激素耐药型和激素依赖型肾病患儿肾脏病理计量分析

目的 探讨病理计量分析评价激素耐药型（SR）和激素依赖型（SD）肾病综合征患儿的肾脏病理损害及其临床应用意义。方法 采用自行研制的评分法,对73例SR型和SD型肾病患儿的肾脏病理从病理类型、肾小球病变、小管间质病变、肾脏总的病理损害4个方面计量分析,并以血尿素氮为应变量,临床表现和肾脏病理计分为自变量进行逐步回归分析。结果 （1）病理类型评分结果：微小病变、局灶节段肾小球硬化、膜性肾病、系膜毛细血管性肾小球肾炎和系膜增生性肾小球肾炎五组间的年龄、病程、复发次数、血尿素氮、胆固醇、白蛋白、尿蛋白定量、肾脏总的病理损害的差异均无显著意义,而五组患儿的肾小球病变计分分别为1,5,5,6,5,微小病变组与其他各组比较差异有显著意义（H＝19．278,P＜0．01）。（2）73例患儿肾小球病变计分结果：正常2例（3％）,轻度53例（73％）,中度17例（23％）,重度1例（1％）。且正常、轻度、中度三组间血尿素氮、小管间质病变的差异有显著意义（H＝8．40,P,0．01;H＝11．56,P＜0．05）。（3）肾小管间质病变计分结果：轻度23例（34％）,中度37例（18％）,且轻、中、重三组间病程、复发次数、血尿素氮、肾小球病变计分的差异均有显著意义。（H值分别为25．016,38．775,14．944,10．625,P值均＜0．01）。（4）肾脏损害的总分结果：轻度33例（45％）,中度34例（7％）,重度6例（8％）,轻、中、重三组间病程、复发次数、血尿素氮的差异有显著意义（H值分别为19．42,14．335,18．923,P值均＜0．01）。（5）以血尿素氮为应变量行逐步回归分析,小管间质及肾小球损害对血尿素氮均有显著影响,小管间质病变的回归系数为0．862（P＜0．01）;肾小球病变的回归系数为0．212（P＜0．05）。结论 采用评分法对肾脏病理进行主分评价其病变程度,具有临床实用性,尤其对小管间质病变的计分评价对判断肾功能受损更有价值。     

儿童原发性肾病综合征临床病理及预后的关系

目的 探讨儿童原发性肾病综合征（NS）病理学改变的临床意义和对预后的影响。方法 总结1995－1999年我科收治的32例肾穿儿童原发性NS的临床及病理特征。并对肾小管间质病变进行病理分级。结果 32例NS肾穿病理类型仍以系膜增生性肾小球肾炎（MSPGN）为主占65％（21／32），不同肾小球病理组织类型之间肾小管间质改变无特异性。肾小管间质病变加擀肾小管功能损害亦加重。结论 本文NS病理类型以MSPGN为主。多数肾小管间质的改变随着病理类型的严重程度而加重，部分有小球－小管间质改变不平行现象。肾小管间质改变较重者其预后较差。     

肾病综合征患儿肾组织原位血管紧张素转换酶mRNA表达与激素疗效关系的研究

目的　探讨肾病综合征 (NS)患儿肾脏局部肾素 血管紧张素系统 (RAS)、激素耐药、肾脏病理损害程度之间的关系 ,阐述激素耐药的部分机制。方法　 85例原发性NS患儿按激素敏感型NS(SSNS)、激素依赖型NS(SDNS)、激素耐药型NS(SRNS)分成 3组 ,选 6例行肾切除的肾肿瘤患儿的正常肾组织作为对照组。采用原位杂交的方法检测 4组患儿肾脏原位血管紧张素转换酶 (ACE)mRNA的表达水平 ;评分法半定量评估肾脏的病理损害程度。分析NS患儿肾组织ACEmRNA表达水平、激素反应性、肾脏的病理损害程度间的关系。结果　①在肾小球和小管间质区域的ACEmRNA表达水平均为SRNS组 >SSNS组 >对照组 (P <0 0 1)。②SRNS、SDNS、SSNS肾小球病理损害的评分分别为 :6 6 7± 2 4 3,4 6 8± 2 30 ,4 4 2± 2 87(P <0 0 1) ;小管间质病理损害评分分别为 :10 4 8± 3 77,7 2 0± 2 79,4 2 5± 1 4 8(P <0 0 1)。③肾组织ACEmRNA的表达与肾小球和小管间质病理损害程度呈正相关 ,相关系数分别为 0 4 82 ,0 85。结论　SRNS型NS患儿肾组织ACEmRNA表达增强 ,并且与肾脏的病理损害程度密切相关     

