利妥昔单抗治疗儿童原发性肾病综合征研究进展

原发性肾病综合征(PNS)是儿童时期常见的肾小球疾病,约25%的频复发型肾病综合征(FRNS)或激素依赖型肾病综合征(SDNS)患儿在成年期仍需用激素和(或)免疫抑制剂(ISA)维持治疗;约35%的激素耐药型肾病综合征(SRNS)患儿加用免疫抑制剂仍不缓解.利妥昔单抗是一种人鼠嵌合型抗CD20单克隆抗体,作为PNS的三线药物用于治疗对ISA如环磷酰胺、环孢素、他克莫司、霉酚酸酯等反应不佳、且不良反应严重的FRNS/SDNS和SRNS患儿,已取得较好的近期疗效,其远期疗效及安全性尚需多中心、前瞻性、大样本的随机对照试验证实.     

利妥昔单抗对儿童难治性肾病综合征的治疗进展

难治性肾病综合征是导致儿童终末期肾脏病的主要原因之一,也是临床治疗的棘手问题.虽然包括免疫抑制剂在内的多种治疗方法已经对儿童难治性肾病综合征表现出了良好的治疗效果以及安全性,但是仍有很多患儿不能获得缓解.近年来,新型免疫抑制剂利妥昔单抗用于治疗难治性肾病综合征取得了较好效果,很多病例分析以及临床试验对利妥昔单抗治疗儿童难治性肾病综合征的有效性进行了报道.该文就该药治疗儿童难治性肾病的疗效和安全性等作一综述.     

利妥昔单抗治疗儿童难治性慢性特发性血小板减少性紫癜9例近期疗效

目的观察利妥昔单抗治疗儿童难治性慢性特发性血小板减少性紫癜(ITP)的近期疗效。方法选择我院2008年6月-2010年6月期间确诊为难治性、慢性ITP患儿9例,给予利妥昔单抗100 mg静脉滴注,每周1次,连用4周。结果治疗起效时间1~6周,有效率为67%,复发率为33%,2例切脾患儿无效。结论利妥昔单抗治疗儿童难治性、慢性ITP起效快、缓解率较高、毒副作用小,但有复发倾向。     

利妥昔单抗治疗儿童难治性血小板减少性紫癜合并乙肝病毒感染1例报告及文献复习

目的探讨利妥昔单抗治疗儿童难治性血小板减少性紫癜(rITP)合并乙肝病毒感染的疗效与安全性。方法利妥昔单抗(375 mg/m2,每周1次,连用4周)治疗1例儿童rITP合并既往乙肝病毒感染(Anti-HBs+、Anti-HBe+、Anti-HBc+),同时口服拉米夫定(100 mg/d,1次/d,6个月),监测患儿血小板、免疫球蛋白和乙肝病毒DNA定量的变化,并进行相关文献复习。结果随诊时间6个月,血小板始终>100.0×109/L,乙肝病毒无异常复制。结论在预防应用抑制乙肝病毒复制药物的情况下,利妥昔单抗治疗儿童rITP合并乙肝病毒感染是安全、有效的。     

利妥昔单抗治疗难治性激素耐药型肾病综合征的疗效和安全性

目的:探讨利妥昔单抗(RTX)治疗儿童难治性激素耐药型肾病综合征(SRNS)的疗效和安全性。方法:回顾性分析2013年9月至2018年3月东部战区总医院儿科收治并接受RTX治疗的10例难治性SRNS患儿的临床资料。结果:10例患儿发病年龄(4.47±2.75)岁,男女各5例;5例(50%)肾活检为局灶节段性肾小球肾炎,...     

利妥昔单抗联合地塞米松治疗小儿难治性特发性血小板减少性紫癜临床效果观察

目的探讨应用利妥昔单抗(美罗华)联合地塞米松治疗儿童难治性特发性血小板减少性紫癜(RITP)的疗效及安全性。方法选择诊断为RITP的患儿22例,按照治疗方法不同分为对照组(大剂量地塞米松冲击治疗)和观察组(美罗华联合小剂量地塞米松治疗)。治疗结束后,对两组患儿进行疗效评价、观察记录药物不良反应,并应用流式细胞术测定11例观察组患儿治疗前后CD20+B细胞的数量变化。结果观察组临床疗效优于对照组,差异有显著性(P<0.05),治疗期间两组不良反应比较无明显差异,美罗华治疗后,有效组患儿外周血小板数量较治疗前明显升高,外周血CD20+B细胞数量较治疗前显著降低。结论美罗华联合地塞米松治疗RITP疗效确切,毒副作用较小。     

