儿童隐匿性肾炎的临床和病理研究

目的：探讨儿童隐匿性肾炎的临床和肾组织病理改变特点及其关系。方法：回顾性分析肾活检的323例隐匿性肾炎患儿的临床和肾组织病理改变情况。结果：323例隐匿性肾炎患儿中，单纯性血尿229例，单纯性蛋白尿19例，血尿伴蛋白尿75例。肾组织病理改变类型包括：轻微病变103例（31，89％）、基本正常74例（22．91％）、IgA肾病（IgAN）73例（22．60％）、薄基底膜病（TBMN）27例（8．36％）、系膜增生性肾炎（MsPGN）18例（5．57％）、局灶增生性肾炎（FPGN）10例（3．10％）、膜性肾病（MN）8例（2，48％）、局灶节段肾小球硬化（舢）8例（2．48％）、微小病变（MCD）1例（0，31％）、IgM肾病（IgMN）1例（0．31％）。单纯性血尿组中肾组织结构基本正常的比例较血尿伴蛋白尿组明显偏高（P〈0，01）；血尿伴蛋白尿组中IgAN的比例高于单纯性血尿组和单纯性蛋白尿组（分别P〈0．01、P〈0．05）。IgAN的Lee分级：单纯性血尿组中Ⅰ、Ⅱ级85．00％，Ⅲ级及以上15．00％；血尿伴蛋白尿组中Ⅰ、Ⅱ级58．10％，Ⅲ级及以上41．90％，明显高于单纯性血尿组（x^2=6．47，P〈0．05）。结论：儿童隐匿性肾炎的病理以轻微病变、基本正常、IgAN为常见表现，血尿伴蛋白尿患儿病变较单纯性血尿患儿为重。     

儿童孤立性血尿207例病理分析

目的：探讨小儿孤立性血尿的病理类型.方法：对207例符合孤立性血尿诊断标准的患儿行肾活检术,肾组织进行光镜、电镜及免疫荧光检查.结果：轻微病变72例（34.8%）;正常肾小球61例（29.5%）,其中局灶节段性肾小球透明样变性5例.IgA肾病36例（17.4%）;薄基底膜肾病21例（10.1%）,其中正常肾小球7例,伴轻微病变14例;系膜增生性肾小球肾炎13例（6.3%）,其中伴薄基底膜肾病2例;局灶增生性肾炎4例（1.9%）.结论：轻微病变与正常肾小球占第一位,IgA肾病是小儿表现为肉眼血尿的孤立性血尿的主要原因,但表现为孤立性血尿的IgA肾病病理变化相对较轻,为轻微病变或轻度系膜增生.所有病例均无新月体形成、肾小球硬化和小管间质受累.提示孤立性血尿患儿预后良好.     

呈局灶节段性肾小球硬化的IgA肾病临床病理特点

目的探讨呈局灶节段性肾小球硬化（FSGS）的IgA肾病（IgAN）的临床和病理特点。方法选取我院1988年1月至2002年2月经肾活检确诊为IgAN的患者587例，其中呈FSGS85例，呈弥漫性系膜增生性肾小球肾炎（MsPGN）162例，呈弥漫性系膜增生性肾小球肾炎伴局灶节段性肾小球硬化（MsPGN伴FSGS）185例，比较3种类型IgAN临床和病理资料。结果FSGS型IgAN占同期所有IgAN的14．5％，临床类型以大量蛋白尿型为主，占37．64％。肾小球球囊黏连发生率高达74．12％，小管间质纤维化发生率97．65％，病理分级以LeeⅣ～Ⅴ级为主，免疫病理以IgA—MG型为主，与MsPGN伴FSGS型和MsPGN型的IgAN相比，FSGS型IgAN病程较长，高血压、肾功能不全发生率较高（P〈0．05），而血尿的发生率与后两者无明显区别。结论呈FSGS型IgAN大量蛋白尿、高血压、肾功能不全的发生率高，病变较重，预后较差。     