难治性肾病30例临床与病理的关系

目的 探讨难治性肾病的临床与病理特征之间的关系。方法 对30例小儿难治性肾病的临床特点、病理特征及治疗结果进行分析。结果 小儿难治性肾病临床表现大多为蛋白尿 血尿占70％。最常见病理类型为系膜增殖性肾小球肾炎，占43.3％；其次为局灶节段性肾小球硬化，占20％；再次为膜增殖性肾小球肾炎占13．3％，而IgA肾病、微小病变等病理类型少见。临床表现为单纯蛋白尿组病理类型以系膜增殖性肾炎为主，临床表现多为对激素依赖、频复发；蛋白尿 血尿组病理特征以局灶节段性肾小球硬化、膜增殖性肾炎最多，对激素治疗表现的反应为激素耐药。结论 临床表现为蛋白尿 血尿的小儿肾病大部分为难治性肾病，病理改变多样，以系膜增殖性肾小球肾炎最多见。临床及病理分型、对激素治疗反应三者关系密切。     

小儿难治性肾病病理特征的研究

目的　探讨小儿难治性肾病病理特征。方法　对小儿难治性肾病１４０ 例的临床及病理特征进行了分析。结果　小儿难治性肾病最常见的病理类型为系膜增殖性肾小球肾炎（ Ｍｅｓ ＰＧＮ）占３９－３％ ,其次为局灶节段性肾小球硬化（ＦＳＧＳ） 占２９－３％ ,再次为轻微病变和微小病变（ＭＣＤ） 占１４－３ ％ ;膜增殖性肾小球肾病、膜性肾病、硬化性肾炎、ＩｇＭ 肾病、ＩｇＡ肾病等病理类型少见。１４０ 例中单纯性肾病者以ＭｅｓＰＧＮ、ＦＳＧＳ、ＭＣＤ 为主,肾炎性肾病可见于各种类型;激素依赖患儿以轻、中度ＭｅｓＰＧＮ、ＦＳＧＳ及ＭＣＤ 为主;频繁复发者以ＭＣＤ 及轻、中度ＭｅｓＰＧＮ 为主;而激素耐药患儿可见于各种病理类型。结论　小儿难治性肾病病理类型多样,以ＭｅｓＰＧＮ、ＦＳＧＳ、ＭＣＤ 多见。病理类型与临床分型、对激素治疗反应等有关。     

