RIBRON: el registro español informatizado de bronquiectasias. Caracterización de los primeros 1.912 pacientes

IntroductionThe SEPAR Spanish Bronchiectasis Registry (RIBRON) began as a platform for the collection of longitudinal data on patients with this disease. The objective of this study is to describe its operation and to analyze the characteristics of bronchiectasis patients according to sex.MethodsA total of 1912 adult patients diagnosed with bronchiectasis in 43 centers were included between February 2015 and 2019. All patients had complete data consisting of at least 79 basic required variables, controlled by an external audit.ResultsMean age was 67.6 (15.2) years; 63.9% were women. The most common symptom was productive cough (78.3%) which was mucopurulent-purulent in 45.9% of cases. The most common etiology was post-infectious (40.4%), while 18.5% were idiopathic. Pseudomonas aeruginosa was the most frequently isolated microorganism (40.4%), of which 25.6% were associated with chronic infection. The annual number of mild-to-moderate/severe exacerbations was 1.62 (1.9)/0.59 (1.3). Half of the patients (50%) presented with airflow obstruction (17% severe). The most frequent radiological localization was lower lobes. The average FACED/E-FACED/BSI values were 2.06 (1.7)/2.67 (2.2)/7.8 (4.5), respectively. Overall, 66.7% of patients were taking inhaled corticosteroids, 19.2% macrolides, and 19.5% inhaled antibiotics. Women presented a less severe profile than men in clinical and functional terms, and a similar infectious, radiological and therapeutic profile.ConclusionsRIBRON represents an excellent map of the characteristics of bronchiectasis in our country. Two thirds of patients are women who presented lower disease severity as a specific characteristic.     

比较分析 α-糜蛋白与尿激酶雾化吸入在支气管扩张治疗中的应用效果

目的比较分析α-糜蛋白与尿激酶雾化吸人在支气管扩张治疗中的应用效果。方法选择2011年5月-2013年1月葛洲坝中心医院治疗的支气管扩张患者50例,随机分为观察组和对照组,每组各25例。对照组患者给予仪一糜蛋白雾化吸入治疗。观察组患者给予尿激酶雾化吸人治疗,对两组患者临床疗效及治疗前后动脉血气指标进行分析比较。结果①观察组患者治愈率为32．0％,总有效率为96．0％;对照组患者治愈率为24．0％,总有效率为80．0％;观察组患者的治愈率和总有效率均明显高于对照组,差异有统计学意义（P〈0．05）。②治疗后观察组PaCO2水平[（40．4±8．4）mmHg]明显低于对照组[（48．2±9．0）mmHg],PaO2水平[（86．6±11．2）mmHg]明显高与对照组[（72．4±10．5）mmHg],差异有统计学意义（P〈0．05）。结论尿激酶较仅一糜蛋白雾化吸入在支气管扩张治疗中具有较好的应用效果,值得推广。     

支气管扩张合并感染患者血清suPAR、PCT和CRP水平及其临床意义

目的分析支气管扩张合并感染患者血清可溶性尿激酶型纤溶酶原激活物受体(soluble urokinase-type plasminogen activator receptor,suPAR)、降钙素原(procalcitonin,PCT)和C反应蛋白(C-reactive protein,CRP)水平的检测意义。方法收集2016年5月—2019年5月在广安市人民医院就诊的100例支气管扩张合并感染患者(研究组),根据病情严重程度将其分为轻度(n=23)、中度(n=56)、重度(n=21)3个亚组;将同期30例健康体检者纳入对照组。比较研究组各亚组间及研究组与对照组受试者血清suPAR、PCT、CRP水平差异,应用受试者工作特征曲线(receiver operating characteristic curve,ROC)及曲线下面积（area under curve,AUC）评价suPAR、PCT、CRP及三者联合对支气管扩张合并感染的诊断价值。结果研究组血清suPAR、PCT、CRP水平高于对照组(P<0.05);研究组各亚组间血清suPAR、PCT、CRP水平差异有统计学意义(P<0.05),各指标水平轻度组低于中、重度组,中度组低于重度组(P<0.05);血清suPAR、PCT、CRP水平与受试者是否患支气管扩张合并感染密切相关(P<0.05)。suPAR AUC为0.951(P<0.05),临界值为5.88 ng/mL,敏感性、特异性分别为0.850、1.000;PCT AUC为0.943(P<0.05),临界值为0.13 ng/mL,敏感性、特异性分别为0.860、0.967;CRP AUC为0.921(P<0.05),临界值为5.16 mg/L,敏感性、特异性分别为0.830、0.933;三者联合预测AUC为0.989,敏感度、特异度分别为0.950、1.000,优于各自单独预测(P<0.05)。结论血清suPAR、PCT、CRP水平升高与支气管扩张合并感染发生、发展密切相关,联合检测对支气管扩张合并感染诊断有一定价值。     

