慢性阻塞性肺疾病合并肺曲菌病一例及文献复习

目的:提高对慢性阻塞性肺疾病合并侵袭性肺曲菌病(COPD合并IPA)临床特点、诊断及治疗的认识.方法:回顾性分析2011年4月收治的一例COPD合并IPA患者的临床资料及诊治经过,并复习相关文献.结果:男患,“咳嗽、咳痰30余年,气短3年,加重1月余”入院,肺部CT示双肺多发结节影、空洞影,经抗炎、抗念珠菌治疗无效,CT下肺结节病灶活检病理示肺曲菌.抗曲菌治疗后症状好转、肺部影像明显吸收.结论:COPD合并IPA正逐渐引起重视,临床特征无明显特异性,肺部影像以结节影、空洞影多见,早期常规治疗无效时应积极抗曲菌治疗,可明显改善症状,降低死亡率,病理活检是确诊的依据.     

Focus: Rare Disease: Pulmonary Alveolar Microlithiasis – A Review

Pulmonary Alveolar Microlithiasis (PAM) is a rare genetic disorder causing widespread deposition of calcium-phosphate crystals in the alveolar space. A hallmark of the disease is the discrepancy between perceived symptoms upon diagnosis compared with the extensive, sandstorm-like appearance of the microliths on chest X-ray or HRCT. Caused by a defective sodium-dependent phosphate transport protein due to loss-of-function variants of the SLC34A2 gene, PAM is an autosomal recessive transmitted disorder, and as such has a high correlation to consanguinity. The most common variants of the SLC34A2 gene are single nucleotide biallelic changes, but larger deletions are described. Initial suspicion of PAM on radiological examination should be followed by genetic testing to verify the diagnosis and identify the disease-causing variant. When not available, the diagnosis can be made by means of invasive techniques, such as transbronchial forceps or cryobiopsy, or a surgical lung biopsy. In families with a history of PAM, genetic counseling should be offered, as well as preimplantation/prenatal testing if necessary. As of writing this review, no definitive treatment exists, and PAM may in some cases progress to severe pulmonary disease with respiratory failure and potential death. Patients with PAM should be offered preventative and symptomatic treatments such as vaccinations and oxygen therapy when needed. In some cases, lung transplantation may be required.     

Focus: Rare Disease: Cystinuria: Review of a Life-long and Frustrating Disease

Cystinuria, accounting for about 1-2% of kidney stones in adults, carries significant morbidity beginning at a young age [1]. Cystine stone formers have more stone events compared to other stone formers, as well as more surgical interventions, potentially contributing to faster progression to chronic kidney disease (CKD), and end-stage kidney disease (ESKD) [2]. Successful medical therapy for cystine stone formers may be limited by adherence to the extensive lifestyle changes and the adverse side effect profiles of some interventions, leading to decreased quality of life for these patients relative to other stone formers.     

慢性阻塞性肺疾病发病机制的研究进展

慢性阻塞性肺疾病(chronic obstructive pulmonary disease, COPD)是呼吸系统中的常见病和多发病,其发病率和致死率居高不下。根据WHO调查数据显示,预计到2020年, COPD将成为全球第三大致死病因。COPD的形成与发展涉及众多因素,包括遗传因素和环境因素等。COPD的发病机制至今未明,氧化应激、炎症机制、蛋白酶/抗蛋白酶失衡以及细胞凋亡与之密切相关。另外,自身免疫反应、微生物组的变化以及无效的受损修复在COPD的发病机制中同样具有关键作用。因此,深入了解并研究COPD的发病机制对预防与治疗该疾病具有重要意义。     

