中老年IgA肾病的临床病理特征和转归

目的　探讨中老年IgA肾病临床和病理特性及其与预后的关系。方法　分析 65例 45岁以上IgA肾病临床和病理特征 ,并与 659例年轻患者 (≤ 45岁 )比较。分析肾存活率并与年轻组比较。结果　中老年组血胆固醇、甘油三酯以及高血压或肾功能不全发生率显著高于年轻组 (P<0 0 5)。≥ 60岁亚组收缩压和尿蛋白显著高于 45～ 59岁亚组 (P <0 0 5 )。两组Lee氏病理分级分布无显著性差别 (P >0 0 5)。中老年组肾小管 间质指数和血管指数显著高于年轻组 (P <0 0 5) ,但肾小球指数和新月体指数无显著性差异 (P >0 0 5)。两组 5年肾存活率均为 85 6 % ,无显著性差别(P >0 0 5)。结论　中老年IgA肾病高血压、高脂血症和肾功能不全发生率较高 ,肾血管和肾间质损害比较突出 ,故应注意积极控制血压和高脂血症。     

IgA肾病合并高血压患者的临床与病理特点分析

目的探讨IgA肾病合并高血压患者的临床与病理特点。方法根据血压状况将317例IgA肾病患者分为高血压组119例和非高血压组198例,分析两组临床和病理特点。结果高血压组肾功能不全的发生率明显高于、血尿的发生率明显低于非高血压组(P<0.05);高血压组24 h尿蛋白定量、尿NAG酶、血肌酐、血尿酸及年龄均显著高于非高血压组,血浆白蛋白水平显著低于非高血压组(P均<0.05);高血压组肾小球硬化指数、间质指数和血管指数均高于非高血压组,病理损害程度为重度者的比例明显高于非高血压组(P均<0.05)。结论合并高血压的IgA肾病患者临床上高尿酸血症、贫血、肾功能不全等的发生率高,肾脏病理损害程度较重,其可能的临床风险因素是高蛋白尿及高血肌酐水平;可能的病理风险因素为肾小球硬化、间质小管病变及肾血管病变。     

CD71在IgA肾病肾组织中的表达与临床病理关系

目的研究CD71在人类IgA肾病肾组织中的表达强度及与IgA肾病临床病理关系,分析其作为IgA肾病病理和预后标记物的可能性。方法选取该院180例肾穿刺活检为IgA肾病患者,收集患者临床资料,采用光镜、免疫组织化学、免疫荧光、IgA肾病半定量评分等方法检测IgA肾病组肾组织中CD71的表达,并与正常对照组比较;分析CD71与病理严重程度及临床的相关性。结果 (1)在IgA肾病组,CD71的表达明显高于正常对照组(P0.05),且CD71的表达强度随肾组织病理损害加重而增强;(2)IgA肾病肾组织中CD71表达量与IgA肾病组织学半定量评分法中的系膜细胞增生指数(Ms HI)和系膜基质增多指数(Ms MI)呈正相关(P0.05);(3)IgA肾病肾组织中CD71的表达量与患者的蛋白尿水平呈正相关,与肌酐清除率水平呈负相关(P0.05),而与患者血尿水平无明显相关性(P0.05)。结论 CD71在IgA肾病不同病理肾组织表达不同,随病理损害的加重而增强,且CD71的表达与反应系膜细胞和系膜基质增多指数相关,提示CD71与系膜细胞增殖关系密切;另外,CD71表达量与蛋白尿呈正相关,与肌酐清除率呈负相关,提示其与IgA肾病肾功下降及预后不良有关。因此,肾组织CD71的表达可作为判断该症严重程度和预后的生物学标记物之一。     

影响IgA肾病预后的危险因素分析

目的通过分析IgA肾病患者的临床资料及病理特征,探讨影响IgA肾病患者长期肾存活率的危险因素。方法分析724例肾活检确诊为IgA肾病患者肾活检时的临床资料及病理特征。对所有患者进行随访,每3~6个月检测尿蛋白、血肌酐(Scr)等指标,以Scr值比基础值升高1倍以上为观察终点。随访时间>6个月者才纳入成功随访病例。用非参数乘积限估计法(Kaplan-Meier法)分析生存率,用Cox回归模型分析影响预后的危险因素。结果共有317例IgA肾病患者成功随访,肾活检后平均随访时间为(43·5±32·2)个月。有39例(12·3%)患者进入随访终点,其1、3、5、10年肾存活率分别为99·5%、93·1%、84·5%和60·1%。Cox比例风险模型单因素分析发现病程长、肾活检时血Scr>115μmol/L、尿蛋白>1·0g/24h、高血压、Lee氏分级Ⅳ级或Ⅳ级以上、中重度肾小球硬化、新月体形成、中重度肾间质纤维化和肾小血管损害是影响IgA肾病预后的危险因素;多因素分析结果显示,蛋白尿、血Scr水平、肾小球硬化、新月体形成、肾间质纤维化是影响IgA肾病预后的独立危险因素。结论蛋白尿、肾功能不全、肾小球硬化、新月体形成和肾间质纤维化是影响IgA肾病预后的独立危险因素。     