TGF-β1、VEGF在儿童原发性肾病综合征中的表达及其临床意义

目的研究转化生长因子β1（TGF—β1）和血管内皮生长因子（VEGF）与儿童原发性肾病综合征（PNS）蛋白尿的关系及其在不同病理类型肾组织中的表达差异,探讨PNS蛋白尿的可能发生机制及导致肾纤维化的影响因素。方法应用ELISA法测定正常对照组与NS组患儿血清TGF—β1、VEGF水平,比较两指标与蛋白尿、肾病不同病理类型间的关系及两者间的相关性;应用免疫组化法检测不同病理类型PNS与正常肾组织中TGF—β1、VEGF的蛋白表达,并比较其差异。结果①蛋白尿阳性NS组,其血清TGF—β1、VEGF水平均分别较对照组和蛋白尿阴性组显著增高（P〈0．05）,两指标间呈正相关（r=0．4305）。②在不同病理类型中,局灶节段硬化性肾炎（FSGS）组血清TGF—β1水平显著高于微小病变型肾病（MCNS）组、系膜增生型肾小球肾炎（MSPGN）组及对照组（P〈0．01）。③FSGS及MSPGN组血清VEGF水平较MCNS组与对照组显著增高（P〈0．05）。④TGF-β1、VEGF在病变肾组织中阳性表达面积均显著高于对照组（P〈0．05）。⑤TGF—β1的蛋白表达强度在MCNS、MSPGN、膜性肾病（MN）、FSGS4组中呈逐渐增强趋势,其中FSGS组TGF—β1表达较无纤维化组明显增强（P〈0．05）;而VEGF蛋白表达强度在不同病理类型之间以及有无间质纤维化之间差异无显著性（P〉0．05）。结论①血清TGF—β1、VEGF水平与PNS蛋白尿的产生有关,且与肾脏病理损害类型有关;②NS患儿肾组织均存在TGF-β1、VEGF蛋白表达增强,尤以TGF—β1表达增强为著,且表达强度可能与小管间质纤维化程度有关。     

儿童C1q肾病10例临床与病理分析

目的探讨C1q肾病的临床与病理改变的关系。方法对10例经肾活检确诊为C1q肾病患儿临床表现、肾小球、肾小管及免疫病理特征进行分析比较,6例肾病综合征中环磷酰胺冲击治疗3例,环胞素、霉酚酸酯和甲泼尼龙冲击治疗各1例。结果临床表现为单纯性血尿2例,肾炎综合征、急性肾炎各1例,肾病综合征6例;病理类型为轻微病变、系膜增生性肾小球肾炎各2例,局灶节段性肾小球硬化5例,新月体肾炎1例;肾小管间质1例无改变,Ⅰ级和Ⅱ级各3例,Ⅲ级2例,Ⅳ级1例;免疫荧光:系膜区均有娃著的以C1q为主的沉积。10例患儿平均随访25.7个月;6例肾病综合征均对激素抵抗,加用免疫抑制剂治疗,5例缓解,1例无效,肾功能渐减退。结论C1q肾病临床病理改变多样化,临床以肾病综合征为主,病理以局灶节段性肾小球硬化为主,对激素多不敏感,预后与间质损害程度相关,与C1q沉积无相关性。     

肾病综合征患儿肾脏ACE m RNA表达与病理损害相关性的研究

目的:探讨肾病综合征(NS)患儿肾脏局部肾素 血管紧张素系统(RAS)表达与肾脏病理损害的关系,阐明儿童NS肾脏损害慢性病理进展的部分机制。方法：采用原位杂交的方法检测85例原发性NS患儿肾脏原位血管紧张素转换酶(ACE)mRNA的表达水平,评分法半定量评估肾脏的病理损害。结果：①对照组、NS患儿组肾小球内ACE mRNA表达阳性细胞百分率分别为：(3.97±1.43) %和(22.61±12.30) %,(P<0.01);小管间质区域分别为：(19.15±5.96) %和(58.42±17.61) %,(P<0.01)。②NS患儿5种病理类型间(MCD,FSGS,MN,MPGN,MsPGN),肾小管间质区域的ACE mRNA表达水平各组间差异无显著性意义(P>0.05),肾小球内仅MCD组与其它各组间差异有显著性意义(P<0.01)。③按照肾脏病理损害的程度分成轻、中、重度3组,其ACE mRNA表达阳性细胞百分率分别为：(30.50±6.52) %,(45.20±11.06) %和(54.77±11.86) %,(P<0.01)。结论：在儿童NS肾脏的慢性病理进展中,肾脏局部RAS紊乱可能是重要原因之一。     

