肺表面活性蛋白C基因异常与婴幼儿肺间质性疾病

肺表面活性蛋白C(surfactant protein C,SP-C)基因是目前被发现的唯一仅在肺泡Ⅱ型上皮细胞中表达的肺表面活性蛋白基因,其蛋白表达产物SP-C是构成肺表面活性物质的小分子疏水性蛋白之一,具有调节肺泡液-气界表面张力、维持肺表面活性膜的稳定及参与肺器官局部防御体系等重要的生理功能。SP-C基因异常可造成SP-C结构变化和功能丧失,从而导致各种婴幼儿肺疾病,其中,肺间质性疾病(in- terstitial lung disease,ILD)的发病与SP-C基因突变的关系尤为密切。     

基于深度学习的慢性阻塞性肺病的诊断模型研究

慢性阻塞性肺病(COPD)是一种常见的以持续气流受限为特征的慢性呼吸道疾病,具有很高的发病率和死亡率。目前临床上对COPD的诊断方式十分复杂,不仅耗时且有创或有辐射伤害,不适用于日常筛查。本研究设计了一种基于深度学习的COPD诊断模型。首先,将RespiratoryDatabase @ TR多媒体呼吸数据库中42位COPD患者的肺音数据和来自天津大学胸科医院的24位COPD患者以及37位健康受试者的临床采集肺音数据相结合,分别运用高通滤波器和基于集合经验模态分解(EEMD)及小波熵的去噪算法进行去噪处理,然后通过归一化、交叠剪切、数据扩增完成预处理过程;然后利用二阶谱分析技术提取肺音特征;最后,将特征输入到改进的19层卷积神经网络模型中,实现健康受试者与COPD患者的二分类。实验结果表明,所提出的模型能够有效诊断COPD,其准确度、敏感度、特异性、F1分数和Kappa系数分别达到了98.93%、98.47%、99.41%、98.95%和97.86%,且由于采用了双中心数据并进行了去噪处理,模型可靠性更高,具有重要的临床意义。     

Tetracycline Antibiotics Induce Host-Dependent Disease Tolerance to Infection

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Relationship of Vitamin D Binding Protein Polymorphisms and Lung Function in Korean Chronic Obstructive Pulmonary Disease

Purpose

Multiple genetic factors are associated with chronic obstructive pulmonary disease (COPD). The association of gene encoding vitamin D binding protein (VDBP, GC) with COPD has been controversial. We sought to investigate the types of GC variants in the Korean population and determine the association of GC variants with COPD and lung function in the Korean population.

Materials and Methods

The study cohort consisted of 203 COPD patients and 157 control subjects. GC variants were genotyped by the restriction fragment-length polymorphism method. Repeated measures of lung function data were analyzed using a linear mixed model including sex, age, height, and pack-years of smoking to investigate the association of GC genetic factors and lung function.

Results

GC1F variant was most frequently observed in COPD (46.1%) and controls (42.0%). GC1S variant (29.0% vs. 21.4%; p=0.020) and genotype 1S-1S (8.3% vs. 3.4%; p=0.047) were more commonly detected in control than COPD. According to linear mixed model analysis including controls and COPD, subjects with genotype 1S-1S had 0.427 L higher forced expiratory volume in 1 second (FEV₁) than those with other genotypes (p=0.029). However, interaction between the genotype and smoking pack-year was found to be particularly significant among subjects with genotype 1S-1S; FEV₁ decreased by 0.014 L per smoking pack-year (p=0.001).

Conclusion

This study suggested that GC polymorphism might be associated with lung function and risk of COPD in Korean population. GC1S variant and genotype 1S-1S were more frequently observed in control than in COPD. Moreover, GC1S variant was more common in non-decliners than in rapid decliners among COPD.     

Interstitial Lung Disease of Systemic Sclerosis

Fibrosis of the pulmonary parenchyma is a frequent and serious complication of scleroderma (systemic sclerosis, SSc), resulting in significant morbidity and mortality. During the past decade data have accumulated in support of an inflammatory process affecting the alveoli and distal airways that culminates in irreversible fibrosis in many SSc patients. Recent findings indicate the presence of lung fibroblasts with altered phenotype and biologic activity (myofibroblasts), perhaps arising from the influence of cytokines on resident lung fibroblasts. Acute-phase inflammatory cytokines such as IL-1α, TNF-α, MlP-1α, IL-8 and RANTES are increased in SSc bronchoalveolar lavage (BAL) fluid, as is thrombin, a potent mitogen for lung fibroblasts. Chronic-phase inflammatory and fibrogenic cytokines such as PDGF and TGF-β are also present in increased amounts in SSc BAL fluid. The inciting event(s) and the process(es) leading to the perpetuation of fibrosis in SSc are unknown. Treatment of SSc lung disease has been empiric and generally disappointing, and it is likely that effective treatment awaits a better understanding of the biological events that regulate collagen and other extracellular matrix synthesis.     