利妥昔单抗在儿童硬化性肾炎中的应用

目的研究利妥昔单抗在儿童硬化性肾炎中的临床应用及意义。方法 1例硬化性肾炎患儿,使用利妥昔单抗前检查CD19、CD20、CD3、CD4、CD8、免疫球蛋白定量、补体、血常规、肝肾功能等指标,用药前半小时静脉推注非那根0.5～1 mg/kg,用药前5～10 min静脉推注地塞米松0.1～0.3 mg/kg;利妥昔单抗375 mg/m2稀释于5%葡萄糖溶液300 ml,静脉滴注。1个月后第2次应用,共2次。结果利妥昔单抗第1次静脉滴注后2周眼睑浮肿与双下肢浮肿减轻,1个月检查尿蛋白减少[尿蛋白2+,24 h尿蛋白定量0.68 g/L,尿NAG酶17.2 U/(g.cr)],血清白蛋白增加,血白细胞与肝肾功能无影响;第2次静脉滴注后1周眼睑浮肿与双下肢浮肿消失;3个月复查尿蛋白明显减少[尿蛋白1+,24 h尿蛋白定量0.32 g/L,尿NAG酶13.4 U/(g.cr)],血清白蛋白(35.2 g/L)、胆固醇(5.4 mmol/L)均恢复到正常,Th减少(CD4+CD3+25.0%),B淋巴细胞减少(CD8+CD3+36.0%,Th∶Ts 0.69;CD19+6个/μl,CD19+Gate 0.3%,CD20+6...     

低剂量利妥昔单抗治疗儿童肾病综合征疗效及安全性的前瞻性随机对照研究北大核心CSCD

目的对比利妥昔单抗(rituximab,RTX)低剂量(200 mg/m^(2))与推荐剂量(375 mg/m^(2))重复使用治疗频复发型肾病综合征(frequently relapsing nephrotic syndrome,FRNS)或激素依赖型肾病综合征(steroiddependent nephrotic syndrome,SDNS)维持缓解的疗效和安全性。方法采用随机对照试验研究法,选择2020年9月—2021年12月在安徽省儿童医院儿童肾脏科接受系统治疗的29例FRNS/SDNS患儿为研究对象,按随机数字表法分为推荐剂量组(n=14)和低剂量组(n=15)。分析比较两组患儿的一般特征、RTX治疗前后CD19变化、复发次数、糖皮质激素使用剂量、RTX不良反应及住院费用等差异。结果与治疗前相比,低剂量组与推荐剂量组在应用RTX后均能使B淋巴细胞耗竭,且复发次数减少、糖皮质激素使用剂量下降(P<0.05)。低剂量组RTX治疗后的临床疗效与推荐剂量组相当(P>0.05);而低剂量组第2~4次住院费用下降(P<0.05)。两组在使用RTX过程中及后期随访中无严重不良反应,发生的不良反应差异无统计学意义(P>0.05)。结论低剂量与推荐剂量重复RTX治疗临床疗效和安全性相当,可显著降低FRNS/SDNS复发次数及糖皮质激素使用剂量,在整个治疗周期内无明显不良反应,适合临床推广。     

激素敏感型肾病综合征儿童利妥昔单抗治疗不良事件的系统评价/Meta分析

背景：近20多年来利妥昔单抗（RTX）应用于儿童激素敏感型肾病综合征（SSNS）的治疗较其他免疫抑制剂有更好的疗效,但仍需要积累不良事件的报告情况。 目的：了解儿童SSNS应用RTX治疗后的不良事件。设计：系统评价/Meta分析。方法：检索PubMed、Embase、Cochrane、Scopus和中国生物医学文献服务系统数据库,检索时间为建库至2022年6月26日,以SSNS、RTX构建中英文数据库检索式。同一篇文献初筛、全文筛选和证据提取均由2人完成,有争议和不确定的文献由第3人复核。纳入至少1组干预措施使用RTX治疗1~22岁SSNS患儿的研究。主要结局指标：不良事件发生率。结果：共纳入47篇文献（中文5篇,英文42篇）,7篇双臂干预研究［RCT 5篇,非随机对照试验2篇］和40篇病例系列报告。RTX组较安慰剂或常规免疫抑制剂（对照组）治疗SSNS患儿不良事件发生率（130/184 vs 107/177）、严重不良事件发生率（14/140 vs 9/122）和输液相关反应发生率（19/24 vs 13/24）差异均无统计学意义;感染发生率（56/80 vs 41/62）差异有统计学意义（OR=3.99, 95%CI:1.23~12.97）。RTX治疗SSNS病例系列报告中,不良事件发生率59%（95%CI:55%~63%）,严重不良事件发生率7%（95%CI:6%~9%）,输液反应发生率31%（95%CI:28%~35%）,感染发生率21%（95%CI:18%~24%）,血清病发生率5%（95%CI:2%~10%）,同时报告外周血中性粒细胞减少和缺乏的研究中,中性粒细胞减少发生率9%（95%CI:5%~17%）、缺乏发生率4%（95%CI:2%~10%）,针对低IgG血症研究的低IgG血症发生率为51%（95%CI:42%~60%）。结论：RTX治疗SSNS未增加不良事件、严重不良事件和感染的发生率,低IgG血症和血清病值得关注。     