37例儿童IgA肾病临床与病理分析

为了探讨IgA肾病的临床与病理改变的关系,对37例IgA肾病进行临床分型并与肾小球、肾小管间质改变及免疫病理特点的关系进行比较。结果：临床分型中单纯血尿（血尿）18例占49%,肾百闻不如一见 综合征（肾病）14例占38%,血尿和蛋白尿3例占8%,肾炎综合征（肾炎）2例占5％,肾小球病理损害以Ⅲ级为主占厮４％,临床各型与肾小球病理损害无相关性。肾小管间质改变２４例,血尿组７例占３９％,其中Ｉ级为４３％,Ⅱ级为５７％,肾病组均有改变,其中Ⅱ级１１例占７８％,Ⅲ级３例占２２％,血尿和蛋白尿组2例占66%,肾炎组1例占50%,免疫病理改变为IgA16例,IgAG6例,IgAM10例,IgAGM5例,血尿组以单纯IgA沉积为主占66%,肾病组则以IgAM型为主占50%,提示IgA肾病临床以单纯血尿为主,其次为肾病综合征;肾小球病理损害程度与临床分型无相关性,但肾病组肾小管间质均有改变且程度也较血尿组为重。免疫病理血尿组以单纯IgA为主,而肾病组以IaAM为主。     

基于病例对照研究的儿童肾病综合征低白蛋白血症诊断标准探讨

目的：探讨儿童原发性肾病综合征低白蛋白血症诊断标准。方法：收集1993年1月至2012年12月在北京大学第一医院住院的原发性肾病综合征患儿的临床资料,根据初次诊断肾病综合征时的最低血浆白蛋白水平将患儿分为＜25 g·L^-1 组和25~30 g·L^-1 组。比较两组的肾脏病理类型、临床表型、激素效应、合并症和预后。结果：进入本文分析患儿458例,白蛋白25~30 g·L^-1 组28例,白蛋白<25 g·L^-1 组430例,两组性别和年龄差异无统计学意义。①血浆白蛋白水平<25 g·L^-1 组和25~30 g·L^-1 组分别有12例和141例行肾脏病理分析,两组肾脏病理类型分布差异无统计学意义,均以局灶节段性肾小球硬化症为主,两组微小病变或轻微病变分别占21.9%和8.3%;②2组临床表型、免疫抑制剂的应用种类、严重合并症的发生情况和激素相关不良反应的发生情况差异均无统计学意义, 2组激素效应分布差异有统计学意义,血浆白蛋白水平<25 g·L^-1 组和25~30 g·L^-1 组激素敏感比例分别为68.4%（294/430）和50.0%（14/28）;③血浆白蛋白水平25~30 g·L^-1 组严重预后发生率（14.3%,4/28） 高于<25 g·L^-1 组（4.4%,19/430）。结论：具有大量蛋白尿伴有血浆白蛋白25~30 g·L^-1 的患儿肾脏病理类型以非微小病变为主,其预后较血浆白蛋白<25 g·L^-1 者更为严重,建议早期行肾活检。     

Childhood idiopathic nephrotic syndrome in Turkey

BACKGROUND: It has been reported that there are racial and regional differences in peak incidence age, histopathological features and response to steroid therapy in childhood idiopathic nephrotic syndrome. METHODS: One hundred and thirty-eight patients with a diagnosis of idiopathic nephrotic syndrome, followed up in 1994-2000, were assessed retrospectively. The aim of this study was to assess the patients' response pattern to steroid therapy, to determine whether the duration of the initial steroid therapy alters the steroid response pattern of the disease and to assess renal biopsy results. RESULTS: One hundred and fourteen patients who initially received only steroid therapy and were followed up regularly were classified according to response pattern. Of the 114 patients, 30 children had an initial response, 25 children had infrequent relapse, 19 had frequent relapse, 25 had steroid dependence and 15 children had steroid resistance. The 99 patients with steroid responsive nephrotic syndrome were divided into two groups with respect to duration of the initial steroid therapy. There was no statistically significant difference between standard and short therapy groups with respect to the steroid response patterns. Percutaneous renal biopsy was performed in 43 of the 138 patients. Mesengioproliferative glomerulonephritis was the most common histopathological lesion, followed by membranoproliferative glomerulonephritis. The proportions of membranous glomerulonephritis, focal segmental glomerulosclerosis and minimal change nephrotic syndrome were low in our group. CONCLUSIONS: Our study group is similar to one reported from Saudi Arabia with respect to the steroid response pattern and to Saudi Arabian and Nigerian reports with respect to the histopathology. Although it has been reported that short initial steroid therapy was followed by a higher rate of relapses, there was no statistically significant difference between standard and short therapy groups with respect to the relapse rate in our study group.     

