波生坦对高原性肺水肿的治疗作用

目的:探讨波生坦对高原性肺动脉高压的治疗作用及其对NO的影响.方法:SD大鼠随机分为:正常组,安慰剂组和波生坦.除正常组外,其他组置于减压中,8h/d,持续6周.自第4周起,安慰剂组和波生坦组的大鼠在低压低氧前分别给与生理盐水或波生坦灌胃.测定各组大鼠的平均肺动压(mPAP)、右心室肥厚指数(RV/LV+S%)、肺系数;测定各组大鼠动脉血和肺组织中NO的含量以及肺组织中NOS的活性.结果:和正常组相比,安慰剂组大鼠的mPAP,RV/LV+S%和肺系数显著升高;波生坦能显著抑制RV/LV+S%和肺系数的升高,并使mPAP下降至正常组水平.安慰剂组大鼠动脉血及肺组织中NO的含量和肺组织中NOS的活性都明显降低于正常组;波生坦治疗组大鼠动脉血和肺组织中NO的含量以及肺组织中NOS的活性较安慰剂组都显著升高.结论:波生坦通过增加低压低氧大鼠体内NOS的活性和NO的含量,来发挥其治疗高原性肺水肿的作用.     

川芎嗪联合阿托伐他汀治疗肺动脉高压的临床疗效分析

目的:观察川芎嗪联合阿托伐他汀治疗肺动脉高压的临床疗效及安全性。方法:将96例肺动脉高压患者随机分成两组,其中治疗组48人,对照组48人。治疗组患者在常规治疗基础上采川芎嗪联合阿托伐他汀治疗,对照组患者在常规治疗基础上单采用阿托伐他汀治疗,通过检测平均动脉压(mAP)、平均肺动脉压(mPAP)、肺血管阻力(PVR)、心脏指数(CI)、左心室射血分数(LVER)、Borg呼吸困难指数(Borg dyspnea)、6分钟步行距离(6TWD)以及肺功能升级(WHO FC)等指标评价其疗效。结果:两组患者经过6个月的治疗后,mPAP、PVR与治疗前相比显著降低,而CI、LVEF和6MWD均显著升高,差异均有统计学意义(P<0.05),治疗前后两组mAP与Borg呼吸困难指数均没有统计学差异(P>0.05),治疗组的mAP、LVEF以及6TWD与对照组相比差异有统计学意义(P<0.05)。结论:川芎嗪联合阿托伐他汀治疗充肺动脉高压临床疗效优于单纯应用阿托伐他汀。     

重组人生长激素治疗特发性矮小症的临床疗效及安全性研究

目的 探讨重组人生长激素治疗特发性矮小症的临床效果及安全性. 方法 选取2018年2月～2021年2月收治的86例特发性矮小症患儿为研究对象,随机分为对照组和观察组,每组43例.对照组给予常规治疗,观察组使用重组人生长激素治疗,比较两组的治疗效果. 结果 治疗后,观察组的GV、PAH、Ht-SDSBA、Ht-SDSCA...     

他达那非治疗肺动脉高压进展

肺动脉高压是一类以小肺动脉血管重构为特征的恶性肺血管疾病,肺血管阻力进行性升高最终导致右心衰竭死亡。肺动脉高压的药物治疗主要针对前列环素、内皮素、一氧化氮三个靶点。他达那非(Tadalafil)是一种口服、长效、选择性5-型磷酸二酯酶抑制剂,增加环磷酸鸟苷(cGMP)浓度,通过一氧化氮途径起到治疗作用。PHIRST临床试验证实肺动脉高压患者每日一次口服他达那非40mg,能够提高6分钟步行距离,减少临床恶化。本文就他达那非治疗肺动脉高压研究新进展作一综述。     

聚焦治疗肺动脉高压的靶向药物

肺动脉高压(PAH)是一种以血管收缩和重构所致的肺血管阻力升高为病理生理学特征的临床综合征,其呈进行性发展,最终可 导致右心衰竭甚至死亡,预后差。随着对 PAH 病理生理学机制的深入研究,该病所涉不同信号通路的关键位点逐渐被认识,相应的靶向 药物也不断涌现,它们的应用明显改善了PAH患者的生活质量和预后。综述针对PAH 发生发展过程中所涉的3条主要通路:前列环素通路、 内皮素通路和一氧化氮-鸟苷酸环化酶-环磷酸鸟苷通路以及其他靶点(如酪氨酸激酶受体和 Rho 激酶等)而开发的靶向药物研究进展。     

