34例儿童IgM肾病临床与病理分析

目的：探讨儿童IgM肾病临床及肾组织病理的特点。方法：收集整理2008年4月至2009年8月郑州大学第一附属医院儿内科34例IgM肾病患儿病例,对其临床表现及肾组织病理进行分析。结果：34例IgM肾病临床表现为肾病综合征的患儿22例（64.7％）;病理改变类型：微小病变12例（35.3％）,微小病变伴急性肾小管损伤3例（8.8％）,轻微病变6例（17.6％）,轻微病变伴缺血性损伤1例（2.9％）,系膜增生型7例（20.6％）,局灶节段硬化型4例(11.8％),局灶增生型1 例(2.9％);肾小球、肾血管及肾脏病理损害总积分随肾组织IgM沉积度增强而增加。结论：本组IgM肾病患儿大多临床表现为肾病综合征, 肾组织学病理类型多样,病理特点以系膜区IgM沉积为主,肾组织IgM沉积强度与肾脏病理损害程度有关。［中国当代儿科杂志,2010,12（5）：338-340］     

170例婴幼儿肾小球疾病病理类型分析

目的 探讨婴幼儿肾小球疾病的病理与临床特点。方法 对经肾活检确诊的170例婴幼儿肾小球疾病进行分析。结果 原发性肾小球肾炎164例（占96．5％），继发性肾小球明炎3例（1．85），遗传性肾病3例（1．8％）。原发性肾小球肾炎中系膜增生性肾炎67例（40．9％），其他依次为微小病变37例（22．6％），IgM肾病29例（17．7％），毛细血管内增生性肾炎9例（5．5％），膜性肾病8例（4．9％），轻微病变6例（3．7％），IgA肾病5例（3．0％），局灶节段性肾小球硬化2例（1．2％）和C1q肾病1例（0．6％）。继发性肾小球肾炎中紫癜性肾炎2例，药物性肾损害1例。遗传性肾病中先天性肾病综合征3例。原发性肾小球的临床类型肾病综合征150例（占88．2％），其中单纯性肾病133例，肾炎性肾病17例。结论 婴幼儿肾小球疾病中原发性肾小球肾炎占绝大多数。系膜增生性肾炎和微小病变为最常见的病理类型。原发性明小球肾炎的临床类型以肾病综合征为主，其中单纯性肾病占绝大多数。继发性肾小球肾炎主要为紫癜性肾炎。遗传性肾病以先天性肾病综合征多见，仅见于小于6月的婴幼儿和新生儿。婴幼儿肾脏疾病经皮肾活检对患者的临床诊断和指导治疗是有益的。     

227例小儿原发性肾小球疾病232次肾活检结果分析

本文报告4年来我院227例小儿原发性肾小球疾病(PGD)肾活检结果,其中5例作肾活检复查。临床表现为肾病综合征103例,单纯性血尿或/和蛋白尿97例,急性肾炎16例,迁延性肾炎3例,慢性肾炎3例,家族性肾炎5例。病理表现共10类,其中微小病变(MCN)13例(5.7%),系膜增生性肾炎(MsFGN)140例(61.7%),局灶性肾炎(FGN)12例(5.3%),膜性增生性肾炎(MPGN)9例(4%),膜性肾炎(MN)2例(0.9%),局灶节段性肾小球硬化(FSGS)9例(4%),新月体性肾炎(CrGN)1例(0.4%),硬化性肾小球肾炎(SCGN)3例(1.3%),弥漫毛细血管内增生性肾炎(EnPGN)15例(6.6%),IgA肾病(IgAGN)23例(10.1%)。5例肾活检复查结果:2例由MCN转为FSGS,1例MCN转为MsPGN,1例MsPGN转为兼有FSGS,1例由FGN转为MsPGN。     

儿童急性肾炎综合征的临床及病理诊断

目的　探讨儿童急性肾炎综合征临床诊断和病理诊断的相关性及早期肾活检的意义。方法　分析比较 43例急性肾炎综合征临床、病理诊断结果及部分病例的临床经过。结果　临床诊断链球菌感染后肾炎 4例 ,病理诊断 :毛细血管内增生性肾小球肾炎 3例 ,新月体肾炎 1例 ,其中 1例毛细血管内增生性肾小球肾炎 ,1年后复查肾活检为局灶节段性肾小球硬化 ,5年后进入终末期肾衰 ;临床诊断其它原发性肾小球肾炎 2 3例 ,病理诊断 :毛细血管内增生性肾小球肾炎 2例 ,新月体肾炎 1例 ,系膜增生性肾小球肾炎 9例 ,IgA肾病 8例 ,局灶节段性肾小球硬化 2例 ,狼疮肾炎 1例 ,临床诊断紫癜肾炎 13例 ,狼疮肾炎 3例 ,均与病理诊断相符合。结论　急性肾炎综合征 ,尽早肾病理检查对发现严重肾脏病变、认识急性链球菌感染后肾炎的转归和急性肾炎综合征的病因诊断 ,指导治疗 ,改善预后有重要价值     