以毛细血管内皮细胞弥漫增生为主要表现的IgA肾病的临床病理特点及其预后

目的探讨以毛细血管内皮细胞弥漫性增生为主要表现的IgA肾病(EPIgAN)的临床、病理特点和预后。方法分析北京大学第一医院近15年来IgA肾病(IgAN)的临床和病理资料,比较EPIgAN与非EPIgAN临床病理特点和肾脏存活率;分析EPIgAN预后及其影响因素;观察激素治疗对EPIgAN预后的影响。结果920例IgAN中符合EPIgAN47例,占5·1%。EPIgAN与非EPIgAN相比,肾穿时尿蛋白升高、高血压和水肿多见,细胞新月体明显,而肾小球硬化和肾间质纤维化则较轻;对所有研究对象进行随访,其中36例EPIgAN患者完成随访,平均随访62个月。100例非EPIgAN患者完成随访,平均随访时间64个月。两组共7例到达随访终点。Kaplan-Meier分析两组自然预后差异无显著性(LogRank,P=0·52);Cox回归分析内皮弥漫增生不是影响IgAN预后的危险因素(P=0·27);激素治疗能降低EPIgAN尿蛋白,但随访期内肾脏存活率与对照组差异无显著性。结论EPIgAN肾穿时临床表现重、组织活动性病变多而慢性化指标少,内皮弥漫增生不是影响IgAN预后的危险因素。     

老年患者IgA肾病的临床与病理分析

目的了解老年IgA肾病的临床与病理特点。方法回顾性对比分析该院近20年老年IgA肾病(老年组)患者并将同期年龄<60岁的患者作为对照组。结果①两组均以男性多见,分别占59.18%(29/49)及60.71%(924/1 522),两组在性别构成方面差异无统计学意义(P>0.05);②两组的患者占该院同期同年龄肾活检患者的比例分别是12.60%及35.99%,二者差异显著(P<0.01);③老年患者在发生水肿、高血压、尿素氮、肌酐升高比例、合并肾功能不全、尿蛋白定量均较对照组高,而血浆白蛋白低于对照组(P<0.05)。血尿的发生率两组无差别(P>0.05);④临床诊断老年患者慢性肾小球肾炎及肾病综合征常见,而对照组则以慢性肾小球肾炎及隐匿性肾小球肾炎常见(P<0.01);⑤血中IgG、IgA、IgM、C3、C4平均浓度、IgA/C3浓度之比两组差别无统计学意义(P>0.05)。结论①IgA肾病男性多于女性,老年患者较年轻患者少见;②老年患者临床表现较年轻患者重。肾病综合征表现明显高于年轻患者;③IgA浓度及血清IgA/C3比值对IgA肾病的诊断有一定的帮助。     

老年IgA肾病患者不同部位血管内皮生长因子水平及其与尿蛋白的相关性

目的探讨老年IgA肾病患者不同部位血管内皮生长因子(VEGF)水平及其与尿蛋白的相关性。方法收集该院经肾活检确诊的67例IgA肾病老年患者资料,采用免疫组化法检测肾脏组织VEGF的表达情况,酶联免疫吸附法分别检测血和尿VEGF水平并分析三者在不同病理参数组间分布情况,采用直线回归分析尿蛋白与VEGF的相关性,分析不同尿蛋白和VEGF组合的生化指标和治疗效果。结果 (1)IgA肾病组的尿和血VEGF水平高于健康对照组和狼疮性肾炎组,肾组织VEGF表达水平高于狼疮性肾炎组(P<0.01);且IgA肾病的VEGF水平在Lee氏分级、肾小管间质损伤和Katafuchi总积分不同程度组间分布有统计学差异,且随着病理严重程度的增加而升高;(2)IgA肾病不同程度尿蛋白患者的肾组织VEGF表达、尿和血VEGF水平有统计学差异(P<0.05),尿蛋白水平与肾组织VEGF表达(r=0.59,P<0.01)、尿VEGF(r=0.46,P<0.01)和血VEGF(r=0.37,P<0.05)呈正相关;(3)IgA肾病同一尿蛋白水平不同VEGF水平组间的sAlb、血IgA、Scr、eGFR、β2-MG、尿渗透压、BUN和治疗显效率有统计学差异(P<0.05)。结论 IgA肾病老年患者肾脏组织的VEGF表达增强、血和尿VEGF水平升高,均与尿蛋白呈正相关,且不同尿蛋白和VEGF组合的病理指标和治疗效果差异显著。     