Inspiratory muscle strength training improves lung function in patients with the hypermobile Ehlers–Danlos syndrome: A randomized controlled trial

As exertional inspiratory dyspnea is a common disabling complaint in hypermobile Ehlers–Danlos syndrome (hEDS) often also known as joint hypermobility syndrome (JHS), we investigated inspiratory muscle (IM) strength in patients with hEDS, and we assessed the effects of IM training (IMT) on IM strength, lung function, and exercise capacity. A prospective evaluation of IM strength followed by a randomized controlled trial of IMT was performed in women with hEDS. Sniff nasal inspiratory pressure (SNIP) was used to routinely measure IM strength and IMT was carried out using a pressure threshold device. IM strength (main outcome), cardiopulmonary function, exercise capacity, and emotional distress of both the treated and control groups were evaluated at the start and at the end of the 6‐week training period. IM strength was reduced (<80% of predicted) in 77% of patients (80/104). Lung function was normal, although 24% of patients had a higher forced expiratory vital capacity (FVC) than normal and 12% of patients had a higher total lung capacity (TLC) than normal. Both the IMT and control groups (n = 20) had similar baseline characteristics. Significant changes were noted only in the IMT group after IMT. At the end of the program, IMT improved SNIP (20%) (before: 41 ± 17 cm H₂O [28, 53] vs. after: 49 ± 18 cm H₂O [34;65]), six‐minute walking distance (6MWD) (60 m) (455 ± 107 m [379,532] vs. 515 ± 127 m [408, 621]), and forced expiratory volume in one second (FEV1) (285 mL) (94 ± 14% pred [84,104] vs. 103 ± 11% pred [94, 112]). IM strength is significantly reduced in patients with hEDS. IMT improved IM strength, lung function, and exercise capacity. Our findings suggest that IMT should be added to usual care.     

Alzheimer病与Parkinson病患者记忆损害的比较研究

目的：测查Alzheimer病、Parkinson病患者的记忆功能，了解这两种疾病病人记忆损伤的特征。方法：采用多维记忆评估量表中的12个分测验对30例原发性Parkinson病患者、30例临床诊断可能的Alzhdmer病患者及年龄、性别和教育程度与2个病人组相匹配的2个正常对照组进行外显记忆、内隐记忆及日常生活记忆等记忆功能的评定。结果：AD组在所有的分测验和记忆因子上均差于对照组；PD组在图画再认、数字广度、汉词回忆、图形再生、经历定向等分测验和记忆广度、再认记忆、自由回忆、日常记忆、外显记忆等记忆因子上均显著低于对照组：经以教育年限为协变量校正后发现。除自由组词、残图命名、数字广度、空间广度、汉词回忆、常识记忆、记忆广度、内隐记忆外，AD组在其余各分测验和记忆因子上均存显著差于PD病人组。结论：AD病人存在着广泛的记忆功能障碍：PD病人存在着工作记忆和策略记忆的障碍：两者的记忆功能障碍存在着明显的不同。     

Hyperhomocysteinemia Associates with Small Vessel Disease More Closely Than Large Vessel Disease

Background: Hyperhomocysteinemia was believed to be an independent risk factor for stroke and associate with small vessel disease (SVD) related stroke and large vessel disease (LVD) related stroke differently. However it''s still unclear which type of stroke associated with homocysteine (HCY) more strongly because the conclusions of previous studies were contradictory. In this study we focused on the subclinical angiopathies of stroke, i.e., SVD and LVD instead of stroke subtypes and sought to compare the associations between HCY level and different angiopathies. Methods: 324 non-stroke patients were enrolled. Sex, age, HCY level and other vascular risk factors were collected. MRI and angiographies were used to determine the type of angiopathies and their severity, i.e., the scores of leukoaraiosis (LA), plaques and numbers of silent brain infarctions (SBI). LVD was defined as the presence of atherosclerotic plaques of cerebral arteries. SVD was defined as the presence of either LA or SBI. 230 patients were deemed to have LVD; 180 patients were deemed to have SVD. Spearman''s correlation test and logistic regression were used to analyze the association between HCY level and different angiopathies. Results: The correlation between HCY level and scores of plaques was weaker than that of the scores of LA and numbers of SBI. Hyperhomocysteinemia was an independent risk factor for SVD (OR = 1.315, P <0.001), whereas the association between HCY level and LVD was not that significant (OR = 1.058, P = 0.075). Conclusion: HCY level associated with SVD more strongly than LVD.     

Superantigens in Human Disease

Superantigens have been implicated in a wide variety of human diseases. Yet, solid evidence for their role in pathogenesis is available only for Toxic Shock Syndrome and a few other conditions. This evidence is critically reviewed herein.     