氨茶碱联合罗红霉素治疗支气管扩张的效果

目的 研究氨茶碱联合罗红霉素治疗支气管扩张的效果及对肺功能的影响.方法 将本院2013年8月至2015年12月收治的84例支气管扩张患者随机分为对照组与实验组,各42例.对照组采用罗红霉素治疗,实验组采用氨茶碱与罗红霉素联合治疗.比较两组治疗效果、治疗前后肺功能指标变化.结果 治疗后,两组PEF、FEV1及FVC指标均有所提高,但对照组肺功能指标提高程度低于实验组,差异有统计学意义(P＜0.05);对照组治疗有效率(76.2％)低于实验组(92.9％),差异有统计学意义(P＜0.05).结论 氨茶碱联合罗红霉素治疗支气管扩张效果显著,可改善患者肺功能,提高患者生活质量.     

A targeted next-generation sequencing panel reveals novel mutations in Japanese patients with primary ciliary dyskinesia

Objective

Primary ciliary dyskinesia (PCD) is a rare genetic disorder caused by functional impairment of cilia throughout the body. The early diagnosis of PCD is important for the prevention of long-term sequelae; however, this is often challenging because of the phenotypic heterogeneity of PCD and difficulty in genetic analysis. The majority of PCD patients in Japan are not diagnosed properly. To diagnose PCD more accurately, we developed a targeted next-generation sequencing (NGS) panel.

A Review of Non-Cystic Fibrosis Pediatric Bronchiectasis

With the implementation of vaccination programs and the use of antibiotics, developed countries have seen a decline in infection-related pediatric bronchiectasis. However, significant morbidity from bronchiectasis is still seen and both infectious and noninfectious causes of bronchiectasis in the pediatric population remain. A review of the literature will be presented including causes of pediatric bronchiectasis, clinical symptoms and signs, laboratory evaluation and imaging, as well as treatment options. This review stresses the importance of early evaluation and treatment in children with recurrent cough, sinusitis, potential foreign-body aspiration, or gastroesophageal reflux to prevent the complications of ongoing respiratory disease and bronchiectasis.     

DR在支气管扩张症诊断中的应用研究

目的 探讨DR胸部影像中对支气管扩张症病灶进行计算机图像后处理的应用价值。方法 选取100例经临床或CT确诊的支气管扩张症的DR胸部影像。（1）采用影像对照方法，评价DR平片，影像增强片对支气管扩张病灶的显示能力。（2）对DR影像增强前后的囊状透光区进行灰度测量，比较其囊壁囊腔平均差值，观察其灰度轮廓的特征。结果 （1）DR平片，影像增强片对支气管病灶的显示，有显著差异。（2）DR胸片影像增强前后囊状病灶灰度轮廓线呈凹形城墙样曲线，而正常支气管管腔轮廓线无此改变，（3）DR胸片影像增强前后囊壁囊腔灰度平均差修正人显著差异。结论 DR胸片经数字化影像增强技术处理，对显示支气管扩张症的病灶明显优于处理前的DR平片，灰度轮廓线与影像增强技术有助于提高支气管扩张症的诊断准确率。     

泛复舒联合体位引流治疗支气管扩张的临床研究

目的 探讨泛福舒联合体位引流对支气管扩张患者治疗的临床疗效.方法 选择支气管扩张患者78例,经过急性期治疗病情得到控制后,以自愿原则随机双盲分为两组,对照组单纯体位引流,治疗组口服泛福舒的同时体位引流,观察两组患者3个月后免疫指标变化和6个月内临床症状即每日咳嗽咳痰量及呼吸道再次感染次数.结果 治疗组病例咳嗽咳痰的症状减轻显著高于对照组(P=0.046),呼吸道再次感染次数显著下降(P=3.43× 10-12);治疗组治疗后CD4+、CD4+/CD8+、NK细胞活性值均较治疗前明显提高(P＜0.0 1),CD8+活性值较治疗前降低(P＜ 0.01).对照组治疗前后CD4+、CD4+/CD8+、NK值均无明显变化.治疗组患者体内IgG、IgA的表达水平在治疗后显著高于对照组患者(P＜0.01).结论 泛福舒联合体位引流可减少支气管扩张患者每日咳嗽及咳痰量,降低呼吸道再次感染发病率,增强机体免疫力.且泛福舒药物耐受性好,副反应小.     

1例老年急性髓细胞白血病伴支气管扩张患者的药学监护

摘 要 目的:探讨急性髓细胞白血病伴支气管扩张患者治疗中的药物治疗方案及药学监护要点。方法：通过对1例老年急性髓细胞白血病伴支气管扩张患者诊治过程实施药学监护,分析患者的治疗方案,根据患者的病情变化提供用 药建议,监护患者安全用药,促进了药物的合理化使用。结果：药师应根据患者的具体情况,将药学理论和临床实践相结合,提高药物治疗水平,真正做到治疗个体化。结论：通过实施药学监护可以促进合理用药和个体化给药,避免不良反应的发生。