肾炎患者血清鳞状细胞癌抗原异常升高的临床意义

目的：探索血清鳞状细胞癌抗原（ squamous cell carcinoma antigen,SCC）在肾炎患者中异常升高的临床意义。方法：检测100例住院肾炎患者和100例健康体检者血清SCC、血白蛋白、24 h尿蛋白,并对所有肾炎患者进行肾活检。结果：与正常对照组相比,肾炎组SCC数值和阳性率均明显升高;在肾炎组中,尿蛋白大于1.5 g组的SCC数值高于尿蛋白少于1.5 g组;白蛋白＜35 g/L组的SCC数值高于白蛋白〈35 g/L组。以上差异均具有统计学意义（P＜0.05）。不同病理类型肾炎组SCC数值比较显示,轻微肾小球病变组明显高于毛细血管内增生性肾炎组、膜增生性肾炎组、新月体肾炎组、硬化性肾炎组（P均＜0.05）。结论：肾炎患者血清SCC异常升高具有临床意义,且合并大量蛋白尿（＆gt;1.5 g）或低白蛋白（＜35 g/L）者升高更明显。病理类型为轻微肾小球病变的患者往往更容易出现SCC数值的异常升高。     

CKD1～2期250例IgA肾病患者临床与病理活动病变的相关性分析

目的：探讨原发性IgA肾病（IgAN）患者临床表现与病理活动病变的关系。方法：将250例经肾活检确诊,慢性肾脏病（CKD）1~2期的IgAN患者,按其临床表现分成3组：表现为镜下血尿或（和）蛋白尿为A组,102例;表现为肉眼血尿为B组,85例;表现为肾病综合征为C组,63例。对比各组临床和病理指标,并进行临床与病理活动病变的相关性分析。结果：（1）各组间资料比较：C组的血IgM、C3、C4水平显著高于A组与B组（P〈0.01）,C3沉积强度显著小于A组与B组（P〈0.01）;（2）临床与病理相关性分析：尿红细胞计数、尿NAG酶与新月体数目、肾小球系膜细胞增生程度呈显著正相关（P〈0.01）,尿NAG酶与肾间质炎细胞浸润程度呈显著正相关（P〈0.01）,尿NAG酶与纤维素样坏死的产生呈正等级相关（P〈0.05）。结论：IgAN的某些临床指标与病理中的活动性病变具有一定的相关性,尿红细胞计数和尿NAG酶是反映病理活动性病变指标,这些指标与新月体数目、肾小球系膜细胞增生、肾间质炎细胞浸润程度呈显著相关性。     

IgA肾病520例临床病理分析

目的研究IgA肾病(IgAN)的临床和病理特点及其相互关系。方法对1992年11月～2003年6月温州医学院附属第一医院肾内科病理室肾活检诊断的原发性IgAN520例进行临床与病理分型关系的分析。结果520例IgAN临床表现以无症状性尿检异常最常见,占346例(66.5%),其次是慢性肾炎和肾病综合征,分别占77例(14.8%)和66例(12.7%)。病理类型以局灶节段硬化性肾小球肾炎最常见,占186例(35.8%),其次是系膜增生性肾小球肾炎、轻微病变肾小球肾炎和局灶节段增生性肾小球肾炎,分别为116例(22.3%)、104例(20%)和63例(12.1%)。结论IgAN的临床病理表现多样化并具有一定特点。临床表现最常见为无症状性尿检异常,在病理上最常见的是局灶性肾小球病变类型。     

肾小球肾炎足细胞脱落与肾组织钠氢交换调节因子2表达的研究

目的探讨肾小球足细胞脱落情况及其与病理改变的关系;探讨6类肾炎中肾组织钠氢交换调节因子2（NHERF2）基因表达量的变化及其与足细胞损伤的关系。方法将患者分为肾炎组（原发性肾炎和狼疮性肾炎）、非肾炎组、健康对照组;用间接免疫荧光法检测尿足细胞数量,用荧光定量PCR检测肾组织NHERF2蛋白,比较不同类型肾炎足细胞脱落情况及NHERF2基因表达量。结果原发性肾炎和狼疮性肾炎（LN）组患者尿足细胞脱落较健康对照组显著增多（P〈0．05）;活动期LN患者尿足细胞脱落较缓解期LN显著增多（P〈0．05）;原发性肾炎组中局灶节段硬化性肾小球肾炎（FSGS）患者尿足细胞脱落最多,其次为膜性肾病（MS）患者,二者与健康对照组相比,差异均有统计学意义（P〈0．05）。MS患者尿足细胞脱落比微小病变性肾病（MCD）患者显著增多（P〈0．05）。原发性肾炎组和LN组患者肾组织NHERF2基因表达量均较非肾炎组显著降低（P〈0．05）。肾炎患者尿足细胞脱落数量和肾组织NHERF2基因表达之间具有相关性（r=0．318,P〈0．05）。结论根据尿足细胞脱落数量可初步推断FSGS和LN的病理改变类型、预测LN的疾病进展和预后,可能为临床MS与MCD的鉴别提供依据;证明肾小球足细胞脱落可能与肾组织NHERF2基因表达减低有关,提示足细胞NHERF2基因表达缺陷可能参与足细胞损伤脱落的机制。     