频复发/激素依赖肾病综合征儿童利妥昔单抗治疗1年以上随访情况的系统评价/Meta分析

背景：权威指南和共识均推荐将利妥昔单抗（RTX）应用于儿童激素敏感型肾病综合征（SSNS）中频复发/激素依赖肾病综合征（FRNS/SDNS）的治疗,但仍存在临床适应证不统一、治疗和随访方案多样等问题。目的：了解RTX首疗程治疗缓解期FRNS/SDNS随访1年以上复发和激素使用情况结局。设计：系统评价/Meta分析。方法：检索PubMed、Embase、Cochrane、Scopus和中国生物医学文献服务系统数据库,从建库至2022年6月26日,以SSNS、FRNS、SDNS和 RTX构建中英文数据库检索式。同一篇文献初筛、全文筛选和证据提取均由2人完成,有争议和不确定的文献由第3人复核审查。纳入至少1组干预措施使用RTX治疗1~22岁SSNS患儿的研究。主要结局指标:RTX干预后随访≥1年的复发率、首次复发时间,激素累积剂量和停用比例。结果:符合本文临床结局的文献26篇（RCT 8篇、非随机对照试验 1篇、队列研究8篇、病例系列报告9篇）,中文文献1篇,英文文献25篇。基于FRNS/SDNS病例的随访≥1年复发率的9项研究的Meta分析显示,RTX较对照组复发率下降了78%(OR=0.22,95%CI:0.09~0.53),在FRNS/SDNS+（RTX干预前已使用其他免疫抑制剂）亚组病例中,RTX较对照组复发率下降了67%(OR=0.33,95%CI:0.12~0.94),在FRNS/SDNS-（RTX干预前未使用其他免疫抑制剂）亚组病例中,RTX-（不联用其他并免疫抑制剂）较对照组复发率下降了85%(OR=0.15,95%CI:0.03~0.68)。基于20项研究的Meta分析显示,RTX复发率42% (95%CI:32%~53%)。基于FRNS/SDNS+随访≥1年首次复发时间的9项研究的Meta分析显示,首次复发时间9.89（95%CI: 7.14~12.65)月。基于FRNS/SDNS-开始干预至随访≥1年中位首次复发时间的3项研究的 Meta分析显示,RTX（1~2剂）较对照组中位首次复发时间长20 d,中位生存比（MSR）为0.69(95%CI:0.52~0.87）。基于FRNS/SDNS的12个月激素累积剂量减少结局的4项研究的Meta分析显示,RTX较对照组年激素累积剂量减少明显,差异有统计学意义(SMD=-1.12,95%CI:-1.49~-0.74)。基于FRNS/SDNS的随访3个月激素停用率的2项研究的Meta分析显示,RTX是对照组（CNI或CTX）随访3个月激素停用率的14.6倍 (OR=14.62,95%CI:5.43~39.39)。基于FRNS/SDNS+的RTX治疗6个月停用激素率的3项研究的Meta分析显示,停用激素率68%（95%CI:56%~79%）。结论:与对照组相比,RTX从随访1年的首次复发时间中获益有限,可从激素减量中获益但不能从停用激素率中获益。RTX治疗FRNS/SDNS随访12个月较安慰剂治疗或空白对照至少可降低88%的复发率,FRNS/SDNS接受RTX治疗随访1年复发率43%。RTX治疗FRNS/SDNS+可获得10个月的无复发生存时间。     

儿童特发性膜性肾病诊疗进展

膜性肾病(MN)是一种免疫复合物介导的肾小球疾病,大多表现为肾病综合征.儿童MN多为继发性MN,最常继发于乙肝病毒感染或系统性红斑狼疮,近年来儿童特发性MN的发病率有增加趋势.M型磷脂酶A 2受体(PLA 2 R)抗体和1型血小板反应蛋白7 A域(THSD 7 A)抗体最初在成人中发现,可作为成人特发性MN诊断、判断疾...     