儿童急性肾炎综合征的临床及病理诊断

目的0：探讨儿童急性肾炎综合征临床诊断和病理诊断的相关性及早期肾活检的意义。方法：分析比较43例急性肾炎综合征临床、病理诊断结果及部分病例的临床经过。结果：临床诊断链球菌感染后肾炎4例,病理诊断：毛细血管内增生性肾小球肾炎3例,新月体肾炎1例,其中1例毛细血管内增生性肾小球肾炎,1年后复查肾活检为局灶节段性肾小球硬化,5年后进入终末期肾衰;临床诊断其它原发性肾小球肾炎23例,病理诊断：毛细血管内增生性肾小球肾炎2例,新月体肾炎1例,系膜增生性肾小球肾炎9例,IgA肾病8例,局灶节段性肾小球硬化2例,狼疮肾炎1例,临床诊断紫癜肾炎13例,狼疮肾炎3例,均与病理诊断相符合。结论：急性肾炎综合征,尽早肾病理检查对发现严重肾脏病变、认识急性链球菌感染后肾炎的转归和急性肾炎综合征的病因诊断,指导治疗,改善预后有重要价值。     

问：频复发和激素依赖病例肾小球病理类型有无改变？

问：频复发和激素依赖病例肾小球病理类型有无改变？答：对此问题目前有两种认识。一是频复发病例肾小球病变本来就个一定是微小病变，而是系膜增生型（尤其是ＩｇＭ肾病）或局灶节段性肾小球硬化。如有人对１６例早期频复发患儿做肾活检，发现仅４例是微小病变型。二是原...     

单纯性血尿患儿肾组织免疫病理及肾小管血管内皮细胞生长因子的表达

目的 观察单纯性血尿患儿肾组织免疫病理变化及肾小管血管内皮细胞生长因子(VEGF)的表达,探讨VEGF在单纯性血尿发病机制中的作用.方法 选取2004年1月-2007年3月在苏州大学附属儿童医院就诊并行肾活检的32例单纯性血尿患儿,均于肾活检后,取其部分肾组织经快速冷冻切片行常规病理和免疫荧光分析.同时采用免疫组织化学方法 检测其肾小管VEGF表达情况,并进行统计学分析.结果 不同临床类型的单纯性血尿患儿肾组织病理主要表现为系膜增生性改变,其中轻度增生和弥散性增生分别占37.50%和46.88%,少数伴局灶/节段性细胞增生、坏死、纤维化和肾小球硬化.不同临床类型、病理类型的病例均可见IgA和(或)IgM沉积,其中系膜区以IgA沉积为主者占62.50%,且常伴IgM和(或)IgG沉积;以IgM沉积为主(不伴IgA)者占37.50%,其中仅有IgM者9例,占28.13%,少数伴IgG沉积.与对照组比较,各单纯性血尿组患儿肾小管VEGF表达均显著降低(P＜0.05).不同病理类型间弥散性系膜增生(Ⅲb级)患儿肾小管VEGF阳性表达率略低于轻微改变(Ⅰ级)患儿,二者比较无显著性差异(P＞0.05).伴局灶/节段性改变(Ⅱ级)的患儿肾小管VEGF阳性表达率均较Ⅰ级和Ⅲb级患儿降低,其中Ⅱb加Ⅲb与Ⅲb组间比较无显著性差异(P＞0.05),Ⅰ级与Ⅱb加Ⅲb和Ⅱa加Ⅲb级间,及Ⅲb级与Ⅱa加Ⅲb级间比较均存在显著性差异(Pa＜0.05);Ⅱa加Ⅲb级无症状血尿组肾小管VEGF表达率低于Ⅱb加Ⅲb组,二组间比较无显著性差异(P＞0.05).结论 单纯性血尿患儿肾组织主要表现为系膜增生性改变,伴IgA和(或)IgM沉积,提示免疫炎性反应在本病发病机制中起重要作用.各组病例肾小管VEGF表达降低,肾脏保护因素减弱,可能也是单纯性血尿发生、发展的重要影响因素.     

