Sideropenic Dysphagia综合征膜性蹼26例分析

目的 :研究SideropenicDysphagla (S -D)综合征膜性蹼产生的机制。方法 :回顾分析S -D综合征2 6例的临床资料。结果 :影像学检查显示膜性蹼在颈段食道前壁呈 2mm深的模样陷凹。血液学检查为缺铁性低血红蛋白性贫血改变。结论 :缺铁性贫血是S -D综合征的原因 ,铁剂治疗有效     

活血软坚方对大鼠膜性肾小球肾炎肾小管间质损害影响的实验研究

目的：观察活血软坚方对大鼠膜性肾小球肾炎（MN）肾小管间质损害的影响，并探讨活血软坚中药对MN伴发小管间质损伤的作用机制。方法：用阳离子化牛血清白蛋白复制大鼠MN模型，将实验动物随机分为正常组、模型组、雷公藤组、治疗组，观察24h尿蛋白、血浆白蛋白、胆固醇、三酰甘油、血肌酐、尿素氮等生化指标，并对肾组织进行光镜、电镜、免疫荧光观察；采用RT—PCR的方法检测ColⅠmRNA和ColⅢmRNA的表达。结果：本方能明显降低蛋白尿及血清胆固醇、三酰甘油、血肌酐、尿素氮，升高血清白蛋白，减少免疫复合物沉积，改善肾小球及肾小管的病理损伤。结论：活血软坚方能减轻尿蛋白对肾小管的损伤，减少细胞外基质在肾间质的积聚，减轻肾脏病理损伤，从而达到保护肾功能的作用。     

Intramembranous fine deposit disease associated with collagen disorders: a new morphological entity?

Summary A distinct, hitherto unknown renal histopathological appearance, consisting of diffuse thickening of the glomerular basement membrane (GBM) with fine intramembranous electron-dense deposits, was observed in the renal biopsies from three patients with collagen diseases. In each case, proteinuria was mild with normal urinary sediment. On light microscopy there were no particular abnormalities but a mild thickening of the glomerular capillary wall. Immunofluorescence studies revealed faint linear or extremely fine granular IgG deposition along the capillary wall. On electron microscopy, the GBM was diffusely thickened with fine intramembranous electron-dense deposits without spike formation. No other deposits were seen in the glomerulus. These histological features resembled those of membranous glomerulonephritis (MGN), although the possibility of the early change of MGN is excluded by specific findings in these cases. Other GBM-thickening diseases such as diabetic glomerulosclerosis were ruled out clinically and histologically. Our cases have a singular renal histopathology which differs from any of the previously established classifications of glomerular lesions. It may be a specific change associated with some type of collagen disease.     

Membranous laryngotracheobronchitis,a complication of measles

Membranous laryngotracheobronchitis is a very serious infection which affects the larynx, trachea and bronchi, requiring aggressive therapeutic measures. It has been recently rediscovered as a cause of disease in children. However, it is a very unusual complication of measles. Two infants with measles and membranous laryngotracheobronchitis are reported.     

Membranous lipodystrophy

Summary The case is described of a 35-year-old housewife diagnosed as having membranous lipodystrophy (as described by Nasu et al. in 1970 and called lipomembranous polycystic osteodysplasia by Hakola in 1972). The main symptom of this patient was a slowly progressive dementia. Skeletal symptoms were not seen. The computerized tomogram of the brain showed calcification of bilateral basal ganglia and the plain roentgenograms of the bones revealed cystic radiolucent areas at the distal end of the bones of the patient's extremities. Histological examination of the curetted material from the right talus revealed a membranocystic pattern. The fatty tissue curetted from the cyst of the talus and the lysosomal enzymes of the white blood cells were biochemically normal. A possible relationship between this disease entity and connective disorders is considered.     

C1q的沉积对V型狼疮性肾炎和原发性膜性肾病的鉴别诊断意义

目的:研究C1q在V型狼疮性肾炎、原发性膜性肾病及病理组织学为不典型膜性肾病肾活检标本中的沉积,分析其不同及意义。方法:对V型狼疮性肾炎、原发性膜性肾病和不典型膜性肾病的患者的肾活检组织进行C1q免疫组化染色,并收集临床和血清学指标,进行统计学分析。结果:V型狼疮性肾炎会出现C1q的沉积,原发性膜性肾病不会出现C1q的沉积,一些病理组织学表现不典型的膜性肾病,会出现C1q的沉积,后者阳性率与狼疮性肾炎接近,与膜性肾病相比,差异有统计学意义。结论:C1q阳性且病理组织学为不典型膜性肾病的患者,极有可能是早期的V型LN。     

