尿和高血压,病变呈进行性,可继续发展为弥漫性硬化性肾小球肾炎,25%～30%的FSGS患儿5 a后进展至慢性肾衰竭,对激素治疗效果不理想。因此,早期诊断与及时治疗特别重要。现就原发性FSGS的诊断与治疗策略进行阐述。     

环孢素A治疗皮质激素耐药的儿童局灶节段性肾小球硬化疗效观察

目的观察环孢素A(CSA)治疗皮质激素耐药的儿童局灶节段性肾小球硬化(FSGS)的疗效。方法1997年6月至2004年10月中山大学附属第一医院儿科收治皮质激素耐药的FSGS患儿14例,应用CSA进行治疗,开始剂量为5mg/(kg.d),以后根据CSA血质量浓度调整剂量,维持CSA血质量浓度在150~300μg/L。有效者用药6个月至1年后逐渐减量。结果完全缓解10例(71.4%),部分缓解2例(14.3%),无效2例(14.3%),CSA治疗皮质激素耐药的FSGS总有效率达85.7%。CSA治疗期间泼尼松使用剂量减少。完全缓解10例中6例停药,其中5例复发,4例再用CSA治疗,尿蛋白再次转阴,1例放弃治疗。1例原有肾功能不全者治疗后血肌酐进行性升高。结论CSA治疗皮质激素耐药的FSGS有一定的疗效,但停药后复发率高,治疗中需注意CSA副反应。     

Desmin蛋白与局灶节段性肾小球硬化的相关性研究

目的 探讨局灶节段性肾小球硬化(FSGS)大鼠Desmin蛋白与足细胞损伤在FSGS发生发展中的作用.方法 测定阿霉素诱导的FSGS大鼠24 h尿蛋白定量(24 h UP)、血生化指标,计算内生肌酐清除率(Ccr);观察组织学变化并计算肾小球硬化指数(SI)、肾小球细胞外基质面积与肾小球面积之比(ECM/GA);免疫组织化学方法检测肾小球Desmin蛋白的表达,用图像分析软件测定肾小球Desmin蛋白表达的平均光密度值,并将FSGS大鼠肾小球Desmin蛋白的表达与以上指标进行相关性分析.结果 FSGS组大鼠的24 h UP、胆固醇、SI及ECM/GA明显高于正常对照组(P<0.001),白蛋白、Ccr较正常对照组明显降低(P<0.001);FSGS组大鼠Desmin蛋白的表达上调.Desmin蛋白的表达变化与24 h UP、SI及ECM/GA呈正相关(r=0.899～0.930,P均<0.05),与Ccr呈负相关(r=0.927,P<0.01),与白蛋白、胆固醇无相关性(r=0.371、0.710,P均>0.05).结论 大量蛋白尿及内生肌酐清除率下降可以作为肾小球足细胞损伤的重要特征,足细胞数量是决定FSGS的主要因素之一.     

局灶性节段性肾小球硬化的研究现状

局灶性节段性肾小球硬化是儿童期肾功能衰竭的主要病因之一，发病机制复杂，目前有关研究认为肾小球脏层上皮细胞的数目及结构的改变导致蛋白尿及肾小球硬化。局灶性节段性肾小球硬化的临床治疗较为困难，目前无一种方案的应用可取得完全缓解，早期联合、多环节针对发病机制用药的综合治疗方案可取得较好的缓解率。     

儿童原发性局灶节段性肾小球硬化212例临床病理分析

目的 探讨小儿原发性局灶节段性肾小球硬化(FSGS)的临床表现、肾脏病理和治疗的疗效特点,以减少或延缓终末期肾病(ESRD).方法 对近10年病理确诊的原发性FSGS患儿212例进行了回顾性分析,依据2004年D'Agati原发FSGS组织病理学新分型标准,将患儿分为非特异型、门部型、细胞型、顶部型、塌陷型共5个亚型,比较不同病理分型与其临床表现、实验室指标及治疗的疗效、分析评估病理类型与预后的关系.结果 原发FSGS临床诊断以肾病综合征最多178例(83.9%)[其中单纯型97例(45.8%),肾炎型81例(38.2%)];肾病水平蛋白尿14例(6.6%);蛋白尿或伴镜下血尿20例(9.4%).肾组织病理学分型以非特异型最多86例(40.6%),门部型25例(11.8%),细胞型58例(27.4%),顶部型31例(14.6%),塌陷型12例(5.6%).其临床病理的相关性表明,顶部型多呈单纯型肾病,塌陷型多呈肾炎型肾病,此两种亚型之间差异有统计学意义(P＜0.05),细胞型多为小婴儿,临床类型介于两者之间.本组患儿对激素的反应较差,顶部型及非特异型激素初治可敏感,但后多为频复发、依赖或转为耐药的病例;塌陷型、门部型和细胞型多为原发耐药的病例;顶部型与塌陷型两者差异有统计学意义(P＜0.05).激素联合免疫抑制剂治疗,50%以上获得完全缓解.塌陷型2年肾存活率为67%,3年肾存活率为41%.结论 原发FSGS病理分5种亚型,临床主要表现为不同程度的蛋白尿、部分伴有血尿和肾功能不全;顶部型治疗反应较好,塌陷型治疗反应及预后较差.     