生长激素治疗特发性矮小疗效观察及分析

目的:探究生长激素对特发性矮小(idiopathic short,ISS)的作用及临床疗效。方法:在我院临床接受ISS治疗的病患中,随机选取84例作为研究对象。按照随机分组法把其分为观察组(44例)和对照组(40例)。对照组进行常规治疗。观察组每日睡前注射生长激素0.16—0.20IU/kg。对比分析两组病患治疗后身高、身高标准差及生长速率情况。结果:对照组治疗后无明显增高现象,而观察组增高现象显著。差异均有统计学意义(均P0.05)。观察组疗效优者占63.6%(28/44),优良率为95.5%(42/44),均明显高于对照组的35%(14/40),80%(32/40),差异均有统计学意义(均P0.05)。结论:生长激素用于治疗ISS病患疗效显著,并能有效改善ISS病患身高、生长速率,安全性有保证,值得临床治疗广泛推荐。     

MMF联合泼尼松治疗成人特发性膜性肾病的疗效及安全性研究

目的:探究吗替麦考酚酯(MMF)联合泼尼松(Pre)治疗成人特发性膜性肾病(IMN)的疗效及安全性。方法:选取2015.06-2017.06我院收治的102例行IMN患者列为研究对象,将患者按随机数字表法以1:1比例分为对照组与观察组,每组各51例,对照组患者使用Pre进行治疗,观察组患者使用MMF+Pre进行治疗,治疗12个月。比较两组治疗12个月后疗效,治疗前、治疗后6个月及12个月后肾功能相关指标[24 h尿蛋白定量(24 h UP)、血清胱抑素C(CysC)、血清尿素氮(BUN)、血清肌酐(Scr)、血清白蛋白(Alb)]、脂代谢指标[总胆固醇(TC)、甘油三酯(TG)],并记录用药间期出现的药物不良反应。结果:治疗12个月后,研究组患者总有效率为90.20%,显著高对照组74.51%(P0.05);治疗6个月、12个月后,两组患者24 h UP、Cys C、TC、TG水平较治疗前均依次显著下降而Alb水平显著上升(P0.05),且观察组上述指标与同期对照组对比差异显著(P0.05);而两组BUN和Scr水平较治疗前差异不显著(P0.05),两组对比无统计学意义(P0.05)。治疗期间,观察组患者药物不良反应率为11.76%,显著低于对照组27.45%(P0.05)。结论:应用MMF联合泼尼松治疗成人IMN疗效更佳,可显著改善患者肾功能,并改善患者脂代谢,药物方案安全性较高,具有较高应用价值。     

慢性栓塞性肺动脉高压的药物治疗进展

药物治疗对不能手术和在围手术期的慢性栓塞性肺动脉高压患者的治疗起重要的作用。传统的药物如抗凝药物、利尿剂、洋地黄类、氧疗等不能改变疾病的预后,治疗效果也有限。近年来,一些新型药物被用于治疗肺动脉高压,包括前列腺环素类似物、内皮素受体拮抗剂和磷酸二酯酶5的抑制剂等。它们在非栓塞性肺动脉高压的治疗中可以明显改善预后,由于相似的病理机制,所以它们对栓塞性肺动脉高压也可能有效,但证据有限。本文将对这些药物在慢性栓塞性肺动脉高压治疗中的应用作一总结。     

枸橼酸坦度螺酮胶囊治疗神经性头痛50例疗效观察

目的:观察枸橼酸坦度螺酮胶囊治疗神经性头痛的临床效果。方法:将100例神经性头痛患者随机分为治疗组和对照组各50例。对照组给予氨酚羟考酮片、甲钴胺片治疗,治疗组在对照组的基础上加用枸橼酸坦度螺酮胶囊,对比两组临床疗效及视觉模拟评分(VAS评分)。结果:治疗后两组患者VAS评分均显著减小,其中治疗组相比对照组降低更明显,差异均有统计学意义(均P0.05);治疗组患者总有效率为88.0%(44/50)显著高于对照的68.0%(34/50),差异具有统计学差异(P0.05);两组患者均无明显不良反应。结论:枸橼酸坦度螺酮胶囊联合氨酚羟考酮片、甲钴胺片治疗神经性头痛疗效优良,能有效缓解痛疼程度,副作用小。     

缺氧性肺动脉高压的治疗进展

摘要：缺氧性肺动脉高压（HPH）是指由于长期缺氧导致血管内皮细胞损伤,血管活性因子水平失调,以血管收缩及血管重塑为主要病理表现的一类肺动脉高压症（PAH）,HPH发病率高,死亡率高,除了肺移植,目前尚无根治办法,对于HPH的治疗一直是研究热点,本文将结合近年国内外最新研究从基因靶向、离子通道、线粒体、NO相关药物、西药、中药领域对HPH的治疗做一概述。     