慢性阻塞性肺疾病急性加重对患者生活质量的影响

目的:探讨慢性阻塞性肺疾病急性加重对患者生活质量的影响。方法:以中国中医科学院广安门医院呼吸科长期慢病管理计划中确诊的慢性阻塞性肺疾病患者为研究对象,记录其在2013年1月至12月间发生的慢性阻塞性肺疾病急性加重事件的情况,并采用面对面访谈式问卷调查方法,在2013年12月对患者进行肺疾病评估测试、焦虑自评量表评分及抑郁自评量表评分,以探讨急性加重发生对患者健康的影响。结果:504例慢性阻塞性肺疾病患者中,过去1年间220例(43.7%)有急性加重发作,其CAT评分、SAS评分、SDS评分均高于未发生急性加重患者,差异均有统计学意义(P0.05)。急性加重发作患者中,66.4%的生活质量均受到中等程度及以上影响,高于无急性加重发生组(52.1%),差异有统计学意义(P0.05)。此外,有急性加重发作的患者合并焦虑和/或抑郁状态的患病率也高于无急性加重发作的患者,差异有统计学意义(P0.05)。结论:慢性阻塞性肺疾病急性加重对患者生活质量、精神状态均可能产生不利影响。     

聚焦慢性阻塞性肺病治疗药物

慢性阻塞性肺病(COPD) 是肺部进行性疾病,需长期治疗。预计到2020 年,每年COPD 将会导致全球600 多万人死亡。尽管对新型药物开发投资不断增加,但该领域的治疗仍以缓解症状的支气管扩张吸入剂为主,而不是治愈性疗法,故应对COPD 的创新药的研发仍困难重重。与乳腺癌等疾病领域相比,呼吸系统药物的研发成功率不到50%,但每种获批药物的研发成本却翻倍。因此,制药公司致力于药品生命周期的管理,在当前治疗的基础上进一步改进。其策略包括不同种类药物联用、开发新配方和各种剂量剂型,改善疗效,方便给药。其中以长效β 受体激动剂 (LABA)/ 长效毒蕈碱拮抗剂(LAMA) 的定量复方药的需求增长最多。领导市场的GSK 公司的LABA 沙美特罗和吸入型皮质类固醇 (ICS) 氟替卡松的复方药Advair,就是应用以上方法的典型。虽已出现潜在新型靶标,但这些药物还处于早期开发阶段。吸入型LABA/ICS 定量复方药短期内仍是 COPD 治疗领域的主力军。     

细辛脑联合多索茶碱治疗慢性阻塞性肺病疗效观察

目的：观察细辛脑联合多索茶碱治疗慢性阻塞性肺疾病急性加重期（AECOPD）的临床疗效。方法：将164例AECOPD患者随机分为观察组（82例）和对照组（82例）,对照组予低流量吸氧、抗炎、抗感染、纠正水电解质紊乱等综合治疗,观察组在此基础上加用注射用细辛脑、多索茶碱静脉治疗。观察两组治疗前后症状、体征改善情况及血气分析变化。结果：观察组与对照组临床总有效率分别为93．9％、81．7％（P〈0．05）,观察组在发热、咳嗽咳痰、喘息症状消失时间,以及肺部罗音减少50％以上时间均显著短于对照组（P〈0．01）,在FEV1、FEV1／FVC、PEF、PaO2、PaCO2等指标也显著优于对照组（P〈0．01）。结论：在常规治疗基础上加用细辛脑、多索茶碱可以有效提高AECOPD患者的临床疗效。     

细辛脑联合多索茶碱治疗慢性阻塞性肺病疗效观察

目的:观察细辛脑联合多索茶碱治疗慢性阻塞性肺疾病急性加重期（AECOPD）的临床疗效。方法:将164例AECOPD患者随机分为观察组（82例）和对照组（82例）,对照组予低流量吸氧、抗炎、抗感染、纠正水电解质紊乱等综合治疗,观察组在此基础上加用注射用细辛脑、多索茶碱静脉治疗。观察两组治疗前后症状、体征改善情况及血气分析变化。结果:观察组与对照组临床总有效率分别为93.9%、81.7%（P<0.05）,观察组在发热、咳嗽咳痰、喘息症状消失时间,以及肺部罗音减少50%以上时间均显著短于对照组（P<0.01）,在FEV1、FEV1/FVC、PEF、PaO₂、PaCO₂等指标也显著优于对照组（P<0.01）。结论:在常规治疗基础上加用细辛脑、多索茶碱可以有效提高AECOPD患者的临床疗效。     