儿童肾病综合征病理类型与年龄、性别的关系

目的 探讨肾病综合征患儿的肾脏病理和性别、年龄分期的关系.方法 对1 116例经肾穿刺活检明确肾脏病理的原发性肾病综合征患儿临床资料进行回顾性分析.结果 1 116例患儿中男817例,女299例,男女比例2.73:1;平均年龄(7.3 ± 3.3)岁,其中婴幼儿期90例,学龄前期294例,学龄期409例,青春期323例.肾脏病理轻微病变(MCNS)222例,占19.9%;系膜增生性肾小球肾炎(MsPGN)726例,占65.1%;膜增生性肾小球肾炎(MPGN)55例,占4.9%;膜性肾小球肾炎(MN)27例,占2.4%;局灶节段硬化性肾小球肾炎(FSGS)86例,占7.7%.肾脏病理类型在4个年龄分期的分布差异有统计学意义,MCNS患儿以学龄前期最多,MsPGN患儿以学龄期最多,MPGN和MN患儿以青春期最多,FSGS的患儿以学龄期最多.MCNS患儿的男女比例为5.3:1,MsPGN为2.4:1,MPGN为0.96:1,MN为3.5:1,FSGS为2.7:1,肾脏病理类型在性别上的分布差异有统计学意义(P ＜ 0.05).结论 对于无法开展肾活检的医院或有肾穿刺禁忌证的患儿,可根据年龄和性别结合临床检验初步推断肾脏病理变化的轻重,进一步指导治疗、判断预后.     

Idiopathic nephrotic syndrome in New Zealand children, demographic, clinical features, initial management and outcome after twelve-month follow-up: results of a three-year national surveillance study

Aim: To describe the demographic, clinical features, steroid response, histopathology and complications of all children diagnosed with idiopathic nephrotic syndrome (INS) in New Zealand over a 3-year period. Methods: A questionnaire seeking relevant clinical information was sent to all paediatricians who reported a new case of nephrotic syndrome to the New Zealand Paediatric Surveillance Unit. A follow-up questionnaire was sent to reporting paediatricians after the first 12 months of follow-up. Results: The incidence was 1.9 children per 100,000 under age 15 years. There was no significant difference in INS between ethnic groups. Approximately 80.4% were steroid responsive with median time to response of 8.4 days and mean time to relapse was 15.1 +/- 12.1 weeks (10.1-19.8 95% confidence interval). Follow-up at 12 months after diagnosis showed that two-thirds were either steroid dependent or frequent relapsers. Steroid resistance patients had a more variable course with some developing chronic renal failure and other remaining persistently nephrotic. Conclusion: The incidence and outcome of children with INS are similar to overseas studies. A large variety of steroid treatment regimens were noted. Current evidenced-based guidelines to treat INS were used infrequently.     

Cyclophosphamide treatment of steroid dependent nephrotic syndrome: comparison of eight week with 12 week course. Report of Arbeitsgemeinschaft für P?diatrische Nephrologie.