Apelin-APJ Signaling: a Potential Therapeutic Target for Pulmonary Arterial Hypertension

Pulmonary arterial hypertension (PAH) is a progressive disease characterized by the vascular remodeling of the pulmonary arterioles, including formation of plexiform and concentric lesions comprised of proliferative vascular cells. Clinically, PAH leads to increased pulmonary arterial pressure and subsequent right ventricular failure. Existing therapies have improved the outcome but mortality still remains exceedingly high. There is emerging evidence that the seven-transmembrane G-protein coupled receptor APJ and its cognate endogenous ligand apelin are important in the maintenance of pulmonary vascular homeostasis through the targeting of critical mediators, such as Krűppel-like factor 2 (KLF2), endothelial nitric oxide synthase (eNOS), and microRNAs (miRNAs). Disruption of this pathway plays a major part in the pathogenesis of PAH. Given its role in the maintenance of pulmonary vascular homeostasis, the apelin-APJ pathway is a potential target for PAH therapy. This review highlights the current state in the understanding of the apelin-APJ axis related to PAH and discusses the therapeutic potential of this signaling pathway as a novel paradigm of PAH therapy.     

Risk Factors for Hemoptysis in Idiopathic and Hereditary Pulmonary Arterial Hypertension

Introduction

When hemoptysis complicates pulmonary arterial hypertension (PAH), it is assumed to result from bronchial artery hypertrophy. In heritable PAH, the most common mutation is in the BMPR2 gene, which regulates growth, differentiation and apoptosis of mesenchymal cells. The aim of this study is to determine the relationship in PAH between the occurrence of hemoptysis, and disease progression, bronchial artery hypertrophy, pulmonary artery dilation and BMPR2 mutations.

Methods

129 IPAH patients underwent baseline pulmonary imaging (CT angio or MRI) and repeated right-sided heart catheterization. Gene mutations were assessed in a subset of patients.

Results

Hemoptysis was associated with a greater presence of hypertrophic bronchial arteries and more rapid hemodynamic deterioration. The presence of a BMPR2 mutation did not predispose to the development of hemoptysis, but was associated with a greater number of hypertrophic bronchial arteries and a worse baseline hemodynamic profile.

Conclusion

Hemoptysis in PAH is associated with bronchial artery hypertrophy and faster disease progression. Although the presence of a BMPR2 mutation did not correlate with a greater incidence of hemoptysis in our patient cohort, its association with worse hemodynamics and a trend of greater bronchial arterial hypertrophy may increase the risk of hemoptysis.     

Quality of Life,Safety and Efficacy Profile of Thermostable Flolan in Pulmonary Arterial Hypertension

Background

Flolan (epoprostenol sodium) is most commonly prescribed to patients with severe pulmonary arterial hypertension (PAH) owing to the requirement that the drug be delivered by continuous intravenous infusion and the reconstituted solution may only be administered up to 24 hours when it is maintained between a temperature of 2°C and 8°C. The aim of this single-arm, open label study was to describe the effects of the new thermostable formulation of Flolan on health-related quality of life (HRQoL) and ease of administration in subjects switching from the currently marketed Flolan to the reformulated product.

Methods

Following a 4-week run-in period and after 4 weeks of treatment with the reformulated product, patients completed the SF-36 HRQoL questionnaire and a study-specific questionnaire evaluating ease of administration, along with World Health Organization (WHO) functional class, six-minute walked distance (6MWD) and N-terminal-pro B-type natriuretic peptide (NT-proBNP) assessment.

Results

16 participants completed the study. The SF-36 scores remained unchanged from baseline to Week 4. Conversely, there were small improvements for the majority of the study-specific questionnaire items and 14 (88%) subjects preferred the reformulated product to the currently marketed Flolan. There was no significant change in the dose of reformulated product, 6MWD, Borg dyspnoea index, WHO functional class and mean NT-proBNP levels. No significant changes in haemodynamic parameters were seen from baseline to 2 hours post transition in a subset of patients undergoing catheterization.

Conclusion

The reformulated product was not associated with significant improvement in HRQoL compared with the currently marketed Flolan as measured by the SF-36. However, most subjects preferred the reformulated product to the currently marketed Flolan. Moreover, the 2 formulations of Flolan had similar safety and efficacy profiles.