Combined Heart Rate Variability and Pulse Oximetry Biofeedback for Chronic Obstructive Pulmonary Disease: Preliminary Findings

The purpose of this study was to examine the feasibility of an intervention that included heart rate variability (HRV) biofeedback and walking with pulse oximetry feedback to improve functioning and quality of life for patients with chronic obstructive pulmonary disease (COPD). Twenty patients with COPD participated in 5 weekly sessions of HRV biofeedback and 4 weekly sessions of walking practice with oximetry feedback, with instructions for daily home practice. Primary outcomes measures were the distance walked in 6 min (6MWD) and overall quality of life, as measured by the St. George's Respiratory Questionnaire (SGRQ). Secondary outcomes included measures of self-efficacy, self-reported disability, anxiety, depression, dyspnea before and after the 6MWD, and HRV at the frequency of respiration during spontaneous and paced breathing. After 10 weeks of training, participants showed statistically and clinically significant improvements in 6MWD and quality of life. Significant changes were also seen in self-efficacy, disability, dyspnea before and after the 6MWD, and HRV amplitude during spontaneous breathing. We conclude that our intervention is feasible for patients with COPD and that further research using a randomized controlled design is warranted.     

Klotho Reduction in Alveolar Macrophages Contributes to Cigarette Smoke Extract-induced Inflammation in Chronic Obstructive Pulmonary Disease

Abnormal inflammation and accelerated decline in lung function occur in patients with chronic obstructive pulmonary disease (COPD). Klotho, an anti-aging protein, has an anti-inflammatory function. However, the role of Klotho has never been investigated in COPD. The aim of this study is to investigate the possible role of Klotho by alveolar macrophages in airway inflammation in COPD. Klotho levels were assessed in the lung samples and peripheral blood mononuclear cells of non-smokers, smokers, and patients with COPD. The regulation of Klotho expression by cigarette smoke extract (CSE) was studied in vitro, and small interfering RNA (siRNA) and recombinant Klotho were employed to investigate the role of Klotho on CSE-induced inflammation. Klotho expression was reduced in alveolar macrophages in the lungs and peripheral blood mononuclear cells of COPD patients. CSE decreased Klotho expression and release from MH-S cells. Knockdown of endogenous Klotho augmented the expression of the inflammatory mediators, such as MMP-9, IL-6, and TNF-α, by MH-S cells. Exogenous Klotho inhibited the expression of CSE-induced inflammatory mediators. Furthermore, we showed that Klotho interacts with IκBα of the NF-κB pathway. Dexamethasone treatment increased the expression and release level of Klotho in MH-S cells. Our findings suggest that Klotho plays a role in sustained inflammation of the lungs, which in turn may have therapeutic implications in COPD.     

Asymptomatic Presentation of Chronic Pulmonary Paracoccidioidomycosis: Case Report and Review

Asymptomatic presentations of chronic pulmonary paracoccidiodomycosis have been reported since 1959, usually published in case series of paracoccidiodomycosis. Incidental radiographic findings on chest roentgenogram led to diagnostic evaluation in these cases. An unusual case in a female patient is described, and 24 previously reported cases are reviewed.     

COPD评估测试与BODE指数的相关研究

目的：探讨COPD评估测试（COPD Assessment Test,CAT）中文版在我国慢性阻塞性肺疾病患者生活质量评价中的价值,并探讨其与BORD指数相关性。方法：选择2010年6月至2012年6月在新疆维吾尔自治区人民医院呼吸与危重症医学科就诊的89例COPD患者,在急性期和稳定期分别进行CAT评分及BORD指数评分。将结果进行配对t检验,评价CAT量表对COPD患者病情变化的敏感性,再进行相关性检验,评价其有效性。结果：配对t检验显示CAT评分在稳定期较急性期有明显改善（P〈0.01）,与临床症状、肺功能、呼吸困难指数改善一致,CAT评分分值与BORD指数相关性较好（r=0.541,P〈0.000）。结论：CAT评分是评价我国COPD患者生活质量有效、敏感、可行的方法。     

Unequal Twice-Daily,Sustained-Release Theophylline Dosing in Chronic Obstructive Pulmonary Disease

Three regimens of sustained-release theophylline (SRT), Theostat® were administered to 12 male patients with chronic obstructive pulmonary disease in a randomized cross-over trial. Each 7-day treatment consisted of

treatment A—8 mg/kg at 0700 hr and 4 mg/kg at 1900 hr

treatment B—6 mg/kg at 0700 hr and 6 mg/kg at 1900 hr

treatment C—4 mg/kg at 0700 hr and 8 mg/kg at 1900 hr.

