The clinical and pathological characteristics of Chinese patients with pauci-immune crescent golmerulonephritis

Objective　To investigate the clinical and pathological characteristics of pauci-immune crescent glomerulo～nephritis (PICGN) in Chinese patients. Methods　During 13 years (1985-1998), 6400 patients underwent non-transplanting renal biopsy. Twenty-four patients were diagnosed as PICGN. All clinical and laboratory data of these patients were collected from the patients’ records and used for detailed analysis. The diagnosis is based on clinico-pathologic findings. Results　Of the 24 patients, 16 were females and 8 were males, with median age of 33 years (ranged 10-76 years). Microscopic polyarteritis (MPA) (33.3%) and systemic vasculitis (8.3%) were the secondary diseases. The incidence of PICGN was 0.38% in renal biopsies and 22.9% in crescentic glomerulonephritis. Clinically, most patients (75.0%) showed rapidly progressive nephritis with enlarged kidneys. At onset, gross hematuria was noted in 58.3% of patients, hypertension in 45.8%, nephrotic syndrome in 41.7%, and oliguria in 25.0%. However, systemic symptoms were rare except for anemia. Pathologically, necrosis of glomerular capillaries (62.5%), infiltration of monocytes and neutrophil cells in glomeruli (66.7%), and vasculitis in the interstitium (53.3%) were observed. In addition, glomerulosclerosis was noted in 45.8%, severe tubular atrophy in 83.3% and interstitial fibrosis in 75.0%. Anti-neutrophil cytoplasmic antibodies (ANCAs) were positive in 52.2%. All patients except two received intensively immunosuppressive therapy. Sixteen patients were available for long-term follow up (median 29.8 months, range 8-92 months). Twelve of them had life-sustaining renal function, four had normal serum creatinine (<124?μmol/L) and only 4 patients were dialysis-dependent. Conclusion　PICGN is not rare in China. Early diagnosis and administration of immunosuppressive therapy, particularly in patients with rapidly progressive glomerulonephritis (RPGN), are important for good prognosis.     

内啮合齿轮泵月牙板与齿面间的润滑性能

内啮合齿轮泵在工业实际中应用广泛,固定月牙块与外齿轮齿面间的润滑性能是其性能的重要方面。本文构建了能够对内啮合齿轮泵月牙板与齿面间流体润滑的数值计算模型,选取LL4724型内啮合齿轮泵为对象进行数值计算研究,系统研究了压油腔压力、齿顶间隙、小齿轮转速等因素对小齿轮齿顶与月牙板下表面之间的油膜压力分布及周向流量的影响规律,得出了具体的规律性曲线,并对这些曲线进行了分析。     

Anterior fracture dislocation of sacroiliac joint: A rare type of crescent fracture

Crescent fractures of the pelvis are usually described as posterior sacro iliac fracture dislocations. Rarely anterior displacement of the fractured iliac fragment along with dislocation has been reported in crescent fractures. Four cases of anterior fracture dislocation of the sacro iliac joint managed in the last two years by a single surgeon are presented. The injury mechanism, radiological diagnosis, management protocol along with functional outcomes of all the four patients have been discussed. CT scan is essential in the diagnosis and preoperative planning of this injury pattern. Early fixation along with proper reduction leads to excellent functional outcome in this subset of lateral compression injuries of the pelvis.     

结膜新月形切除联合巩膜固定术治疗中重度结膜松弛症的疗效观察

目的：观察结膜新月形切除联合巩膜固定术治疗中重度结膜松弛症的临床疗效。方法采取结膜新月形切除联合巩膜固定术对27例（41眼）中重度的结膜松弛症患者进行治疗,术后随访1年。结果术后37眼裂隙灯下检查松弛的球结膜完全消除,结膜褶皱消失,结膜切口愈合良好,有效率为90.24％。结论结膜新月形切除联合巩膜固定术是治疗中重度结膜松弛症的安全可靠、疗效满意的方法。     

吲哚胺2,3-双加氧酶在新月体肾炎肾组织中的表达变化

目的 检测新月体肾炎患者肾组织中吲哚胺2,3-双加氧酶(indoleamine 2,3-dioxygenase,IDO)的表达情况及其与肾组织浸润炎细胞的增殖和肾小管-间质病理损害程度之间的关系.方法 采用免疫组织化学染色法检测正常肾组织和新月体肾炎患者肾组织IDO的表达情况,并进一步分析其表达与肾间质浸润PCNA阳性炎细胞数及肾小管-间质病理损害程度之间的相关关系.结果 正常肾组织未检测到IDO表达,而在新月体肾炎患者肾小管上皮细胞IDO的表达显著上调,并且新月体肾炎肾小管上皮细胞IDO的表达强度与肾小管-间质PCNA阳性浸润细胞数及肾小管-间质病理损害程度显著负相关.结论 IDO在新月体肾炎肾小管上皮细胞中高表达并与肾小管-间质炎细胞增殖及小管-间质病理损害相关,提示IDO可能通过抑制炎细胞增殖活性参与新月体肾炎肾小管-间质病理损害过程.     