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目的探讨儿童特发性膜性肾病(IMN)的临床和病理特征。方法 1999年7月至2009年7月在中山大学附属第一医院确诊的IMN患儿13例,回顾性分析其临床病理特征、治疗及转归情况。结果 13例IMN患儿中男11例,女2例;就诊时中位年龄为11.4(3.0～14.5)岁;肾穿时中位病程为3.7(1.4～65.3)个月。确诊时临床表现为肾病综合征(NS)9例(单纯型1例,肾炎型8例),血尿蛋白尿3例,复发性肉眼血尿1例,伴高血压1例(7.7%)。所有患儿的肾功能均正常。肾脏病理结果:(1)光镜病理分期:Ⅰ期4例,Ⅱ期9例。4例伴肾小球球性硬化(球性硬化肾小球百分比分别为11.8%、2.9%、7.5%、4.5%),1例伴节段性硬化(节段硬化肾小球百分比为4.5%),3例伴肾小管萎缩,1例伴细胞纤维性新月体形成(3.8%)。(2)免疫荧光:以IgG、C3在肾小球基底膜(GBM)上皮下沉积为主(前者12/12例、后者10/12例),少数伴IgM、Fg、C1q和IgA沉积,但沉积强度较IgG、C3弱。(3)电镜:13例患儿GBM均有不同程度增厚,2例毛细血管腔内出现微血栓。根据患儿临床表现及病理分期予激素和(或)免疫抑制剂...     

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??Objective??To summarize the clinicopathological features??treatment and prognosis of childhood idiopathic membranous nephropathy??IMN??. Methods??The clinical manifestations??pathologic features??treatment and prognosis of 22 IMN children who were diagnosed by pathology from January 2005 to February 2017 were retrospectively analyzed. Results??Twenty-two children??12 males and 10 females?? had an onset age range of 3 to 15 years. Clinical manifestations??hematuria and proteinuria in 4 cases??18.18%????nephrotic syndrome??NS?? in 18 cases??81.82%??. Renal biopsy results??8 cases??36.36%?? in stage??5 cases??22.73%?? in stage ??-??5 cases??22.73%?? in stage ??1 case??4.54%?? in stage ??-??atypical membranous nephropathy in 3 cases??13.64%??. Among them??12 cases??54.54%?? of M-type phospholipase A2 receptor??PLA2R?? positive were detected and 83.33%??10/12?? PLA2R positive children with an onset age older than 10 years old. Immunofluorescence showed predominantly IgG and C3 deposition. Treatment programs??in the 4 cases whose clinical manifestations were hematuria and proteinuria??2 cases were given glucocorticoid and ACEI drug treatment??the other 2 cases were given ACEI drugs alone??and they had complete remission. The 18 patients with NS all were treated with glucocorticoid and ACEI??and immunosuppressive agents were given to steroid-resistant patients. During the follow-up??18 cases??81.82%?? were completely relieved??3 cases??13.64%?? were partially relieved??and 1 patient??4.54%?? was lost of follow-up. Conclusion??Clinical manifestations of IMN are mostly nephritis nephrotic syndrome??mainly steroid-resistant nephrotic syndrome. Pathological performance mainly is??-??stage. The positive rate of PLA2R in junior age was lower than adolescence. Glucocorticoid combined with immunosuppressive agents has a higher response rate.     

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??Abstract?? Objective To investigate the children with idiopathic membranous nephropathy??IMN?? about the clinical and pathological characteristics?? efficacy and prognosis?? providing the reference for the clinical diagnosis and treatment.Methods Summarize and analyze the clinical manifestation?? pathological features?? treatment and prognosis of 20 cases of IMN patients in Paediatric Nephrology of Shengjing Hospital of China Medical University between 2006.2 and 2014.2. Results ??1??In 20 cases of children with IMN??there were 13 male cases and 7 female cases, male to female ratio 1.86??1. The age ranged from 4 to 14 years?? the mean age being 10.65 ± 3.18 years??from onset to renal biopsy the was 7 to 190 days??with an average of 59.1 ± 55.9 days.There were 5 cases of below nephrotic proteinuria in clinical manifestations?? ??including simple proteinuria in 1 case?? hematuria and proteinuria in 4 cases????and 15 cases of nephrotic proteinuria?? of which 1 case with acute renal insufficiency.??2??Renal pathology:light microscopy showed that there were 2 cases of stage I membranous nephropathy??17 cases of stage II membranous nephropathy and 1 cases of stage III membranous nephropathy??among which 16 cases were with mild mesangial cells proliferation and mesangial matrix increase??4 cases with partial glomerular sclerosis??percentage of glomerular sclerosis was 2.4%??3.4%??3.7% and 5.5%??respectively????and 7 cases with focal tubular atrophy and interstitial fibrosis.Immunofluorescence showed there was IgG and C3 deposition?? partly with IgM??Fg??C1q??and IgA deposition.??3??According to the level of proteinuria the patients were treated with glucocorticoid and renin angiotensin converting enzyme inhibitor??ACEI?? or combined with immunosuppressive therapy with a result of 12 cases completely relieved and 8 parthy relieved.??4??In the 19 cases followed up for 2-7 years??12 cases obtained complete remission?? of which 2 cases replased in 1.5 year and 2 years after drug withdrawal. Eight cases had partial remission??of which 7 cases were still receiving sequential treatment??while 1 case lost follow-up because of refusing the use of immunosuppressant. Conclusion The onset of IMN is mainly in elder children and male is more than female.Nephrotic syndrome is the major clinical manifestations of IMN. Glucocorticoids combined withimmunosuppressive drugs can achieve satisfactory therapeutic effect.     

儿童原发性膜性肾病16例病理特点与远期预后

目的 了解儿童原发性膜性肾病(IMN)病理特点及其远期预后。方法 回顾性收集1979至2010年6月复旦大学附属儿科医院肾脏风湿科经病理诊断为IMN的连续病例为研究对象,分析一般情况、临床表现、病理特点、治疗和随访情况,探讨其远期预后。结果 16例IMN患儿进行分析,占同期肾穿刺活检病例（1 710例）的0.94%。男10例,女6例,年龄2～12岁,平均（5.2±2.6）岁。①临床表现以肾病综合征为主（11例,68.8%）,无症状性蛋白尿5例（31.2%）,伴有高血压2例(12.5%）,起病时伴有肾功能不全2例（12.5%）。②14/16例行肾组织电镜检查,其中Ⅰ期6/14例（42.9%）,Ⅰ~Ⅱ期6/14例（42.9%）,Ⅱ期1/14例（7.1%）,Ⅱ~Ⅲ期1/14例（7.1%）。病理学检查均未见肾小管萎缩、间质纤维化等肾小管间质损伤。③未达到大量蛋白尿标准的7例患儿予随访观察,其中1例病情进展予激素和免疫抑制剂治疗;达到大量蛋白尿标准的9例患儿均予足量激素(2 mg·kg-1·d-1)治疗,其中5例激素依赖或耐药加用免疫抑制剂治疗。至2010年6月,2例失访,14例IMN患儿随访12~91个月,平均（34.0±18.7）个月,在起病后3~16个月均达完全缓解,无一例进展至慢性肾脏疾病;2例起病时伴肾功能不全的患儿肾功能均恢复。结论 儿童IMN临床表现以肾病综合征为主,小年龄、病理分期较轻且不伴肾小管间质损伤患儿的预后相对较好。     

儿童特发性膜性肾病临床病理特点及治疗探讨

目的 了解儿童特发性膜性肾病(IMN)的临床病理特点,探讨其治疗方案.方法 回顾性分析25例病理确诊的IMN患儿的临床病理特点,总结其不同治疗方法 的疗效.结果 儿童IMN占同期所有肾穿刺活检(简称肾穿)患儿的3.81%.25例IMN中男9例,女16例;起病年龄2～14岁,平均(9.4±3.4)岁;肾穿时病程0.4～11.0个月,中位数2.5个月.临床表现为肾病综合征肾炎型21例(84%),肾小球肾炎4例(16%).全部患儿均伴血尿,其中肉眼血尿7例,高血压4例,并发血栓2例,肾功能不全1例.病理分期IMNⅡ期21例(84%).伴中重度小管间质损害者6例,伴局灶节段硬化2例.22例肾病综合征及肾病水平蛋白尿患儿中,21例首选糖皮质激素治疗,其中20例符合评价激素疗效标准:激素敏感1例(复发后转为激素耐药),19例为激素耐药(95%).后续治疗包括继续单纯激素减量隔日治疗8例,其中完全缓解5例,部分缓解3例;激素联合免疫抑制剂治疗12例,该12例连同首选联合免疫抑制剂治疗1例、激素治疗5周联合免疫抑制剂治疗1例,共14例.结论 本组患儿IMN临床表现以肾病综合征为主,均伴有不同程度血尿.绝大多数初治激素耐药,但部分病例减量隔日治疗过程中获缓解,联合免疫抑制剂治疗及疗效尚需进一步临床验证.

