Left Main Coronary Atresia in a Child with Absent Pulmonary Valve Syndrome: Early Entrapment of an Innocent Bystander

Atresia of the left main coronary artery is a rare anomaly that, if left untreated, has an unfavorable outcome. We hereby report left main coronary artery atresia in a child with tetralogy of Fallot with absent pulmonary valve and discuss the possible developmental basis of the association.     

Evaluation of von Willebrand factor in COPD patients

OBJECTIVE:

To compare the absolute serum von Willebrand factor (vWF) levels and relative serum vWF activity in patients with clinically stable COPD, smokers without airway obstruction, and healthy never-smokers.

METHODS:

The study included 57 subjects, in three groups: COPD (n = 36); smoker (n = 12); and control (n = 9). During the selection phase, all participants underwent chest X-rays, spirometry, and blood testing. Absolute serum vWF levels and relative serum vWF activity were obtained by turbidimetry and ELISA, respectively. The modified Medical Research Council scale (cut-off score = 2) was used in order to classify COPD patients as symptomatic or mildly symptomatic/asymptomatic.

RESULTS:

Absolute vWF levels were significantly lower in the control group than in the smoker and COPD groups: 989 ± 436 pg/mL vs. 2,220 ± 746 pg/mL (p < 0.001) and 1,865 ± 592 pg/mL (p < 0.01). Relative serum vWF activity was significantly higher in the COPD group than in the smoker group (136.7 ± 46.0% vs. 92.8 ± 34.0%; p < 0.05), as well as being significantly higher in the symptomatic COPD subgroup than in the mildly symptomatic/asymptomatic COPD subgroup (154 ± 48% vs. 119 ± 8%; p < 0.05). In all three groups, there was a negative correlation between FEV₁ (% of predicted) and relative serum vWF activity (r² = −0.13; p = 0.009).

CONCLUSIONS:

Our results suggest that increases in vWF levels and activity contribute to the persistence of systemic inflammation, as well as increasing cardiovascular risk, in COPD patients.     

Assessment of inhaler techniques employed by patients with respiratory diseases in southern Brazil: a population-based study

OBJECTIVE:

To identify incorrect inhaler techniques employed by patients with respiratory diseases in southern Brazil and to profile the individuals who make such errors.

METHODS:

This was a population-based, cross-sectional study involving subjects ≥ 10 years of age using metered dose inhalers (MDIs) or dry powder inhalers (DPIs) in 1,722 households in the city of Pelotas, Brazil.

RESULTS:

We included 110 subjects, who collectively used 94 MDIs and 49 DPIs. The most common errors in the use of MDIs and DPIs were not exhaling prior to inhalation (66% and 47%, respectively), not performing a breath-hold after inhalation (29% and 25%), and not shaking the MDI prior to use (21%). Individuals ≥ 60 years of age more often made such errors. Among the demonstrations of the use of MDIs and DPIs, at least one error was made in 72% and 51%, respectively. Overall, there were errors made in all steps in 11% of the demonstrations, whereas there were no errors made in 13%.Among the individuals who made at least one error, the proportion of those with a low level of education was significantly greater than was that of those with a higher level of education, for MDIs (85% vs. 60%; p = 0.018) and for DPIs (81% vs. 35%; p = 0.010).

CONCLUSIONS:

In this sample, the most common errors in the use of inhalers were not exhaling prior to inhalation, not performing a breath-hold after inhalation, and not shaking the MDI prior to use. Special attention should be given to education regarding inhaler techniques for patients of lower socioeconomic status and with less formal education, as well as for those of advanced age, because those populations are at a greater risk of committing errors in their use of inhalers.     

重症医学科老年心衰患者监测血清降钙素原水平指导抗感染治疗的临床应用价值

目的 探讨血清降钙素原（PCT）水平在老年心衰患者抗感染治疗策略中的价值.方法 选取2013年1月至2014年2月本院住院的老年心衰患者共165例,随机分为常规治疗组（85例）和PCT指导治疗组（80例）.常规治疗组根据患者临床症状和病情变化决定抗菌药物的使用,而PCT指导治疗组根据患者血清PCT浓度决定抗菌药物的使用.观察两组患者的临床有效率、住院病死率、住院时间、住院总费用、抗菌药物疗程、抗菌药物费用、二重感染率以及半年随访期间肺部感染的发生率.结果 常规治疗组和PCT指导治疗组在临床有效率（81.2％ vs 82.5％）、住院病死率（5.9％vs 5.0％）、半年随访期间肺部感染的发生率（32.9％ vs 31.3％）等方面差异均无统计学意义（P值均＞0.05）;而PCT治疗组患者在住院总费用（5 854元vs 9 652元）、抗菌药物疗程（10.2 d vs 16.7 d）、抗菌药物费用（2 543元vs 3 646元）及二重感染率（2.5％vs 7.1％）等方面均低于常规治疗组（P值均＜0.05）.结论 老年心衰患者在PCT指导下使用抗菌药物,能减少抗菌药物使用量,降低住院费用,降低二重感染的发生率.     