伴高尿酸血症的IgA肾病临床与病理分析

目的 探讨血清尿酸对IgA肾病临床、病理及预后的影响.方法 回顾性分析我院2007年1月至2010年10月456例经肾穿刺活检病理确诊为原发性IgA肾病住院患者的临床和肾脏病理特点资料.采用t检验和x2检验进行统计学处理.结果 456例IgA肾病患者中高尿酸血症者127例,发生率为27.9％,高尿酸血症组平均年龄、男性所占比例、高血压发生率、血清胆固醇、甘油三酯、体质量指数、肌酐、尿蛋白定量(24h)水平显著高于血尿酸正常组(P＜0.05,P＜0.01);高尿酸血症组肾组织病理病变程度显著重于血尿酸正常组(P＜0.01),分别为肾小球积分(8.1±0.8和5.3±0.9),肾小管间质积分(4.2±0.4和2.7±0.4),血管病变积分(1.43±0.60和0.76±0.29).结论 高尿酸水平对IgA肾病有明显影响,积极降低血清尿酸,有效控制上述临床指标,可望减轻肾组织损害,延缓IgA肾病的进展.     

IgA肾病患者肾内小动脉病变的临床及病理意义

目的建立IgA肾病小动脉病变评估标准;了解伴或不伴高血压的IgA肾病患者肾内小动脉病变状态及其意义。方法纳入首都医科大学附属北京安贞医院肾内科2010年3月至2013年10月确诊为IgA肾病的患者305例,用计算机图像分析软件测量肾内小动脉内、外直径,评估管壁增厚程度,分析它们与临床及病理表现的联系。结果小动脉病变评估标准:肾内小动脉内外径比0.48正常,0.45~0.48轻度增厚,0.45重度增厚。IgA肾病患者管壁增厚组的收缩压、舒张压、血肌酐(sCr)及缺血硬化肾小球(IGS)百分比较正常组均显著升高(P0.05),尿渗透压(UOP)显著降低(P0.05)。在血压正常的IgA肾病患者中,管壁增厚组的sCr及IGS百分比较正常组均显著升高(P0.05),UOP均降低,但仅重度组差异有统计学意义(P0.05)。结论正常及高血压的IgA肾病患者均常出现肾内小动脉病变,管壁增厚,其常合并较重的肾小球及肾小管功能损伤。     

呈发作性肉眼血尿的IgA肾病22例临床分析

目的:总结伴有肉眼血尿的IgA肾病患者临床及病理特点。方法:选取经肾活检病理诊断为IgA肾病的患者76例,根据患者病程中是否出现肉眼血尿分为研究组22例和对照组54例。应用Katafuchi半定量积分法分析患者肾脏病变程度,比较2组患者病理学改变及临床特点。结果:①研究组患者前驱感染发生率显著高于对照组,高血压发生率及血肌酐水平低于对照组,平均病程短于对照组(均P<0.05)。2组患者24h尿蛋白排泄量无统计学差异(P>0.05)。②研究组患者肾脏病理积分、血管积分、球硬化积分及血管壁增厚积分低于对照组(均P<0.05)。结论:伴有肉眼血尿的IgA肾病患者发病期多伴有前驱感染,病程中高血压发生率、血肌酐水平较不伴有肉眼血尿的IgA肾病患者低;病理改变较不伴有肉眼血尿的IgA肾病轻,预后相对较好。     

Antihypertensive therapy with diltiazem and comparison with hydrochlorothiazide

Fourteen hypertensive patients with a mean sitting systolic and diastolic blood pressure (BP) of 153 +/- 16/100 +/- 4 mm Hg were treated successively with hydrochlorothiazide and diltiazem for 8 weeks each. The BP response and changes in heart rate, left ventricular size and function, and plasma catecholamine concentrations and renin activity were monitored. The 2 drugs had comparable antihypertensive effects, with mean decreases of 14, 9 and 11 mm Hg for the sitting, standing and supine diastolic BP, respectively, during hydrochlorothiazide treatment and mean decreases of 16, 18 and 12 mm Hg during diltiazem treatment. Heart rate was unchanged, although plasma norepinephrine concentrations increased significantly during diltiazem treatment. Plasma renin activity increased slightly, from 0.6 to 0.9 ng/ml/hour during diltiazem treatment, but the change was not significant (p less than 0.10). Left ventricular ejection fraction and end-diastolic volume were not affected by either agent. In conclusion, diltiazem is an effective antihypertensive agent, which because of its benign side effect profile, may be useful as a step 1 agent.     