原发性IgA肾病402例临床与病理分析

目的分析不同类型IgA肾病（IgAN）的临床与病理特点。方法回顾性分析我院收治的402例IgAN患者。根据其临床表现将其分型,并对各类型的临床表现、组织病理、免疫病理进行回顾性分析。结果临床表现为反复发作性肉眼血尿型（R-GH）85例（21．1％）;单纯性肉眼血尿型（I-GH）19例（4．7％）;无症状性尿检异常（Uab）135例（33．6％）;大量蛋白尿型（MP）96例（23．9％）;高血压型（HT）36例（9．0％）;血管炎或新月体型（Cres）者31例（7．7％）。各类型的IgAN临床表现不同,表现为R-GH和Uab者,病理改变以系膜增殖为多见;MP者57％为系膜增殖,约26％为局灶节段硬化;HT型55％以上的患者为局灶节段硬化;膜增殖病变者主要表现为大量蛋白尿;轻微病变者多为孤立性肉眼血尿,也可见于无症状尿检异常和大量蛋白尿者。结论不同类型的IgAN在临床表现、病理等方面均有显著性差异,提示它在发病机制及治疗上应有所区别。     

IgA肾病合并急性肾损伤患者尿沉渣镜检及临床病理改变分析

目的探讨IgA肾病(IgA nephropathy, IgAN)合并急性肾损伤(acute kidney injury, AKI)患者尿沉渣镜检及临床病理特点。方法该研究为回顾性研究。选取2013年1月31日至2015年7月31日在北京大学第一医院经肾活检确诊的IgAN患者为研究对象, 根据肾活检时是否合并AKI将患者分为AKI组和非AKI组。肾活检当日留取中段晨尿样本行尿沉渣镜检, 观察尿液中细胞及管型等有形成分改变。比较AKI组和非AKI组IgAN患者临床资料、尿沉渣及肾脏病理检查结果的差异。Logistic回归分析法分析临床病理和尿沉渣指标与AKI及尿沉渣指标与IgAN牛津病理分型评分的相关性。结果该研究纳入IgAN患者502例, 年龄(36.1±12.1)岁, 男性261例(52.0%)。IgAN患者肾活检时合并AKI 57例(11.4%), 包括肉眼血尿相关AKI 10例、急性肾小管间质性肾炎10例、新月体性IgAN 9例、恶性高血压肾损伤6例及多种病因或病因不明22例。与非AKI组相比, AKI组患者男性比例、合并恶性高血压比例、24 h尿蛋白量、尿红细胞数及肉眼血尿、白细...     

伴和不伴新月体形成的IgA肾病的临床与病理研究

目的：探讨伴和不伴新月体形成的原发性IgA肾病（IgAN）临床和病理特点。方法：分析128例经肾活检确诊IgAN患者,分析其临床特点,并根据新月体形成所累及肾小球的比例分组：无新月体形成为A组,69例;≤10%为B组,25例;〉10%〈50%为C组,34例。根据Katafushi积分分析肾脏病理。结果：（1）临床方面：B组和C组的血尿素氮（BUN）较A组高（P〈0.05）;C组血肌酐（Scr）较A组高（P〈0.01）;B组和C组的收缩压（SBP）较A组高（P〈0.01）;C组舒张压（DBP）较A组高（P〈0.01）。（2）病理方面：C组肾小球总积分比A组、B组高（P〈0.01）,B组肾小球总积分比A组高（P〈0.01）;B组球性硬化积分比A组高（P〈0.01）,C组球性硬化积分比A组高（P〈0.01）;C组节段损害、肾小管间质、炎症细胞浸润积分比A组高（P〈0.01）。结论：原发性IgAN随着新月体形成所累及肾小球的比例增加,血压有逐渐升高趋势;肾脏病理损害逐渐加重,故对有新月体形成原发性IgAN应早期干预治疗,以延缓肾病进展。     