人肺腺癌细胞系A549线粒体差异蛋白质组学研究

目的 研究人肺腺癌细胞系A549与人正常支气管上皮细胞系16HBE线粒体蛋白质组的差异表达.方法 传代培养细胞系A549及16HBE,用线粒体提取试剂盒获取细胞线粒体蛋白质,进行双向凝胶电泳,运用液相色谱串联质谱分析技术筛选出A549和16HBE细胞系线粒体间表达水平显著差异的蛋白,所得结果通过Data Analysis软件标峰,用MASCOT进行结果搜索和数据分析.结果 双向电泳结果显示A549、16HBE细胞系线粒体存在差异的蛋白质点共41个,3倍以上差异的16个,A549细胞系中表达上调的15个,表达下调的26个,其中3倍以上差异表达上调的7个,表达下调的9个.运用液相色谱串联质谱技术鉴定出A549细胞系线粒体表达上调的蛋白质2个:AAA+ ATP酶家族结构域蛋白3B、tRNA鸟嘌呤糖基转移酶,表达下调的蛋白质7个:热休克蛋白75、复合物Ⅲ亚基1、复合物Ⅲ亚基2、鸟氨酸氨基转移酶、异柠檬酸脱氢酶亚基α、SLP-2、抗增殖蛋白.结论 应用亚细胞蛋白质组学方法,鉴定出肺腺癌细胞系线粒体差异表达蛋白,为阐明肺腺癌发生的分子机制、筛选早期诊断标志物提供了有益的线索.     

肺动脉高压患者肺功能变化特点研究

目的探讨肺动脉高压患者进行常规肺功能（PFT）及脉冲震荡肺功能（IOS）测定的临床意义。方法对51例经右心导管检查确诊的特发性肺动脉高压（IPAH）患者及慢性血栓栓塞性肺动脉高压（cTEPH）患者和20名健康者依次进行脉冲震荡肺功能及常规肺功能检测,分析两组人群肺功能各项指标的变化特点。结果IPAH患者一氧化碳弥散量占预计值百分比（D。CO％pred）较CTEPH患者明显下降（76．60±19．98和93．62±18．77,P〈0．01）,肺动脉高压组较正常对照组FEVlOpred、FVC％pred、FEV1／FVC、最大呼气中期流量占预计值百分比（MMEF％pred）、最大自主通气量占预计值百分比（MVV％pred）、DLCO％pred等指标明显降低（分别为82．17±16．19和98．38±7．95,88．13±16．64和97．93±9.7,78．66±7．47和84。47±4．58,57．34±16．14和81.18±17．82,83．08±30．41和108．21±27．04,83．61±21．07和109．86土15．73,P〈O．01）,气道总阻力占预计值百分比（Rtot％pred）、Z5占预计值百分比（Z50pred）、R5占预计值百分比（R5Yopred）、R5-R20、X5、AX、Fres等指标明显升高（分别为131．16±46．22和90．90士28．99,146．98±59．22和104．21±19．93,139．57±53．68和100．10±8．79,1．68±1．52和0．45±0．25,1．37±1．19和0．89±0．20,12．78±15．63和5．73±2．49,17．22±5．94和10．33±1．87,P〈O．01）。WHO肺动脉高压功能分级（WH0FC）UI级患者较Ⅱ级患者R5-R20、X5、AX、Fres等指标明显升高（分别为2．28±1．88和1.11±0．73,1．77±1．55和0．98±0．45,18．27±20．47和7．50±5．20,18．92±6．50和15．58±4．93,P〈0．05）。结论IPAH患者较cTEPH患者平均确诊年龄更低。但确诊时血流动力学指标受损更严重,肺功能指标除弥散功能IPAH患者更差外,其他肺功能改变两类患者基本类似。肺动脉高压患者存在轻度限制性及阻塞性通气?     

泛素-蛋白酶体系统与呼吸系统疾病

蛋白是细胞功能的主要执行者,蛋白的质量和数量与细胞功能和疾病有着密切的联系。蛋白的降解系统控制着蛋白的数量,泛素一蛋白酶体系统是细胞内蛋白的主要降解系统,负责细胞内大部分蛋白的降解。对于泛素一蛋白酶体与心血管、肿瘤和神经系统的关系目前研究的比较多,一些新的I艋床药物也应运到临床为患者带来福音。而该系统与呼吸系统疾病关系的研究并不多,了解泛素一蛋白酶体系统在呼吸系统疾病中的作用对于呼吸系统疾病的防治有重要的意义。     

血管活性肠肽在慢性阻塞性肺病相关肺动脉高压中的研究进展

血管活性肠肽（VIP）是心肺系统最丰富的肽类物质之一,具有强大的抗炎、肺血管与气道舒张作用,以及免疫调节、调节气道分泌等广泛的生物学作用,可减缓COPD相关肺动脉高压（PH）的进展。然而,由于其血浆半衰期较短,给药途径困难,故临床有待进一步研究。本文就现阶段VIP对COPD相关PH治疗的研究进展作一综述。