Abstract：

Objective To investigate the clinicopathological feature and treatment of idiopathic membranous nephropathy(IMN)in children.Method A retrospective analysis of 25 cases of biopsyproven IMN seen between January 2004 and December 2009.Result The incidence of IMN was 3.81% in all the children patients who underwent renal biopsy.Of 25 patients with IMN,nine were boys and sixteen were girls.The mean age at onset was(9.4±3.4)years with a range of 2-14 years.Renal biopsies were performed at a median 2.5 months(range 0.4-11 months)after onset.The clinical manifestations included nephrotic syndrome(NS)nephritic type in 21 cases(84%)and glomerulonephritis in 4 cases.All patients presented with hematuria,and 7 had macroscopic hematuria.Hypertension was noted in 4 patients.Two patients were complicated with thrombosis.One patient was in a chronic renal insufficiency(CRI)state.According to the MN staging criteria,21 cases were in stage Ⅱ IMN(84%).Six patients showed moderate or severe tubulointerstitial lesion.Focal segmental glomerulosclerosis(FSGS)was found in two patients.Of the 22 patients with NS and nephrotic proteinuria,21 cases were treated with prednisone initially and in 20 of them the efficacy of corticosteroid therapy was evaluated:one of them was steroid sensitive(became steroidresistant after relapse)and all the others were steroid-resistant(95%).The subsequent treatment:eight of them were treated with prednisone followed by a taper to alternate-day therapy.Five of them had complete remission and three partial remission.Twelve cases were treated with combined therapy of prednisone and immunosuppressive agents. Of these 12 cases together with one case who received initially combined treatment with prednisone and immunosuppressive agent and one case treated with prednisone initially for five weeks then with combined therapy contained another immunosuppressive agent,totally 14 cases,5 had complete remission,2 partial remission,3 did not achieve remission,and 3 had unknown response.Conclusion Of the patient cohort,the predominant presenting feature was nephrotic syndrome,and with different degree hematuria.Almost all of them were steroid resistant,but followed by a taper to alternate-day therapy,some could achieve remission.The effect of a combination of prednisone and immunosuppressive agent is needed to be further proven in children.     

Clinicopathological characteristics of the focal and segmental form of idiopathic membranous nephropathy: Comparison with the typical form of this disease

Although idiopathic membranous nephropathy (IMN) is thought to represent a diffuse glomerulopathy, it was found that three of 31 children histologically diagnosed as IMN showed focal and segmental deposition of immunoglobulin G (IgG) and C3 on the glomerular capillary walls. The present study attempted to comparatively investigate clinical and pathological features of the diffuse group and the focal segmental group in 31 IMN children. Immunofluorescence study revealed that 28 of 31 IMN exhibited diffuse granular deposition of IgG along glomerular capillary walls. In contrast, focal and segmental deposition of IgG and C3 was noted in three children with IMN. In addition, focal and segmental electron-dense deposits were identified in these cases. In two children of the focal segmental group, immunofluorescent patterns of IgG deposition were unchanged even at the second biopsy. The focal segmental form of IMN tended to occur in younger children than diffuse IMN. However, other clinical parameters such as the range of proteinuria, hematuria, serum albumin and prognosis did not show any significant differences between both groups. Electrophoretic profiles of urinary proteins on sodium dodecylsulfate-polyacrylamide gel electrophoresis were not different between both groups. It is proposed that the focal segmental form of IMN may have a distinctive glomerulopathy from the typical form of IMN.     

儿童原发性膜性肾病研究进展

原发性膜性肾病(PMN)是儿童期罕见的一种肾病病理类型,临床表现为肾病综合征或无症状蛋白尿,常伴镜下血尿、氮质血症或高血压;病理特征为肾小球基底膜增厚伴或不伴钉突形成,颗粒状IgG和补体C 3沿肾小球毛细血管襻沉积.近年来儿童PMN发病率升高引起国内外学者的关注,但其发病机制尚不清楚,且无统一的治疗方案.文章综述近年P...