Glomerular ultrastructures and prognosis of the patients with urinary abnormalities found by school screening program

Detailed histopathological study were performed and compared with clinical features in 120 children with serial renal biopsies who were found by school screening program. 41 cases (34.2%) of IgA nephropathy (IgAN), 26 cases (21.7%) of thin membrane disease (TMD) and 22 cases (18.3%) of normal glomeruli [( Normal]) accounted for 74.2% of all biopsies. 81 cases (67.5%) were revealed to be minor glomerular abnormalities by light microscopy and which contained 26 cases (32.1%) of TMD, 22 cases (27.2%) of [Normal] and 19 cases (23.4%) of IgAN. The frequency and the severity of proteinuria was significantly higher in IgAN than in TMD and [Normal] (P less than 0.01, P less than 0.05). Hematuria was significantly greater in [Normal] than in IgAN. In the 71 follow-up cases, no patient went to renal insufficiency, moreover, urinary abnormalities had disappeared in 25.4% of the patients including IgAN, TMD, [Normal], nonIgA proliferative glomerulonephritis, incomplete foot process disease and MPGN. [Normal] consisted of stationary or exercised urinary abnormality.     

肾小球疾病患者尿沉渣足细胞分子podocin和podocalyxin分析

目的：以ELISA法检测尿沉渣足细胞podocin,podocalyxin排泌并观察其与不同肾小球疾病的关系。方法：共收集我院自2010年5月～8月以来行肾活检证实为肾小球疾病的患者,收集其临床资料,并以ELISA法检测尿沉渣足细胞分子podocin,podocalyxin。结果：共40个患者,男15例,女25例,平均年龄（38.27±16.33）岁,增殖性肾小球疾病患者19例：IgA肾病10例,新月体性肾炎2例,IgM肾病2例,Ⅳ（A/G）型狼疮性肾炎5例;非增殖性肾小球疾病患者19例：微小病变型（MCD）5例,局灶节段硬化性肾小球肾炎（FSGS）8例,膜性肾病（MN）6例,另原发性肾淀粉样变性2例,对照健康自愿者10例。尿podocin分子排泌在正常对照组最低,在增殖性肾小球疾病和非增殖性肾小球疾病间差异无统计学意义（P〉0.05）,增殖性肾小球疾病尿podocin排泌高于肾淀粉样变性患者（P〈0.05）。新月体肾炎的尿podocin排泌显著高于其他肾小球疾病（P〈0.05）,后依次FSGS,IgA肾病,狼疮性肾炎,MN,IgM肾病,MCD;尿沉渣podocalyxin排泌在正常对照组最低,而增殖性肾小球肾炎和非增殖性肾小球肾炎间差异无统计学意义（P〉0.05）。新月体肾炎尿podocalyxin排泄量最高,其后依次为FSGS,IgA肾病,MN,狼疮性肾炎,MCD,IgM肾病,以肾淀粉样变性最低。尿podocalyxin与podocin呈正相关,尿podocin与血C3呈负相关。结论：ELISA法检测尿沉渣足细胞分子检测可对肾小球疾病患者的肾病理类型提供参考,以正常人尿podocin,podocalyxin排泌最少,增殖性肾小球疾病和非增殖性肾小球疾病间差异无统计学意义,新月体性肾炎尿沉渣podocin及Podocalyxin高于其他疾病患者,FSGS患者的尿沉渣podocin及Podocalyxin排泌量也较多,肾淀粉样变性患者的尿沉渣podocin及Podocalyxin最低,血清C3与尿podocin的排泌呈负相关。     

Detection of urinary macrophages expressing the CD16 (Fc gamma RIII) molecule: a novel marker of acute inflammatory glomerular injury