新月体狼疮肾炎的治疗效果

目的:探讨不同治疗方法对新月体狼疮肾炎(CLN)病人的疗效.方法:临床及病理检查确诊为CLN病人41例,根据不同的免疫抑制治疗方法分为三组:A组11例,甲基强的松龙(MP)冲击合并霉酚酸酯(MP MMF)治疗;B组20例,MP冲击合并间断环磷酰胺(MP CTX)静脉治疗;C组10例,MP冲击治疗;观察三组治疗方案的近、远期疗效.结果:近期疗效:病人血清肌酐(SCr)从治疗前(320±241)μmol/L降至(195±141)μmol/L,蛋白尿和贫血也均有不同程度的改善;远期疗效:比较A组和B组病人的远期疗效无明显差异,而优于C组.结论:MP CTX和MP MMF这两种方法诱导缓解CLN,近、远期疗效相似,均优于单独MP治疗组.     

以IgA沿肾小球毛细血管襻沉积为主的急进性肾小球肾炎

目的:明确以IgA沿肾小球毛细血管襻沉积为主的急进性肾小球肾炎的临床与病理特点。方法:分析解放军总医院全军肾脏病研究所收治的1例以IgA沿肾小球毛细血管襻沉积为主的急进性肾小球肾炎病例,分析其临床特点、病理与电镜特征,以及对强化免疫抑制治疗的反应。结果:该病例临床表现为急进性肾炎综合征,肾功能恶化发展迅速。但无肺出血及全身性血管炎症状。血清自身抗体系列、抗GBM抗体与ANCA均为阴性。病理光镜示肾小球环绕型新月体形成,肾小血管无炎症改变。冰冻切片与石蜡微波修复直接免疫荧光示IgA沿肾小球毛细血管襻细颗粒状沉积,间接免疫荧光法检测血清IgA型抗GBM为阴性;电镜示节段性上皮下、基底膜内及系膜区电子致密物沉积。对强化激素冲击与免疫抑制剂治疗效果欠佳。结论:本病例为特殊类型的急进性肾小球肾炎,根据其临床特点、血清学检测、免疫荧光及电镜检查结果,无法归于目前急进性肾炎的分型,对其临床特点应予重视。     

心包新月征在非钙化性缩窄性心包炎CT诊断中的价值

目的探讨心包新月征在非钙化性缩窄性心包炎CT诊断中的价值。方法选择非钙化性缩窄性心包炎患者60例作为心包炎组,心脏肿瘤患者60例作为心脏肿瘤组,所有患者均进行CT检查,比较两组患者的心包新月征比例,并观察非钙化性缩窄性心包炎的主要影像表现。结果心包炎组患者CT出现心包新月征37例,占61.67%;心脏肿瘤组患者出现心包新月征2例,占3.33%;心包炎组患者的心包新月征比例显著高于心脏肿瘤组(P <0.05)。缩窄性心包炎的CT征象主要表现为:下腔静脉扩张、胸腔积液、新月征,分别占91.67%、 78.33%、 61.67%。结论心包新月征在非钙化性缩窄性心包炎的诊断中具有重要的临床应用价值,能够作为疾病诊断和鉴别诊断的依据。     

Myeloid cell‐mediated renal injury in rapidly progressive glomerulonephritis depends upon spleen tyrosine kinase

Antibody‐dependent activation of myeloid cells within the glomerulus plays a central role in rapidly progressive forms of glomerulonephritis. The spleen tyrosine kinase (Syk) is expressed by all leukocytes, except mature T cells, and is required for signalling via the B‐cell receptor, Fc receptors, and some integrins. Syk has been proposed as a therapeutic target in glomerulonephritis. However, little is known of Syk activation in human kidney disease, while studies in experimental glomerulonephritis using non‐selective Syk inhibitors require validation via conditional gene deletion. The current study addressed both of these important points. Syk activation (Tyr^525/526 phosphorylation) was examined in a cohort of 96 patients with different glomerulonephritides. Syk activation was evident in infiltrating leukocytes, mainly neutrophils and macrophages, in 36/40 cases of rapidly progressive glomerulonephritis. In contrast, non‐proliferative diseases showed little or no Syk activation. Glomerular and interstitial cells exhibiting Syk activation correlated with renal function and systemic inflammation. Next, we examined mice with conditional Syk gene deletion in myeloid cells (Syk^My) versus Syk^f/f littermate controls in nephrotoxic serum nephritis – a model of rapidly progressive glomerulonephritis. Control Syk^f/f mice featured a transient neutrophil influx at 3 h and severe disease on day 9 of nephrotoxic serum nephritis, with crescent formation, macrophage infiltration, inflammation, kidney fibrosis, and renal dysfunction. In contrast, Syk^My mice had significantly reduced neutrophil and macrophage infiltration despite equivalent glomerular deposition of humoral reactants. Syk^My mice exhibited reduced crescent formation, inflammation, and fibrosis, with improved renal function on day 9 of nephrotoxic serum nephritis. In conclusion, Syk activation is prominent in infiltrating myeloid cells in human rapidly progressive glomerulonephritis, and functional studies demonstrate that Syk deletion in myeloid cells is protective in mouse nephrotoxic serum nephritis. Copyright © 2015 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd.