原发性局限性膀胱淀粉样变性2例临床病理分析

原发性局限性淀粉样变性是以多种因素诱发的特异性糖蛋白复合体在局部组织或器官细胞外沉积为主要病理特征的代谢性疾病,发生于膀胱者罕见。其临床表现及影像学改变和膀胱镜特点极似膀胱肿瘤。我科自1978—2009年收治2例原发性局限性膀胱淀粉样变性患者。现结合文献报告如下。     

肾淀粉样变性10例临床病理分析

目的探讨肾淀粉样变性肾脏损害的临床特征,提高对淀粉样变性肾脏损害的认识。方法回顾性分析10例肾淀粉样变性的临床及病理资料。结果 10例肾淀粉样变性年龄(61.0±9.9)岁,临床主要表现为浮肿、大量蛋白尿,其中4例血压降低。尿κ、λ链升高,尿本周蛋白均阴性,骨髓细胞学检查排除多发性骨髓瘤。肾脏病理刚果红染色呈阳性,其中AL型肾淀粉样变性9例,AA型肾淀粉样变性1例。结论淀粉样变性肾脏易受累,临床表现多样,确诊主要依靠病理活检刚果红染色。     

原发性弥漫性支气管淀粉样变性一例报告

患者 女,49岁。因咳嗽、气短3年,加重2个月,于1997年3月20日以“喘息性支气管炎”收入院。既往从事养鸡工作5年,不吸烟,否认结核、肿瘤及慢性咳嗽史。查体:体温、脉搏、血压均在正常范围,无紫绀,浅表淋巴结不大。轻度三凹征,双肺叩诊音清,可闻及广泛性散在的哮鸣音。心率80次/分,律齐。双下肢无水肿,轻度杵状指。实验室检查:血沉、血谷丙转氨酶、白蛋白和球蛋白定量、血IgG、IgA、IgM均在正常范围。痰瘤细胞、抗酸杆菌各查6次均阴性。肺功能检查示:轻度限制性通气障碍,重度阻塞性通气障碍。胸部X线检查示:双肺广泛性散在的斑片状浸润影,以中下肺为重。纤     

肾淀粉样变性临床病理学分析

目的探讨肾脏淀粉样变性的临床病理学特点。方法对15例经病理诊断为肾淀粉样变性患者的临床资料、免疫荧光、组织化学及光镜检验结果进行回顾性分析和研究。结果疾病初期通常表现为肾病综合征,随着疾病的发展,临床可出现肾功能不全和肾衰竭。原发性肾淀粉样变性11例,继发性肾淀粉样变性4例。光镜下淀粉样物质可沉积于肾小球系膜、基底膜、血管壁及肾间质,这些受累的区域无细胞或细胞过少。随着病程的发展,肾小球可以完全被淀粉样物质替代(3/15)。淀粉样沉积物刚果红染色阳性(15/15)。结论肾脏淀粉样变性的病因是多方面的,其诊断依赖于组织病理学和组织化学,并须结合其临床资料。     

临床药师对原发性淀粉样变性患者的药学监护

目的临床药师对1例原发性淀粉样变性患者进行药学监护,发挥临床药师在治疗团队中的作用。方法通过对患者进行全程的药学监护,监控相关指标及不良反应并预防不良反应,及时给出建议,调整治疗方案,评价治疗效果。结果患者化疗过程中未出现严重的不良反应,顺利完成本周期化疗过程,不但提高了药物治疗的安全性和有效性,而且提高了患者的依从性。结论通过参与临床实践,临床药师为合理用药提供了帮助,提高用药安全性,使患者获得优良的药学服务。     

淀粉样变性的临床表现

淀粉样变性可见于多种疾病,以不同组织内有淀粉样物质沉积,导致器官功能损害为共同病理表现。沉积可以是局部的或全身的,病程可呈良性或恶性。在很多情况下,淀粉样物质沉积常是潜在疾病的部分表现,有关疾病可能是炎症、遗传病或肿瘤,其症状取决于淀粉样物质沉积部位和沉积量。     

原发性肝淀粉样变性鉴别诊断1例

淀粉样变性足多种原因引起的一组临床综合征,其特点是淀粉样物质在组织中沉积,引起器官功能衰竭。主要累及肾、心、肝、脾、胃肠道、皮肤等组织。淀粉样物质侵及肝脏,浸润于肝细胞之间或沉积于网状纤维支架时称为肝淀粉样变性。肝淀粉样变性的症状均为非特异性。如体质量减轻、乏力、肝大、轻度黄疸。该病临床少见,     

淀粉样变性心肌病一例的诊断分析

淀粉样变性心肌病为一种限制型心肌病,临床少见,预后不良.笔者将1例表现为多脏器损害,经病理检查确诊的淀粉样变性心肌病病例报告如下:     

喉淀粉样变性四例报告

喉淀粉样变性(Amgloiolsis)是一种罕见病,据统计约占喉部良性肿瘤的0.68%～1%。本院1982～1989年经病理证实共4例,其中3例术前曾误诊为喉癌。     

淀粉样变性心肌病一例的诊断分析

淀粉样变性心肌病为一种限制型心肌病,临床少见,预后不良。笔者将1例表现为多脏器损害,经病理检查确诊的淀粉样变性心肌病病例报告如下: 1 临床资料 患者男性,66岁,因反复胸闷胸痛气促10年,加重伴双下肢浮肿7周入院。有高血压史十余年。查体:BP 140/100mmHg     

心脏淀粉样变性患者心功能变化的分析和研究

目的：应用超声心动图脉冲多普勒、组织多普勒（DTI）及彩色M型血流传播速率（Vp）技术评价心脏淀粉样变性患者的心脏功能.方法：对经心内膜活检证实并行超声心动图检查的74例心脏淀粉样变性患者的心脏功能变化进行分析,并根据心功能情况分收缩功能正常组和收缩功能减退组.结果：收缩功能减退组较收缩功能正常组二尖瓣E/A比值增高、DT缩短、Vp降低,并显示舒张期血流传播速度缓慢;DTI示E＇及A＇降低,反映充盈压升高的E/Vp和E/E＇比值亦明显升高.所有患者均存在舒张功能障碍.结论：心脏淀粉样变性患者早期即出现舒张功能障碍,而收缩功能尚可维持正常.晚期则舒张和收缩功能均下降,并呈进行性发展.DTI和Vp技术可于早期对舒张功能障碍进行评价.     

国际疾病分类(ICD-10)与临床疾病诊断存在问题的探析

目的：探讨临床医生对疾病诊断更标准化、规范化，适应国际疾病分类(ICD-10)在临床的应用。方法：分析我院2002年以来，应用国际疾病分类过程中与临床疾病诊断之间存在的问题。结果：存在主要疾病诊断选择错误，滥用“术后”作第一诊断，没有写“组织类型”肿瘤的诊断，随意用症状和体征代替诊断，没有写外部原因中毒或损伤的疾病的问题。并对存在问题提出几点改进建议。结论：临床疾病诊断的标准化和规范化，可增加编码正确率，提高国际疾病分类水平。     

缬沙坦对高血压及心率变异性影响的研究

目的:研究缬沙坦对高血压心率变异性的影响。方法:选择80例原发性高血压患者服用缬沙坦,分别在治疗前与治疗14周后进行动态心电图(DCG)及心率变异性(HRV)检查,并与对照组(40例)进行比较。结果:治疗后连续R-R间期标准差(SDNN),相邻R-R均方差(RMSSD),相邻R-R间期大于50ms百分数(PNN50)、高频功率(HF)值均明显上升;低频功率与高频功率比值(LF/HF)显著降低(P<0.01),结论:原发性高血压患者存在HRV下降,而缬沙坦在降压的同时,可提高HRV,改善自主神经功能失调。     

The effect of tafamidis on the QTc interval in healthy subjects

Aims

The transthyretin (TTR) stabilizer, tafamidis, has demonstrated efficacy and safety in the treatment of TTR familial amyloid polyneuropathy (20 mg day⁻¹). Tafamidis use in TTR cardiomyopathy led to the study of the potential effect of tafamidis on the QT_c interval in healthy subjects.

Methods

This randomized, three treatment, three period, six sequence crossover study with placebo, a positive control (moxifloxacin 400 mg) and tafamidis (400 mg, to achieve a supra-therapeutic C_max of ∽20 µg ml⁻¹) was conducted in healthy volunteers at three clinical research units. Oral dosing in each of the three treatment periods was separated by a washout period of  ≥ 14 days. Serial triplicate 12-lead electrocardiograms were performed. QT_c intervals were derived using the Fridericia correction method. Safety and tolerability were assessed by physical examination, vital signs measurement, laboratory analyses and monitoring of adverse events (AEs).

Results

A total of 42 subjects completed the study. The upper limit of the two-sided 90% confidence intervals (CIs) for the difference in baseline-adjusted QT_cF between tafamidis 400 mg and placebo was <10 ms (non-inferiority criterion) for all time points. The lower limit of the two-sided 90% CI between moxifloxacin 400 mg and placebo exceeded 5 ms at the pre-specified moxifloxacin t_max of 3 h post-dose, confirming assay sensitivity. C_max and AUC(0,24 h) for tafamidis were 20.36 µg ml⁻¹ and 305.4 µg ml⁻¹ h, respectively. There were no serious/severe AEs or treatment discontinuations due to AEs.

Conclusions

This thorough QT_c study suggests that a supra-therapeutic single 400 mg oral dose of tafamidis does not prolong the QT_c interval and is well-tolerated in healthy volunteers.     

青岛市中小学衔接过程中学生心理行为问题调查

目的:调查研究中小学衔接期学生心理行为问题.方法:对200名六年级学生评定焦虑自评量表(SAS)、儿童行为量表(父母用),升入初中一年级后再次评定,比较小学升初中后学生心理行为的变化.结果:小学六年级焦虑检出率为3.5%,初一焦虑检出率为3.0%;六年级与初一两时间段学生焦虑检出率总体无统计学差异(x2=0.08,P>...     

原发性高血压与内皮素升高关系的研究

目的:研究原发性高血压患者血内皮素浓度的变化,探讨高血压与内皮素升高的关系.方法:用放射免疫法测定40例原发性高血压血内皮素含量,并与40例健康人(对照组)进行对照分析.结果:高血压组内皮素升高,与对照组比较差异具有非常显著性意义(P＜0.001),并与血压升高程度呈正相关,高血压组Ⅱ、Ⅲ级与Ⅰ级比较差异具有非常显著性的意义(P＜0.01),Ⅲ级与Ⅱ级比较差异具有非常显著性的意义(P＜0.01).原发性高血压并发左心室肥厚(LVH)、心力衰竭组与单纯高血压组内皮素含量比较具有非常显著性的意义(P＜0.01,P＜0.001);并发心力衰竭组与并发LVH组内皮素含量比较差异具有非常显著性的意义(P＜0.01).结论:内皮素是高血压病理发展过程中的重要参与因素,可作为反映高血压病变程度和动态变化的一项指标,有一定的参考价值.     

Primary and secondary nonresponse to infliximab: mechanisms and countermeasures

Introduction: Primary and secondary non-response to infliximab are common in patients with inflammatory bowel disease and remain a management challenge in clinical practice.

Areas covered: This article describes the epidemiology, mechanisms and risk factors for primary and secondary nonresponse to infliximab in patients with inflammatory bowel disease. Data on proactive and reactive therapeutic drug monitoring are examined in this review. An algorithm for evaluation and management of non-response to infliximab is provided. Preventative measures are also discussed. Relevant articles were identified after a literature search using PubMed. Search terms included ‘infliximab’, ‘loss of response’, ‘immunogenicity’, and ‘drug monitoring’. References of identified articles were also reviewed to identify additional references.

Expert opinion: A common cause for primary and secondary non-response include inadequate dosing of infliximab; inadequate dosing can be identified through assessment of drug and anti-drug antibody levels. Therapeutic drug monitoring should be done in patients losing response to infliximab. Use of drug monitoring proactively is still under debate.     

The International Prospective Primary Prevention Study in Hypertension (IPPPSH): Objectives and methods

Summary The International Prospective Primary Prevention Study in Hypertension (IPPPSH), an international multicentre randomised double-blind trial, compares antihypertensive regimens containing a beta-blocker (oxprenolol) with regimens not containing a beta-blocker. The protocol was developed and the study started in 1977. 6,357 patients fulfilled the inclusion criteria and were enrolled by the end of 1980. Patients have been treated in 6 countries for 3 to 5 years by 288 general practitioner and hospital out-patient centres. Follow-up was finished at the end of 1983 when 25,651 years of patient treatment had been completed. At entry patients had a diastolic pressure of 100–125 mmHg with no clinical evidence of cerebrovascular or ischaemic heart disease (W.H.O. Stages I and II hypertension). The study was designed to examine three main objectives: any influence of oxprenolol on the incidence of myocardial infarction and sudden death compared with antihypertensive therapy not containing a beta-blocker; the influence of cerebrovascular accidents in the same two groups; a comparison of the antihypertensive effectiveness, and the subjective and objective tolerability of the regimens. This paper summarises the objectives and methodology, and examines randomisation between the two treatment groups at entry into the IPPPSH.This paper is dedicated to the memory of Professor James Crooks and Professor Franz Gross, two members of the IPPPSH Review Committee whose wisdom and encouragement did so much to help all those involved in the development of this clinical trial     

从中医“治未病”理论探讨心血管疾病的初级预防

目的:中医"治未病"理论指导下探讨心血管疾病的初级预防.方法:选取2010年9月~2013年9月磨山社区无心血管相关疾病及危险因素的320例志愿者,随机分成对照组与干预组。对照组不做任何的干预和指导,干预组按照"合理膳食、适当运动、戒烟限酒、心理平衡同时服用酵素及经络穴位按摩"的健康生活方式给予健康教育指导,预防危险因素的发生。观察两组的临床效果,并进行统计学分析。结果:通过对干预组160例志愿者进行健康教育指导,吸烟发生率为6.3%、血压异常率为1.9%、胆固醇异常率为1.3%、空腹血糖异常率为1.3%、MBI异常率为1.3%,危险因素好发率为8.1%,较对照组危险因素好发率45.6%明显减少,两组对比差异具有显著性。结论:采用健康的生活方式可以明显减少心血管病危险因素的发生,这与防病于未然,治病于初始阶段的中医"治未病"理论精髓相一致,值得基层医疗机构推广应用,同时也为心血管病的临床预防及致力于中医"治未病"研究提供了有价值的参考。     

2型糖尿病与牙周病相关性的初步研究

目的探讨2型糖尿病和牙周病的关系以及牙周治疗和降糖治疗对两种疾病的影响。方法①选择健康人群(20名)、单纯性2型糖尿病患者(20名)和2型糖尿病合并重度牙周病患者(40名),检测其血清C反应蛋白水平、糖化血红蛋白和血糖水平;②对2型糖尿病合并重度牙周病患者进行分组试验性治疗:A组为牙周基础治疗+常规降糖治疗组,B组为强化降血糖组。各治疗观察3个月,观察其疗效。结果①2型糖尿病合并重度牙周病的血清C反应蛋白、糖化血红蛋白、空腹血糖都明显高于单纯性2型糖尿病组及健康对照组(P<0.01)。而单纯性2型糖尿病组高于健康对照组(P<0.01)。②牙周病基础+常规降糖治疗组治疗后牙周袋深度及龈沟出血指数均明显减少(P<0.001,P<0.01),C反应蛋白也明显降低(P<0.001)。同时糖化血红蛋白随之降低(P<0.001)。结论 2糖尿病与牙周病存在相互影响的关系,牙周病基础+常规降糖治疗可有效地改善牙周健康和控制糖尿病。