Peak expiratory flow (PEF) was recorded each day at 0700, 1100, 1500, 1900 and 2300 hr and theophylline plasma levels were determined on the 7th day of each treatment sequence. Cosinor analysis of the data revealed significant circadian rhythms in PEF for each treatment: the mesor (24-hr average) was significantly higher with C and acrophases (Φ, peak time of PEF rhythm) were located at 1426 hr for A and 1425 hr for C; a shift of the acrophase to an earlier timing was detected for B (Φ = 0958 hr. These findings suggest that an unequal, twice-daily SRT dosing with the greater amount of drug at night may be beneficial in the treatment of COPD.     

酚妥拉明联合银杏达莫治疗慢性肺源性心脏病疗效观察

目的：观察酚妥拉明联合银杏达莫对慢性肺源性心脏病心衰患者的疗效,为临床治疗提供依据。方法：将90例患者随机分为治疗组和对照组各45例。两组采用常规内科治疗如控制感染、氧疗、强心、利尿等,治疗组在上述治疗基础上加用酚妥拉明20mg＋银杏达莫25mL于10％葡萄糖注射液500mL中静脉滴注,1次／d,疗程均为10d。结果：治疗组的疗效、血气分析、血液流变学、功能的改善优于对照组,差异具有统计学意义（P〈0．05）。结论：酚妥拉明联合银杏达莫对慢性肺源性心脏痛心衰患者具有良好的疗效及安全性,值得临床推广应用。     

慢性阻塞性肺疾病基因工程动物模型研究进展

慢性阻塞性肺疾病(COPD,简称慢阻肺)是我国常见的高发病率和死亡率的慢性气道炎症性疾病,造成沉重社会经济负担。其发病与遗传及环境因素息息相关。动物模型是研究其发病机制、预防、治疗方案并鉴定潜在治疗靶点及生物标志物的重要工具。随着基因工程技术的发展和慢阻肺相关靶点及基因的不断发现,基因修饰动物模型越来越多地用于慢阻肺的研究。通过检索PubMed中已发表论文,分析了慢阻肺相关动物模型的动物种类及造模方法。然后通过文献检索及数据库挖掘等方法,分析了主要的慢阻肺易感基因及在不同物种中的区别。最后总结了慢阻肺基因工程小鼠和大鼠模型的信息和研究进展,供科研和临床人员参考使用,以便更好地开展慢阻肺的发病机制和防治方法的研究。     

酚妥拉明联合银杏达莫治疗慢性肺源性心脏病疗效观察

目的:观察酚妥拉明联合银杏达莫对慢性肺源性心脏病心衰患者的疗效,为临床治疗提供依据。方法:将90例患者随机分为治疗组和对照组各45例。两组采用常规内科治疗如控制感染、氧疗、强心、利尿等,治疗组在上述治疗基础上加用酚妥拉明20mg+银杏达莫25 mL于10%葡萄糖注射液500 mL中静脉滴注,1次/d,疗程均为10 d。结果:治疗组的疗效、血气分析、血液流变学、功能的改善优于对照组,差异具有统计学意义(P<0.05)。结论:酚妥拉明联合银杏达莫对慢性肺源性心脏病心衰患者具有良好的疗效及安全性,值得临床推广应用。     