肾小球疾病患者尿中Ⅳ型胶原的测定及临床意义

目的探讨尿中Ⅳ型胶原在肾小球疾病诊断中的应用价值。方法应用竞争抑制性酶联免疫吸附实验，检测了２０例健康人及３８例肾小球疾病患者尿中Ⅳ型胶原的水平，并与肾活检的组织病理进行了比较。结果病理改变较为严重的患者以及肾活检为系膜增生性肾小球肾炎并伴有局灶性硬化、粘连和（或）新月体形成的患者，尿中Ⅳ型胶原的含量明显高于正常对照；但肾活检仅显示系膜增生性肾小球肾炎的患者，尿中Ⅳ型胶原水平同正常对照。结论尿中Ⅳ型胶原水平的测定，可以作为一个无创性的临床检测指标，有助于肾小球疾病的病理变化及其程度的诊断。     

原发性难治性肾小球疾病不同病理类型的疗效动态观察

报告7例原发性难治性肾小球疾病,临床治疗近期效果均不满意。肾活检结果系膜增生与局灶节段硬化性肾炎各2例,弥漫增生、膜性肾炎与新月体肾炎各1例,至今5年后随访结果痊愈3例,死亡2例,维持蛋白尿状态2例。表明肾组织病理变化严重,是难治与死亡率高的基础。另提示难治病例多合并高凝状态,治疗过程使用抗凝药不可忽视。     

肾病综合征伴局灶节段性肾小球硬化的激素和环磷酰胺冲击的疗效观察

局灶节段性肾小球硬化（ＦＳＧＳ）在小儿原发性肾病综合征（ＰＮＳ）中占一定比例，亦为难治性肾病（ＲＮＳ）的主要病理类型。我院在２５７例肾活检中证实ＦＳＧＳ者５０例，占１９４％。本组５０例初治时用激素常规方案施治，对其中激素抗药或依赖等３０例ＲＮＳ中的...     

儿童IgM肾病与IgA肾病临床和病理的对比研究

目的比较IgM肾病(IgMN)与IgA肾病(IgAN)患儿在临床及病理方面的异同。方法对经肾活检确诊的38例IgMN及40例IgAN患儿的临床表现、实验室检查及肾脏病理进行对比分析。结果 IgMN患儿的平均发病年龄小于IgAN患儿,平均肾活检前病程长于IgAN患儿,肉眼血尿发生率、尿IgG及尿白蛋白水平均低于IgAN患儿,同时严重肾小球损伤发生率也低于IgAN患儿,差异均有统计学意义(P0.05)。IgMN患儿中,有严重肾小球损伤患儿的血清白蛋白水平更低而尿白蛋白水平更高,与无严重肾小球损伤的同组患儿比较,差异有统计学意义(P0.05);有严重肾小管损伤患儿以男性多见,肉眼血尿发生率、尿白蛋白和N-乙酰-β-D氨基葡萄苷酶(NAG)水平以及出现基底膜厚薄异常的比例高于无严重肾小管损伤的患儿,差异均有统计学意义(P0.05),但发生严重肾小球损伤的差异无统计学意义(P0.05)。在IgAN患儿中,有严重肾小球损伤患儿的蛋白尿、肾小管见RBC管型、C3及Fibrinogen显著沉积和足突融合的发生率均高于无严重肾小球损伤的同组患儿,差异有统计学意义(P0.05);有严重肾小管损伤的患儿的肾功能受损程度、出现重度系膜细胞增生及肾小球纤维硬化情况比无严重肾小管损伤的同组患儿更严重,差异均有统计学意义(P0.05)。结论儿童IgMN与IgAN在临床和病理方面存在差异,IgMN肾脏损伤程度较IgAN轻。与IgAN不同,IgMN患儿的肾小管损伤与肾小球损伤无平行关系。     