儿童原发性局灶节段性肾小球硬化症治疗进展

局灶节段性肾小球硬化症(FSGS)是儿童常见肾小球疾病,临床多表现为肾病综合征.糖皮质激素是FSGS的一线治疗药物,但相当一部分患儿对激素治疗反应不佳,最终进展至终末期肾病,因此各种新型FSGS治疗药物不断开发,并取得一定疗效.文章综述儿童原发性FSGS的治疗进展.     

Low-dose methylprednisolone pulse therapy in Chinese children with steroid resistant focal segmental glomerulosclerosis

BACKGROUND: Focal segmental glomerulosclerosis (FSGS) is a primary glomerular disease that usually progresses to renal failure. Although high-dose pulse methylprednisolone therapy (PMT) has been shown to be effective in the treatment of steroid-resistant FSGS, adverse effects have caused parents to hesitate in approving the treatment. The aim of this study is to investigate whether low-dose PMT based protocol for treatment of young children with steroid resistant FSGS would effectively induce remission of proteinuria and prevent the progression of renal insufficiency. METHODS: This is a retrospective study. The authors treated eight children with steroid-resistant FSGS with intravenous methylprednisolone pulse 10 mg/kg per day for three consecutive days weekly for 8 weeks. Partial responders were treated with the addition of chlorambucil or cyclosporine (CsA) and four fortnightly and eight monthly pulses of high-dose PMT (30 mg/kg per day). RESULTS: Of the eight patients, six attained complete remission initially. The median urinary protein excretion in 24 h decreased from 4.25 to 0.39 g following 8 weeks of low dose (P = 0.012). Marked decrease in urinary protein-creatinine ratio was noted soon after treatment (P = 0.012). There was a significant increase in serum albumin level after treatment compared to the pretreatment condition (median, 3.35 vs 4.1 mg/dL, P = 0.018). Five of the eight patients remained in complete remission, and one of the eight patients relapsed during follow up. Relapse responded to repeated treatments of PMT and cyclosporine. The two patients with partial remission initially progressed to renal insufficiency in one patient and end-stage renal disease in the other patient. CONCLUSIONS: Low-dose PMT caused a significant decrease in the proteinuria of Chinese children with steroid-resistant FSGS with a low frequency of intolerance.     

Outcome of living donor renal allograft survival in children with focal segmental glomerulosclerosis

Abstract:  FSGS is the most frequent GN that may recur in a renal allograft. Compared with adults, the impact of FSGS on graft survival appears to be more significant in children. Thus we decided to assess graft survival and complications after renal transplantation in children with FSGS. Outcome of renal transplantation in 25 children with FSGS who received a renal transplant at Labafi Nejad Hospital was studied and compared with 75 patients as a control group. The mean follow-up duration was 68.16 (s.d. = 41.93) months. Other than demographics, variables such as DGF, acute rejection, number of acute rejection episodes, and graft failure in both groups were evaluated. Acute rejection was seen in 22/25 (88%) of FSGS group, compared to 40/75 (53.3%) in the control group. This difference was statistically significant (p = 0.001). DGF was seen in 4/25 (16%) and 13/75 (17.3%) in the FSGS and control groups, respectively (p = N.S.). The mean graft survival time was 115.61 (s.e.m. = 12.56) and 155.56 (s.e.m. = 7.16) month in FSGS and control group, respectively (p = N.S.). We demonstrated that graft function and survival were not significantly different in the FSGS and control patients. However, acute rejection episodes were more common in FSGS patients but without a significant impact on graft survival.     

Outcome of renal transplantation in adolescents with focal segmental glomerulosclerosis

Using the NAPRTCS database from January 1987 to January 2001, we examined 2687 adolescent (age 13-17 yr) index renal transplants to analyze differences in demographic treatment, and outcomes in adolescents with FSGS compared to other renal disease. 338 (12.6%) of adolescents had a primary diagnosis of FSGS. Adolescents with FSGS were more likely to be black and less likely to receive pre-emptive transplants (p < 0.001). No differences existed in HLA matching or immunosuppression regimens. Acute tubular necrosis occurred in more FSGS adolescents compared to non-FSGS adolescents following LD (11% vs. 4.7%) or CD (25.1% vs. 17.8%) transplants (p < 0.001). There were no significant differences in acute rejection rates between adolescents with FSGS and other adolescents. Graft survival was worse for LD FSGS adolescents (6 yr, 56%) compared to non-FSGS adolescents (77%) (p < 0.001) and was not significantly different from CD graft survival in FSGS (51%) or non-FSGS groups (61%). The relative risk (RR) of graft failure was greatest in LD transplant with FSGS (RR = 1.75; p < 0.001), compared to LD transplants without FSGS (RR = 1.0). Recurrent primary disease accounted for 15.2% of all graft failures in adolescents transplanted for FSGS with no difference between LD (17%) or CD (13.8%) grafts. Recurrent disease accounted for 3.2% of graft failures in adolescents without FSGS. Recurrent disease was the only cause of graft failure that differed between groups (p < 0.001). When compared to patients up to age 12 yr with FSGS, graft survival in both LD and CD transplants was worse in adolescents with FSGS (LD p = 0.035, CD p < 0.001). In conclusion, FSGS has a negative impact on graft survival in adolescents. Recurrence of FSGS results in a loss of the expected LD graft survival advantage in adolescents. Furthermore, adolescents with FSGS have decreased graft survival compared to younger children with FSGS. These data suggest that the rationale for LD transplantation in adolescents with FSGS should be based on factors other than the increased graft survival typically seen with LD transplantation.