两种膜分离器对血浆置换后血细胞数及血生化影响的比较

目的：比较两种不同材料制备的膜型血浆分离器PS-06与Evacure-4A在人工肝血浆置换治疗后对患者血细胞数、肾功能、血电解质及血氨的影响．方法：在内科综合治疗基础上联合人工肝血浆置换治疗患者112例,应用KM-8800型血浆置换装置(Kuraray)和PS-06、Evacure-4A两种膜型血浆分离器,其中应用PS-06膜型血浆分离器(A组)54例,应用Evacure-4A膜型血浆分离器(B组)58例,检测治疗前后的血常规、肾功能、血电解质及血氨．结果：患者术后各项指标的增减百分率比较,其中血小板(PLT)、血红蛋白(Hb)、血氨(NH_3)下降率(治疗后/治疗前)A组与B组间有显著性差异(PLT：92．2%±14．8% vs 99．8%±22．4%,P＜0．05；Hb：88．1%±9．7% vs 94．8%±3．8%,P＜0．01；NH_3：81．2%±22．7% vs 66．6%±13．7%,P＜0．01),其他各项指标增减百分率无统计学意义(P＞0．05)．结论：人工肝血浆置换治疗中Evacure-4A膜型血浆分离器对血小板无明显影响,对血红蛋白影响较小,并能更有效地清除患者体内尿素氮、肌酐及血氨等毒性代谢产物,明显优于PS-06膜型血浆分离器．     

应用全基因组拷贝数变异方法探索参芪膜肾方治疗特发膜性肾病基因水平机制的研究

目的：探索特发膜性肾病患者全基因组拷贝数变异与参芪膜肾方疗效的关系。方法：选取80例经参芪膜肾方或经典的激素联合环磷酰胺方案治疗后完全缓解和无效的IMN病例,分为中药有效组（36例）、中药无效组（11例）、西药有效组（18例）和西药无效组（15例）。提取外周血基因组DNA并应用Affymetrix Genome-Wide Human SNP Array 6.0芯片检测全基因组基因拷贝数,应用CNVhac软件进行拷贝数变异（ CNV）分析。结果：中药有效组与中药无效组在第5、第6及第8染色体上检测到的CNVs差异具有统计学意义（P＜0.05）。其中位于6号染色体上的HLA族基因在中药有效组多数病例中表现为拷贝数扩增,而中药无效组的多数病例则表现为拷贝数缺失。西药有效组与西药无效组之间未检测到具有统计学差异的CNVs。结论：基因背景差异可能是导致参芪膜肾方取得不同疗效的基因水平机制,HLA的同族基因拷贝数变异影响参芪膜肾方疗效的发挥,前者有望成为该方治疗IMN的疗效预测因子,值得进一步深入研究。     

Glomerulopatía por invaginación podocítica; reporte del primer caso en Latinoamérica y revisión de la literatura

BackgroundPodocyte infolding glomerulopathy (PIG) is a condition of uncertain origin, frequently associated with autoimmune diseases. Its specific treatment and clinical course are unknown.It is characterised by thickening of the capillary walls due to the presence of non-argyrophilic intramembranous bubbles similar to those found in membranous glomerulopathy, but without electron-dense deposits of immune complexes in the ultrastructure, where translucent microspheres generated by invagination of the podocyte cytoplasm into the basement membranes are observed.ObjectivesGenerally reported in young females patients. To date, few cases in Asian patients have been reported. Our case is the first to be reported in a Latin American Caucasian patient.MethodsA 38-year-old woman with SLE. In 2014 she presented with nephrotic syndrome empirically treated with corticosteroids (CO) and intravenous cyclophosphamide with good response. She had a relapse in April 2015 with normal renal function and no extrarenal lupus activity, so she was referred to our hospital to be biopsied.ResultsThe biopsy reported focal segmental glomerular sclerosis without deposits of immune complexes in the immunofluorescence. However, methenamine silver staining revealed clear spaces in the capillary walls accompanied by marked podocyte alterations. On electron microscope study, numerous aggregates of microvesicular and cylindrical ultrastructures bound to the membranes were observed, without evidence of dense deposits, and diffuse effacement of pedicel foot processes, confirming the suspected diagnosis.ConclusionsThis is the first reported case of what can be considered a new pathological glomerular entity in a Latin American Caucasian patient, whose clinical course and therapy are still unknown.