肾小球系膜区微量IgM沉积在儿童微小病变型肾病综合征中的意义

目的 探讨肾小球系膜区微量IgM 沉积在儿童微小病变型原发性肾病综合征(PNS)中的意义。方法 以临床诊断为PNS、病理诊断为微小病变(MCD)及肾组织微量IgM 沉积的106 例患儿为研究组,无免疫复合物沉积的MCD 型PNS 患儿81 例为对照组,回顾性分析两组患儿的临床特点、微量IgM 沉积对糖皮质激素及免疫抑制剂疗效的影响。患儿均口服足量泼尼松治疗,对糖皮质激素耐药者或频复发者联用免疫抑制剂治疗。结果 研究组糖皮质激素耐药率高于对照组(27.2% vs 12.3%,PPP>0.05)。研究组和对照组频复发病例联用MMF 治疗后复发频率均显著减少(P结论 MCD 型PNS 患儿肾脏的微量IgM 沉积可能是糖皮质激素耐药及频复发的重要因素;糖皮质激素耐药及频复发患儿联用MMF 治疗可能是较好的治疗方案。     

Clinicl Significance of Mesangial IgM Deposits in Minimal Change Nephrotic Syndrome

The clinicopathologic features and the response to corticosteroid therapy were compared in 9 patients with minimal change nephrotic syndrome (MCNS) and diffuse mesangial IgM deposits (Group I) and in 32 of those without IgM deposits (Group II). However, serum IgM levels in Group I in both relapse and remission were significantly higher than those of Group II and controls (p<0.001). In Group I mesangial IgM deposits were diffuse in 9 (100%), mesangial C_1q was present in 4, IgA and fibrinogen were each observed in 1, respectively. Electron dense deposits in the mesangium were also present in 2 to 5 patients in Group I. No significant differences were found between the two groups in age of onset, sex ratio, laboratory data except for serum IgM level, duration before biopsy, follow-up periods, outcome, and response to steroid therapy. Our data suggest that a more severe degree of either impairment of mesangial clearance of IgM or overproduction of IgM may be involved in patients with MCNS and mesangial IgM deposits but that these patients could not be considered a distinct group of patients.     

Hepatitis B Surface Antigenaemia and Glomerulopathies in Children

ABSTRACT. Serum HBsAg positivity prevalence was studied on 98 children affected by various nephropathies or obstructive uropathies and on a control group of 71 children, consecutively admitted into the same clinical ward, suffering from other than hepatic or renal diseases. No significant difference was found between the group of children with non-glomeruiar nephropathies or obstructive uropathies and the control group. The prevalence of HBsAg positivity was significantly higher in male children with membranous glomerulopathy and in those with lipoid nephrosis than in the control group. All HBsAg positive children with glomerulopathies were chronic carriers of the hepatitis B virus. However, though left undemonstrated, there still lies a probable pathogenetic relationship between the hepatitis B virus infection and membranous glomerulopathy. The authors hypothesize that an impaired immune response in male children with lipoid nephrosis may account for both the hepatitis B virus infection and the development of the glomerular disease.     

Somatomedin in children with chronic renal insufficiency - relationship to growth rate and energy intake

The relationship of energy intake, growth rate and serum concentration of somatomedin-A was evaluated in eighteen children with chronic renal insufficiency. Serum concentrations of somatomedin-A were found to be 0.84 micro/ml in normal children and were elevated to 3.06 micro/ml in children with chronic renal insufficiency prior to dialysis (p less than 0.01). Somatomedin-A concentrations increased during chronic hemodialysis to 5.81 micro/ml and decreased to 1.59 micro/ml following successful renal transplantation (p less than 0.01). Serum concentrations of somatomedin-A correlated with residual glomerular filtration rates (r = -0.5), serum creatinine concentration (r = 0.59), and blood urea nitrogen (r = 0.6). Growth rates correlated with energy intake (r = 0.58) and somatomedin-A concentrations (r = 0.4) in the children with chronic renal insufficiency. Both energy intake and somatomedin-A increased significantly after one year of nutritional supplementation. Our findings are consistent with the hypothesis that somatomedin, like other polypeptide hormones, is elevated in uremia and that increased energy intake may affect the growth of children with chronic renal insufficiency by increasing somatomedin levels.     

儿童急性感染后IgA为主型肾小球肾炎1例报道并文献复习

目的 提高对儿童急性感染后IgA为主型肾小球肾炎（简称肾炎）临床和病理特征的认识。方法分析1例急性感染后IgA为主型肾炎患儿的临床和病理资料,并结合文献分析其临床特点、诊断、发病机制、治疗及预后。结果 本例患儿临床表现为肉眼血尿、大量蛋白尿、急性肾损伤,抗链球菌溶血素“0”(ASO)升高、血清IgA升高等,肾脏病理组织学见肾小球系膜细胞轻度增生性病变,免疫荧光见IgA（+） 、C3（+）沉积于肾小球系膜区和血管襻;电镜见肾小球上皮下“驼峰”样电子致密物沉积伴系膜区少量致密物沉积。在病程9周时患儿肾功能恢复正常,血尿和蛋白尿消失。结论急性感染后IgA为主型肾炎临床和病理特征均具有不典型性,诊断依赖早期肾活检,电镜检查必不可少,短期疗效尚可,远期疗效仍需观察。     

血清胱抑素C浓度测定对病毒性脑炎患儿肾功能损害的诊断价值

目的：探讨血清胱抑素C(Cyst C)浓度在评估病毒性脑炎患儿肾小球滤过功能中的价值。方法：92例病毒性脑炎患儿为病例组,50例健康儿童为对照组,又根据肾小球滤过率（GFR）将病例组分成肾功能正常组、代偿组、失代偿组和衰竭及终末期组。检测各组血清Cyst C、尿素氮(BUN)、肌酐(Cr)浓度。结果：① 与对照组比较,病例组BUN、Cr及Cyst C均显著增高(P0.05）,在其余各病例亚组间差异有统计学意义（P<0.01）。③Cyst C与BUN、Cr呈显著正相关,与GFR呈显著负相关。结论：病毒性脑炎患儿存在不同程度的肾功能损害;Cyst C是一种比BUN和Cr更好地反映肾小球滤过功能的指标,检测血清Cyst C对病毒性脑炎患儿肾脏功能的监测有重要的临床应用价值。     

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.     

儿童过敏性紫癜性肾炎与IgA肾病的临床和病理的对比研究

目的　对比研究过敏性紫癜性肾炎 (HSPN)和IgA肾病在临床、实验室检查及肾脏病理改变上的异同 ,探讨两者的关系。方法　对经肾活检证实的 31例IgA肾病及 12 0例过敏性紫癜性肾炎患儿进行详细的临床及病理对比分析。结果　 2 5 8%的IgA肾病发病年龄在 12岁以上 ,而HSPN中仅占 10 .0 % ,差异有显著意义 (P <0 0 5 )。二组患儿临床表现类型分布上相似 ,但HSPN肾外症状多 ,均有皮肤紫癜 ,5 9 2 %有胃肠症状 ,4 6 7%有关节痛 ,而IgA肾病仅 3 2 %有腹痛。在肾脏病理改变上 ,IgA肾病 35 5 %出现球性硬化、4 1 9%系膜硬化 ,HSPN分别为 3 1%及 6 3% ,但HSPN有 6 5 6 %出现内皮增生 ,IgA肾病仅 2 9 0 % ,差异均有非常显著意义 (P <0 0 1)。IgA肾病中 6 5 %合并有薄基底膜病变 ,HSPN未见薄基底膜病变。电镜下HSPN电子致密物稀疏、松散 ,沉积部位分布较为广泛 ,位于肾小球系膜、内皮下甚至基底膜内 ,而IgA肾病电子致密物成密集团块状 ,主要局限于系膜区及旁系膜区。HSPN患儿中 2 3例 (71 9% )肾小球免疫沉积物中含有IgG ,其中 6例以IgG为主 (4例 )或毛细血管壁有IgG线样沉积 (2例 ) ,而IgA肾病肾小球免疫沉积物中有IgG沉积的仅为 19 4 % ,其多为IgA伴IgM和 (或 )C3 沉积 ,未见以IgG沉积为主以及线样IgG沉积     

MICROANGIOPATHIC GLOMERULOPATHY IN CHILDREN WITH SICKLE CELL ANEMIA

We studied kidney biopsy specimens from three children with sickle cell anemia and microangiopathic glomerulopathy. One child also had cyanotic congenital heart disease. Laboratory evaluation revealed proteinuria and normal serum creatinine in all and normal serum complement in two of the three children at the time of biopsy. In all biopsies, glomeruli were enlarged with diffuse hypercellularity and focal segmental mesangial interposition; capillary loop lumens were congested with sickled erythrocytes. Immune labeling identified segmental immunoglobulin G, C3, and properdin over the glomerular capillary loop walls in each case. Ultrastructurally, the subendothelial zone of the glomerular basement membrane was widened with new lamina densa formation with focal mesangial interposition. The glomerular lesion we describe in these children may be due to endothelial injury related to the altered erythrocytes, glomerular hemodynamics, and the hypercoagulable state characteristic of sickle cell disease.