BACKGROUND: The CD16 antigen is the Fc gamma receptor III. CD14+CD16+ cells are proinflammatory monocytes/macrophages (Mo/M phi) that constitute a minor population in the peripheral blood of healthy individuals. Little is known about the expression of CD16 antigen on Mo/M phi in glomerulonephritis. METHODS: Flow cytometric analyses were performed on urine and blood samples obtained from 209 patients with various renal diseases. Patients variously suffered from rapidly progressive crescentic glomerulonephritis (RPGN), membranoproliferative glomerulonephritis (MPGN), postinfectious acute glomerulonephritis (AGN), Henoch-Sch?nlein purpura nephritis (HSPN), IgA nephropathy (IgAN), membranous nephropathy (MN), minimal change nephrotic syndrome (MCNS), lupus nephritis (LN), acute interstitial nephritis, hereditary nephropathy, idiopathic renal hematuria (IRH), and renal stone. RESULTS: The CD16+ M phi population of cells was present in the urine of hematuria-positive patients with proliferative glomerulonephritis, including AGN, IgAN, RPGN, MPGN, and LN with acute inflammatory lesions, such as endocapillary proliferation, tuft necrosis, and cellular crescents. In contrast, the urinary CD16+ M phi population was negligible in hematuria-positive patients with nonproliferative renal disease, including hereditary nephropathy, IRH, and renal stone and also in patients with proliferative glomerulonephritis lacking acute inflammatory lesions. Total urinary M phi of these patients were much less than those of patients having proliferative glomerulonephritis with acute inflammatory lesions. Transient expansion of the CD16+ M phi population in urine was observed during the acute exacerbation of urinary abnormalities, whereas the disappearance of CD16+ M phi closely preceded the amelioration of urinary abnormalities in patients with proliferative glomerulonephritis. In 38 of the 98 patients positive for CD16+ M phi population in urine, the CD16+ Mo population was negligible in peripheral blood. Immunohistochemically, CD16+ M phi were present in the glomeruli of active proliferative glomerulonephritis, whereas such cells were absent in inactive proliferative glomerulonephritis or nonproliferative glomerular diseases. CONCLUSION: CD16+ M phi may be effector cells involved in the acute inflammation common to all types of proliferative glomerulonephritis. Furthermore, the detection of CD16+ M phi in urine, as well as urinary M phi counts, may serve as a useful indicator of the active stage of proliferative glomerulonephritis.     

Acute poststreptococcal glomerulonephritis superimposed on IgA nephropathy

Superimposition of poststreptococcal glomerulonephritis (PSGN) on the course of IgA nephropathy (IgAN) is uncommon. A case of PSGN during IgA nephropathy is presented. A 30-year-old man who had alternating gross and microscopic hematuria for 7 months underwent a renal biopsy. The first renal biopsy revealed IgAN with mesangial deposits of IgA and C3. Two months later, the patient suffered generalized edema, proteinuria, hematuria, an increased ASO titer and a decreased C3 level. A second renal biopsy revealed diffuse endocapillary proliferative glomerulonephritis with epimembranous hump-like electron-dense deposits of C3, but the original mesangial IgA deposits had disappeared. A diagnosis of acute PSGN was indicated. Two months after the onset of acute nephritic syndrome, the patient remained asymptomatic, except for microscopic hematuria and proteinuria. Some cases with persistent proteinuria or hematuria after PSGN are probably related to preexisting IgAN.     

Clinicopathological features of paediatric renal biopsies in Shanghai over a 31 year period

Aim: To identify the variations in paediatric renal biopsy pathology and clinicopathological features during the past 31 years. Methods: A retrospective analysis of paediatric renal biopsies performed at a single institution in Shanghai from January 1979 to December 2009 was conducted. Results: The major pathologies included minor glomerular abnormalities (MGA, 26.1%), IgA nephropathy (IgAN, 17%) and mesangial proliferative glomerulonephritis (MsPGN) without IgA deposition (11.3%). The major clinical presentations included nephrotic syndrome (NS, 39.4%), haematuria with proteinuria (24.4%) and persistent microscopic haematuria (15.1%). MGA accounted for 46.9% of the cases in NS. IgAN and HSN accounted for 24% and 28.9% of patients with concomitant haematuria and proteinuria, and thin basement membrane nephropathy accounted for 51.2% of cases with persistent microscopic haematuria. The frequency of IgAN (78.6%) was much higher than that of TBMN (29.0%) in patients with persistent microscopic haematuria with abnormal urinary albumin. Conclusion: Minor glomerular abnormalities and IgAN were the major renal diseases in our study population, and the focus of our paediatric nephrologists. The high proportion of TBMN suggested that there should be limited use of renal biopsy for patients with persistent microscopic haematuria and renal biopsy should be performed in the presence of proteinuria or abnormal levels of urinary albumin.     