Trial Registration

ClinicalTrials.gov {"type":"clinical-trial","attrs":{"text":"NCT01462565","term_id":"NCT01462565"}}NCT01462565     

阿奇霉素联合辛伐他汀对慢性阻塞性肺疾病合并肺动脉高压患者肺功能的影响

目的:探讨阿奇霉素联合辛伐他汀治疗慢性阻塞性肺疾病合并肺动脉高压的临床疗效及对患者肺功能的影响。方法:选取2013年6月-2016年3月我院收治的慢性阻塞性肺疾病合并肺动脉高压患者107例,根据治疗方法不同分为对照组(49例)与实验组(58例)。对照组患者采用阿奇霉素治疗,实验组患者在对照组基础上给予辛伐他汀治疗。观察并比较两组患者的临床疗效、不良反应以及肺功能指标的变化情况。结果:实验组患者治疗有效率(87.93%)高于对照组(73.47%),差异具有统计学意义(P0.05)。与治疗前比较,两组患者治疗后1 s用力呼吸容积(FEV1)、用力肺活量(FVC)及FEV1/FVC水平均升高,差异具有统计学意义(P0.05);与对照组比较,实验组患者治疗后1 s用力呼吸容积(FEV1)、用力肺活量(FVC)及FEV1/FVC水平较高,差异具有统计学意义(P0.05)。治疗后,两组患者血清总胆固醇(TC)及三酰甘油(TG)水平均降低,差异具有统计学意义(P0.05);与对照组比较,实验组患者治疗后血清总胆固醇(TC)及三酰甘油(TG)水平较低,差异具有统计学意义(P0.05)。两组患者不良反应发生率比较,差异无统计学意义(P0.05)。结论:阿奇霉素联合辛伐他汀治疗慢性阻塞性肺疾病合并肺动脉高压的临床效果显著,不仅能够改善患者肺功能,降低血脂相关指标水平,并且安全性较高,值得临床推广应用。     

特发性肺纤维化发病机制的研究进展

特发性肺纤维化是指以肺泡上皮细胞损伤、成纤维细胞大量增生和细胞外基质聚集增多为病理特征且病因不明的一类慢性间质性肺疾病。由于病因不清,目前发病率约为16.3/100,000,且缺乏有效诊疗手段和治疗药物。肺纤维化对人体健康危害极大,愈后困难,存活率较低。因此加深对纤维化机制的阐明对于了解疾病的发生、发展和防治就显得十分必要,更是人类对健康的迫切要求。本文就近几年关于特发性肺纤维化发病机制的研究进展作一简要综述。     

小剂量阿奇霉素联合吸入布地奈德和N-乙酰半胱氨酸治疗特发性肺纤维化的临床疗效分析

目的:观察小剂量阿奇霉素联合吸入布地奈德和N-乙酰半胱氨酸对特发性肺纤维化(IPF)患者肺功能及支气管肺泡灌洗液炎症因子表达的影响。方法:随机将56例确诊为IPF的住院患者分为对照组和治疗组,对照组给予常规治疗,吸氧,戒烟,吸入布地奈德及乙酰半胱氨酸;治疗组在常规治疗基础上加服小剂量阿奇霉素片。动态观察两组患者治疗3、6个月的疗效及肺功能的改善情况;完成2个疗程(6个月)治疗后,检测和比较两组支气管肺泡灌洗液相关炎症因子水平的变化。结果:治疗3个月时,两组患者的动脉血氧分压(PO2)、肺活量(VC)、1秒钟用力呼气容积占预计值百分比(FEV1%pred)、最大通气量占预计值百分比(MVV%pred)、单位肺泡容积的一氧化碳弥散量占预计值百分比(DLCO/VA%pred)均较治疗前显著增加(P0.05);治疗6个月时,治疗组的有效率(20/26,76.9%)明显高于对照组(14/30,46.7%),且PO2、VC、FEV1%pred、MVV%pred及DLCO/VA%pred等肺功能指标均显著高于对照组(P0.05);对照组支气管肺泡灌洗液TNF-α、IL-8浓度均较治疗前降低(P0.05),IL-4、IL-10、IFN-γ浓度较治疗前无明显变化;而治疗组支气管肺泡灌洗液TNF-α、IL-8、IL-4、IL-10浓度均较治疗前降低,IFN-γ浓度较治疗前明显增加(P0.05)。结论:阿奇霉素联合吸入布地奈德和N-乙酰半胱氨酸可能通过改变支气管肺泡灌洗液IFN-γ等炎症因子浓度有效改善IPF患者的肺功能。