系统性硬化症相关间质性肺病的治疗

系统性硬化症是一种可累及全身多个系统的结缔组织病,预后较差,死亡率较高。肺部受累引起间质性肺病是系统性硬化症患者常见的死亡原因。国内外尚无系统性硬化症相关间质性肺病的诊疗指南,环磷酰胺是目前唯一治疗该病的一线药物,然而,环磷酰胺的毒副作用限制了其应用。近年来,该病的治疗受到国内外学者越来越多的关注,本文就系统性硬化症相关间质性肺病的治疗策略做一总结,并对新兴的治疗方案进行重点阐述。     

血清铁蛋白检测在心脑血管疾病与代谢性疾病中的临床意义

探讨心脑血管疾病与代谢性疾病患者血清铁蛋白（serum ferritin,SF）的变化及其临床意义。采用罗氏全自动免疫分析仪E170与日立7600型全自动生化分析仪检测82例心脑血管疾病患者、60例代谢性疾病患者、111名健康对照者的血清SF和相关生化指标,用SPSS17.0对结果进行统计分析。结果显示：心脑血管疾病组的SF水平显著高于代谢性疾病组与健康对照组（P〈0.01）,代谢性疾病组与健康对照组SF水平差异有统计学意义（P〈0.05）。心脑血管疾病组SF与血清尿素氮（BUN）、血清载脂蛋白AI（Apo AI）、血清肌酸激酶（CK）、血清胱抑素C（Cys-C）、血清唾液酸（SA）的相关性较显著（P〈0.01）;代谢性疾病组SF与Apo AI的相关性较显著（P〈0.01）。血清铁蛋白在心脑血管疾病与代谢性疾病中均有不同程度的升高,在心脑血管疾病与代谢性疾病临床诊断中具有重要意义。     

超声评价老年心血管病患者颈动脉粥样硬化的价值

探讨超声对老年心血管病患者颈动脉粥样硬化的评价价值。方法 在我院2017年7月~2018年6月收治的老年心血管疾病患者中随机抽取76例设为观察组,随机抽取同期于我院行健康体检的76例老年人设为对照组。两组受检者均进行颈部超声检查,比较两组受检者的颈部血管动脉粥样硬化斑块面积、斑块积分以及IMT值,VA、CCA以及ICA部位、血流量及脑血流量。结果 观察组患者的斑块面积、斑块积分以及IMT值均高于对照组（P＜0.05）;VA、CCA以及ICA部位的狭窄程度均较对照组严重（P＜0.05）;VA、CCA以及ICA部位的血流量与脑血流量均比对照组小（P＜0.05）。结论 超声评价老年心血管病患者颈动脉粥样硬化具有无创性、高效性以及操作简易等优点,可以掌握颈动脉粥样硬化疾病的不同状态,有利于老年患者病情发展与预后情况的判断,对临床工作有着重要指导作用。     

毫米波与疾病的研究进展

毫米波是一种高频电磁波,已发现毫米波在很多疾病的治疗中发挥作用。本文拟对豪米波与疾病的研究进展进行探讨。     

独特型网络与自身免疫病

1974年Jerne提出了免疫调节的独特型网络学说。该学说认为:机体免疫系统中存在着一个复杂的调节网络,其主要成分是抗体分子的可变区上存在着独特型(Idiotyp,Id)决定簇,该Id可被抗独特型抗体(Anti—idiotype antibody, Anti-Id)所识别,并受其相应的Anti-Id所调节。Anti-Id的独特型又被抗抗独特型抗体(Anti-anti-Id anti-body, Anti-anti-Id)所识别。如此继续下去,形成了一个相互联系的复杂网络,即独特型网络。Id网络中发挥调节作用的基础,除游离于循环中的抗体分子上的     

儿童川崎病临床特征分析

目的:分析并比较典型川崎病(KD)与不典型川崎病(IKD)的临床特征。方法:回顾性分析2017-01-01—2017-12-31武汉大学人民医院儿科收治的69例患儿的临床资料,将其分为IKD组(n=25)和KD组(n=44),比较两组患儿的临床特征。结果:两组患儿发热发生率均为100%,IKD组持续发热时间、确诊时间及临床治愈时间均明显高于KD组(P<0.05或P<0.01);两组患儿皮疹、颈淋巴结肿大、口唇皲裂及杨梅舌发生率差异无统计学意义(P>0.05);IKD组手足硬性水肿发生率低于KD组(P<0.05);IKD组肛周脱皮、卡疤红肿、冠状动脉损伤发生率均高于KD组(P<0.05)。结论:IKD具有发热时间久,早期不易确诊,冠状动脉损伤发生率高的特点,影响患儿预后,需早期识别诊断。