吸入高浓度氢气对大鼠慢性阻塞性肺病的保护作用

目的:COPD是严重威胁人类健康的慢性呼吸系统疾病,而目前的治疗手段均不理想。氧化应激在COPD发病机制中发挥重要作用,而氢气具有选择性抗氧化特性。因此探讨氢气对COPD是否具有保护作用具有重要的现实意义。方法:采用被动吸烟法建立大鼠COPD模型,建模成功后对模型动物进行氢气吸入治疗。通过动物肺功能检测、肺组织形态学观察评价氢气对COPD是否具有治疗作用。并进一步通过检测血清及肺组织中MDA水平,以及肺组织中炎性因子水平初步探讨其可能的作用机制。结果:持续8周的烟熏造模,使大鼠体重Lee's指数显著降低,而氢气治疗2周后,Lee's指数较模型组有明显改善。动物肺功能检测显示,持续烟熏使大鼠肺气道阻力明显增加,潮气量和肺顺应性明显降低;氢气吸入治疗2周后,与COPD模型组相比,大鼠肺气道阻力、潮气量和肺顺应性均有明显改善。氢气可以明显改善烟熏引起的肺组织病理改变。氢气对烟熏引起血清及肺组织内MDA、TNF-α、IL-1β水平升高具有明显的抑制作用。结论:吸入氢气对烟熏引起的大鼠COPD具有明显的治疗作用。     

麻杏石甘汤加减治疗慢性阻塞性肺疾病急性发作临床观察

目的：观察麻杏石甘汤加减治疗慢性阻塞性肺疾病急性发作（AECOPD）的疗效。方法：将212例AECOPD患者按随机数字表法分为对照组和治疗组各106例,对照组给予西医常规治疗方案,治疗组在对照组基础上联合应用麻杏石甘汤加减治疗,1个疗程（14 d）后对比疗效及中医症候评分、肺通气功能等相关指标。结果：治疗组临床控制率40．57％优于对照组的24．53,治疗组治疗后咳嗽、痰量、喘息、气短等中医症候评分均低于对照组,FEV1％预计值、FEV1／FVC等肺通气功能指标治疗前后差值均高于对照组,差异均有统计学意义（P＜0．05）。结论：麻杏石甘汤加减治疗AECOPD可清热化痰、解毒祛瘀,促进临床症状缓解,改善肺通气功能,提高疗效。     

降低血粘度对慢性肺心病急性加重期疗效的影响

目的探讨血栓通降低血粘度对慢性肺心病急性加重期疗效的影响。方法将113例慢性肺心病急性加重期患者随机分为两组,观察组63例,对照组50例,两组病例均采取常规治疗(包括抗感染、改善通气、持续低流量吸氧、纠正心衰等),观察组同时应用血栓通注射液350mg加入生理盐水250ml中静滴,每日1次,15天为1疗程。两组病例治疗前、后分别检查血常规、血液流变学、血气分析和凝血常规4项。结果治疗前两组血液流变学数据无显著差异(P>0.05),治疗后观察组的血液流变学数据明显下降,组间差异非常显著(P<0.01)。组内比较观察组治疗后PaO2升高非常显著(P<0.01),PaCO2显著下降(P<0.05);对照组治疗后PaO2显著升高(P<0.05),PaCO2显著下降(P<0.05)。结论血栓通在降低血粘度,改善血液流变学的同时,使慢性肺心病急性加重期患者的低氧血症和高碳酸血症也得到显著纠正。     

慢性肺心病急性发作伴多脏器衰竭的临床护理效应

目的:探讨慢性肺心病急性发作期的护理措施及其临床效应,提高对慢性肺心病急性发作期护理能力,减轻病人痛苦,改善生存质量,延长病人的生命。方法:回顾性分析对36例慢性肺心病急性发作伴多脏器功能衰竭患者进行观察护理,统计对照,制定护理措施,总结护理经验等临床资料。结果:36例病人中急性感染控制,心肺功能明显改善者30例,占83.3%;死亡4例,占16.7%;死亡者中脑、肾功能受累最高,其病死率与受累脏器数成正比,并与病程长,年老龄高,就诊不及时相关。结论:通过实施积极的护理措施,尽可能减少了病人再感染的机会,预防并发症的发生,使患者身心处于主动配合接受治疗的最佳状态,为医生实施治疗方案提供可靠的基础条件与依据,对疾病的预后和转归有重要意义。