In a prospective study (Cytotoxic Drug Study II), 18 children with steroid dependent nephrotic syndrome and steroid toxicity were treated with cyclophosphamide (2 mg/kg body weight/day) for 12 weeks in combination with reducing doses of prednisone (group A). This group was compared retrospectively with 18 children with steroid dependent nephrotic syndrome, studied as part of the Cytotoxic Drug Study I, and who had received cyclophosphamide for eight weeks (group B). There were no differences between the groups in age at the onset of the nephrotic syndrome, age at entry into the study, and duration of the nephrotic syndrome before entry into the study. The number of relapses during the six months before the treatment was the same in both groups. Two years after treatment 12 of 18 children treated with cyclophosphamide for 12 weeks were still in remission. By contrast, only four of of 18 children treated with cyclophosphamide for eight weeks were still in remission. The cumulative rates of sustained remissions were significantly higher (67% and 22%, respectively) in group A. All relapses were observed within 400 days of stopping cytotoxic treatment. No severe side effects of cyclophosphamide occurred up to two years after treatment had been stopped. We conclude that for children with steroid dependent nephrotic syndrome and steroid toxicity cyclophosphamide treatment should be prolonged to 12 weeks to increase the likelihood of a prolonged remission.     

Hepatitis B-associated nephrotic syndrome in Jamaican children

Between December 1984 and November 1996, 171 children under 12 years old presented to the University Hospital of the West Indies with nephrotic syndrome. Hepatitis B surface antigen (HBsAg) was found in ten (6%) of these children, eight of whom had membranous nephropathy (MN), and one each had mesangial proliferative glomerulonephritis (MesN) and minimal change nephrotic syndrome (MCNS). Only those children with MesN and MCNS were steroid-sensitive. The HBsAg-positive status was identified incidentally on screening. At a mean follow-up of 34 months, seven of ten children had experienced complete or partial remission and three had persistent nephrotic syndrome, although none was in renal failure. Six of the ten had biochemical hepatitis. All the children were still HBsAg-positive. Hepatitis B virus (HBV) is a factor contributory to nephrotic syndrome in Jamaican children. As diagnostic clinical markers for HBV-associated nephropathy are usually absent, all children presenting with nephrotic syndrome should be screened for HBsAg. A policy should be implemented in Jamaica for screening pregnant women and at-risk groups for HBsAg, as well as for immunising susceptible neonates, in order to reduce the incidence of HBV-associated pathology.     

儿童狼疮相关性足细胞损伤5例分析

目的提高对小儿狼疮肾炎(lupus nephritis,LN)中足细胞损伤的认识。方法回顾性分析5例临床表现为肾病综合征而病理改变轻微的小儿LN临床及病理特点,与同期非肾病表现、病理改变轻微的7例小儿LN进行比较,并检索国内外文献进行复习、总结。结果临床表现为肾病的LN患儿与非肾病患儿相比,临床表现除尿蛋白量与血白蛋白水平有统计学差异外,性别、年龄、血尿、高血压、肾功能不全、补体及自身抗体水平均无统计学差异;两组病理改变比较显示,肾病组电镜下表现为弥漫足突融合,而非肾病组则未见此表现;光镜、免疫荧光镜检查两组均为正常或轻度系膜增生及少量免疫复合物沉积。结论与狼疮性肾炎典型的病理类型相比,狼疮相关性足细胞病可能是狼疮肾炎中的一种独立的病理类型,在临床工作中要加以重视。     