Beta blocker overdose with propranolol and with atenolol

During a one-month period, two cases of beta-adrenergic blocker overdose were treated by the emergency staff at our hospital. One case of propranolol intoxication demonstrated profound cardiovascular collapse and generalized tonic-clonic seizures. The condition failed to respond to high-dose intravenous pressor agents, but did improve significantly with IV glucagon infusion. The second overdose involved atenolol. Although the blood levels reported were very high, the patient showed no cardiovascular compromise and required only inhaled bronchodilators for an exacerbation of her asthma.     

Safer colonoscopy with patient-controlled analgesia and sedation with propofol and alfentanil.

BACKGROUND: The aim of this study was to assess the efficacy of patient-controlled analgesia and sedation with propofol/alfentanil for colonoscopy compared with continuous drug infusion and conventional nurse-administered medication. METHODS: One hundred fifty patients undergoing colonoscopy on an outpatient basis were randomly assigned to 1 of 3 medication regimens. To maintain blinding, all patients were connected to an infusion pump. Group I patients could self-administer boluses of 4.8 mg propofol and 125 microg alfentanil without restriction. Group II patients received a continuous infusion with 0.048 mg/kg propofol and 0.12 microg/kg alfentanil per minute. Group III patients received intravenous premedication with 0.035 mg/kg midazolam and 0.35 mg/kg meperidine. RESULTS: There were no differences between the groups with respect to pain (visual analogue scale) and procedure time. Patient-controlled analgesia and sedation with propofol/alfentanil (group I) resulted in less of an increase in the transcutaneous partial pressure of carbon dioxide (p = 0.0004) during colonoscopy and less of a decrease in mean arterial blood pressure (p = 0.0021) during recovery, as well as more complete recovery (p = 0.0019) after 45 minutes compared with conventional administration of midazolam/meperidine. Furthermore, patient-controlled analgesia and sedation yielded a higher degree of patient satisfaction than continuous infusion of propofol/alfentanil (p = 0.0033) or nurse-administered midazolam/meperidine (p = 0.0094). CONCLUSIONS: Patient-controlled administration of propofol and alfentanil for colonoscopy may provide a better margin of safety than conventional administration of midazolam and meperidine and results in a higher level of patient satisfaction and shorter recovery.     

Refractory anemia with ring sideroblasts and RARS with thrombocytosis

Disease Overview : Ring sideroblasts (RS) are erythroid precursors with abnormal perinuclear mitochondrial iron accumulation. Two myeloid neoplasms defined by the presence of RS, include refractory anemia with ring sideroblasts (RARS) and RARS with thrombocytosis (RARS‐T). Diagnosis : RARS is a lower risk myelodysplastic syndrome (MDS) with dysplasia limited to the erythroid lineage, <5% bone marrow (BM) blasts and ≥15% BM RS. RARS‐T is a provisional entity in the MDS/MPN (myeloproliferative neoplasm) overlap syndromes, with diagnostic features of RARS, along with a platelet count ≥450 × 10(9)/L and large atypical megakaryocytes similar to those observed in BCR‐ABL1 negative MPN. Mutations and Karyotype : Mutations in the SF3B1 gene are seen in ≥80% of patients with RARS and RARS‐T, and strongly correlate with the presence of BM RS; RARS‐T patients have additional mutations such as, JAK2V617F (～60%), MPL (<5%), and CALR (<5%). Cytogenetic abnormalities are uncommon in both RARS and RARS‐T. Risk stratification : Most patients with RARS are stratified into lower risk groups by the International Prognostic Scoring System (IPSS) for MDS and the revised IPSS. Disease outcome in RARS‐T is better than that of RARS, but worse than that of essential thrombocytosis. Both RARS and RARS‐T have a low risk of leukemic transformation. Treatment : Anemia and iron overload are complications in both diseases and are managed similar to lower risk MDS. Aspirin therapy is reasonable in RARS‐T, especially in the presence of JAK2V617F, but the value of platelet‐lowering drugs is uncertain. Case reports of RARS‐T therapy with lenalidomide warrant additional studies. Am. J. Hematol. 90:550–559, 2015. © 2015 Wiley Periodicals, Inc.     

Experience with the treatment of patients with hypertrophic cardiomyopathy with obsidan and isoptin

Forty-five patients with hypertrophic cardiomyopathy were examined clinically and echocardiographically. The results of their treatment with obsidan and isoptin in relation to various types of central hemodynamic disorders are presented. The data have been obtained making it possible to treat patients differentially with regard to the form of the disease. The treatment of this category of patients requires the echocardiographic monitoring of the parameters of the central hemodynamics and myocardial contractility.