Idiopathic hypercalciuria: association with isolated hematuria and risk for urolithiasis in children. The Southwest Pediatric Nephrology Study Group

A prospective multicenter study was designed to determine the frequency and prognostic importance of hypercalciuria in children with hematuria. Urinary calcium excretion was examined in 215 patients with unexplained isolated hematuria (no proteinuria, urolithiasis, infection or systemic disorder). Hypercalciuria (urinary calcium excretion greater than 4 mg/kg/day) was identified in 76 patients (35%). Compared to patients with normal urinary calcium excretion, children with hematuria and hypercalciuria were characterized by male preponderance, white race, family history of urolithiasis, gross hematuria and calcium oxalate crystals. Renal biopsies were performed in 10 patients with urinary calcium excretion 0.4 to 2.5 mg/kg/day; three had IgA glomerulonephritis, three had glomerular basement membrane thinning, one had proliferative glomerulonephritis and three were normal. Renal biopsies in three patients with hypercalciuria showed focal segmental glomerulosclerosis, hereditary nephritis or no abnormalities. Oral calcium loading tests showed renal hypercalciuria in 26 patients, absorptive hypercalciuria in 15 patients and were not diagnostic in 35 patients. Serum parathyroid hormone, bicarbonate and phosphorus and urinary cyclic adenosine monophosphate concentrations were similar in the three groups of hypercalciuric patients. Urinary calcium excretion after one week of dietary calcium restriction was higher (5.8 mg/kg/day) in renal hypercalciuria than in other hypercalciuric patients (3.4 mg/kg/day), P less than 0.01. One to four years follow-up was available for 184 patients. Eight of 60 hypercalciuric patients developed urolithiasis or renal colic compared to 2 of 124 patients with normal urinary calcium excretion (P less than 0.001). Hypercalciuria is commonly associated with isolated hematuria and represents a risk factor for future urolithiasis in children with hematuria.(ABSTRACT TRUNCATED AT 250 WORDS)     

Pathology, clinical presentations, and outcomes of C1q nephropathy

C1q nephropathy is an uncommon glomerular disease with characteristic features on immunofluorescence microscopy. In this report, clinicopathologic correlations and outcomes are presented for 72 patients with C1q nephropathy. The study comprised 82 kidney biopsies from 28 children and 54 adults with male preponderance (68%). Immunofluorescence microscopy showed dominant or co-dominant staining for C1q in the mesangium and occasional glomerular capillary walls. Electron-dense deposits were observed in 48 of 53 cases. Light microscopy revealed no lesions (n = 27), focal segmental glomerulosclerosis (FSGS; n = 11), proliferative glomerulonephritis (n = 20), or various other lesions (n = 14). Clinical presentations in the patients who had no lesions histology were normal urine examination (7%), asymptomatic hematuria and/or proteinuria (22%), and nephrotic syndrome (minimal change-like lesion; 63%), which frequently relapsed. All patients with FSGS presented with nephrotic syndrome. Those with proliferative glomerulonephritis usually presented with chronic kidney disease (75%) or asymptomatic urine abnormalities (20%). Of the patients with sufficient follow-up data, complete remission of the nephrotic syndrome occurred in 77% of those with a minimal change-like lesion, progression to end-stage renal disease occurred in 33% of those with FSGS, and renal disease remained stable in 57% of those with proliferative glomerulonephritis. In conclusion, this study identified two predominant clinicopathologic subsets of C1q nephropathy: (1) Podocytopathy with a minimal change-like lesion or FSGS, which typically presents with nephrotic syndrome, and (2) a typical immune complex-mediated glomerular disease that varies from no glomerular lesions to diverse forms of glomerular proliferation, which typically presents as chronic kidney disease. Clinical presentation, histology, outcomes, and presumably pathogenesis of C1q nephropathy are heterogeneous.