激素、钙调磷酸酶抑制剂和吗替麦考酚酯三联用药治疗激素耐药型肾病综合征患儿的疗效和安全性

背景少数病初即表现为激素耐药型的儿童原发性肾病综合征,临床治疗较困难,对于无明确遗传因素证据患儿,临床多采用激素联合一种或多种免疫抑制剂进行治疗,但目前尚无统一的药物添加原则或规范的治疗方案。目的观察激素联合钙调磷酸酶抑制剂、吗替麦考酚酯对初治激素耐药型肾病综合征患儿的治疗效果和安全性。设计回顾性非随机对照研究。方法纳入2014年1月至2020年12月北京大学第一医院儿科收治的初治激素耐药型肾病综合征患儿,除外遗传因素后,分为A组(激素+钙调磷酸酶抑制剂+吗替麦考酚酯三联治疗,三种药按先后顺序依次添加)、B组(激素+钙调磷酸酶抑制剂+吗替麦考酚酯三联治疗,激素联合钙调磷酸酶抑制剂治疗3个月以上无效,改为激素联合吗替麦考酚酯治疗3个月以上仍无效,最后三者联用)、C组(钙调磷酸酶抑制剂+吗替麦考酚酯治疗,因类固醇性糖尿病或青光眼停用激素)和D组(激素+钙调磷酸酶抑制剂+美罗华),比较各组的治疗效果。主要观察指标尿蛋白转阴时间、尿蛋白阴性时间百分比、平均复发次数。结果39例患儿纳入分析,A、B、C、D组分别为16、8、3、12例。A、B、C、D组尿蛋白转阴率分别为75.0%(12/16)、75.0%(6/8)、100%(3/3)和75.0%(9/12),组间比较差异无统计学意义(P>0.05)。A、D组平均尿蛋白转阴时间低于B、C组,尿蛋白阴性时间百分比高于B、C组,差异均有统计学意义(P<0.05)。A和D组间平均尿蛋白转阴时间和尿蛋白阴性时间百分比差异均无统计学意义(P>0.05)。各组患儿用药期间未报告药物相关不良反应。结论对于少数原发性激素耐药型肾病综合征患儿,激素联合钙调磷酸酶抑制剂、吗替麦考酚酯三联治疗有较好的效果。     

儿童初发IgA肾病肾病综合征型的临床特点

目的 研究儿童初发IgA肾病肾病综合征型的临床特点,探讨其与原发性肾病综合征的异同,为这两种疾病的临床鉴别诊断提供理论依据。方法 选择50例初发IgA肾病且表现为肾病综合征的患儿为观察对象,以72例初发原发性肾病综合征患儿为对照组,比较两组患儿的临床及实验室检查特点。结果 IgA肾病组患儿肉眼血尿、镜下血尿、高血压、急性肾损伤、贫血、低高密度脂蛋白胆固醇血症、低补体C4血症、激素耐药、肾炎型肾病综合征发生率均显著高于对照组,血IgE升高发生率低于对照组(P< 0.05)。两组血肌酐、血尿酸、血总胆固醇、高密度脂蛋白胆固醇、血IgE、血C4、血红蛋白水平比较差异有统计学意义(P< 0.05)。血IgE< 131.2 IU/mL、高密度脂蛋白胆固醇< 1.35 mmol/L作为临界值对鉴别IgA肾病肾病综合征型及原发性肾病综合征显示了一定的价值(Youden 指数分别为0.535、0.564)。结论 IgA肾病肾病综合征型患儿临床多表现为肾炎型肾病综合征及激素耐药型肾病综合征;临床表现联合高密度脂蛋白胆固醇、血IgE水平有助于鉴别IgA肾病肾病综合征型及原发性肾病综合征 。     

Eight and 12 week courses of cyclophosphamide in nephrotic syndrome.

Seventy-three children with steroid dependent minimal change nephrotic syndrome were randomly allocated to receive treatment with cyclophosphamide (2 mg/kg/day) for either eight or 12 weeks, in combination with prednisolone. All patients had previously relapsed while the dosage was being reduced or within 14 days of discontinuing prednisolone in the six months before receiving cyclophosphamide treatment (steroid dependent), and had severe steroid toxicity. Thirty two patients were treated with cyclophosphamide for eight weeks, and 41 for 12 weeks. There were no differences between the two groups in age at onset of nephrosis or entry into the study, sex ratio, duration of nephrosis, number of relapses before entry, and follow up period after entry. The relapse free rate of patients treated for eight weeks (25%) was similar to that of those treated for 12 weeks (24%) five years after stopping the treatment, and the mean relapse free interval and the sparing effect of cyclophosphamide (if any) on subsequent treatment with steroids did not differ between the groups. We conclude that cyclophosphamide should be used for no longer than eight weeks at a dose of 2 mg/kg/day in children with steroid dependent minimal change nephrotic syndrome.