ObjectiveTo investigate the pathogenesis, clinical manifestations, diagnosis and treatment of common variable immune deficiency (CVID).MethodsOne case of CVID with cellular immunodeficiency leading to bronchiectasis and liver cirrhosis was analyzed retrospectively. Relevant literatures were also searched through WanFang Database, China National Knowledge Infrastructure and PubMed, Ovid, Embase, Cochrane using the key words " common variable immunodeficiency”, " common variable hypogammaglobulinemia” in Chinese and English.ResultsA 52-year-old female patient, complained of cough, expectoration for 20 years, edema for 7 years and aggravated for 3 months with a history of recurrent respiratory infections was hospitalized in the West China Hospital of Sichuan University. The chest computed tomography revealed bronchiectasis, liver cirrhosis and portal hypertension. Laboratory tests showed remarkable hypogammaglobulinemia. The CD4+ T-cell count was below the normal range. Probable diagnosis of CVID was made based on clinical characteristics and laboratory tests. Immunoglobulin infusion with a dose of 20 g was given and the symptoms were relieved. About 288 case reports including 8 000 patients were searched. Most of them were reported individually. Conclusions CVID has a low morbidity and is rare in China. It is mostly caused by genetic factors. When there are recurrent infections in common areas of body, infections in rare areas or infections of conditioned pathogen, clinicians should be vigilant and give intervention as soon as possible. Family and genetic researches could be done when permitted.
Chronic airway diseases constitute the majority of mortality of respiratory diseases in China. The 2017 Global Initiative for Chronic Obstructive Lung Disease has proposed a novel scheme for classification of disease severity. The mainstream for chronic obstructive pulmonary disease (COPD) management has shifted to the combination of long acting β2 agonists (LABA) and long acting muscarinic cholinergic antagonists instead of inhaled corticosteroid and LABA. Tiotropium was effective in early COPD with little or even without symptoms. The manangement strategy on COPD may be moving to the upper stream (early intervention). Greater interest has been focusing on clinical phenotyping and inflammatory pathways in asthma. The greater understanding of the pathogenesis of asthma has been associated with the clinical trial progress which suggests that multiple medications targeting at Th2 pathways may provide benefits for implementing personalized therapy. Medications targeting at neutrophilic airway inflammation and blockade of KIT pathways are expected to provide novel rationales for managing asthma with different phenotypes. There has been a considerable progress in bronchiectasis research in China, particularly in terms of etiology, bacteriology and clinical phenotying investigations. The establishment of bronchiectasis research centers in China may help better understanding of the pathogenesis of bronchiectasis, thus identifying potential targets for intervention, which may provide crucial rationale for future intervention to improve the long-term prognosis.
ObjectiveTo explorer the risk factors for acute exacerbation in patients with bronchiectasis within one year.MethodsFour hundred and twenty-two patients with non-cystic fibrosis bronchiectasis hospitalized were enrolled in The East Region of the People’s hospital of Sichuan between October 2014 and October 2016. The patients’ clinical data were collected, and follow-up began at the time of discharged. The study endpoint was the first acute exacerbation, all patients were followed-up for one year after discharged. The patients were classified into two groups by the occurrence of acute exacerbation or no occurrence. Logistic regression analysis was used to explore the risk factors for acute exacerbation with bronchiectasis.ResultsThe age, sick time, body mass index (BMI) less than 18.5 kg/m2, smoking index, expectoration, hemoptysis, dyspnea, moist sounds, wheezing sounds, types of imaging, CT scores, lung lesion site, sputum culture, whether infected Pseudomonas aeruginosa, level of serum C-reactive protein (CRP), level of serum PCT, serum albumin, arterial carbon dioxide partial pressure, types of respiratory failure, combined with chronic cor pulmonale differed significantly between the two groups (P<0.05), while gender, history of Infection, smoking, cough, chest pain, fever, clubbed-finger, white blood cell counts, neutrophil counts, erythrocyte sedimentation rate, serum globulins, arterial oxygen partial pressure did not significantly differ (P>0.05). Multivariate Logistic regression analysis found that infection with Pseudomonas aeruginosa, BMI<18.5 kg/m2, high level of serum CRP, high level of arterial carbon dioxide partial pressure (PaCO2), high CT score with bronchiectasis, combination with chronic cor pulmonale were risk factors for acute exacerbation in patients with bronchiectasis (P<0.05).ConclusionsInfection with pseudomonas aeruginosa, BMI < 18.5 kg/m2, high serum CRP level, high arterial blood PaCO2 level, high CT score with bronchiectasis and combination of chronic cor pulmonale are risk factors for acute aggravation within 1 year for patients with bronchiectasis. Doctors can identify these risk factors and intervene early, so as to reduce the acute exacerbation of bronchiectasis.
ObjectiveTo evaluate the combination efficacy with Qingfei Yihuo capsule and routine antibiotics as well as mucopolytic agents in the treatment of bronchiectasis acute exacerbation.MethodsThis was a prospective, multi-center, randomized controlled clinical study. The efficacy of Qingfei Yihuo capsule combine with routine antibiotics and mucopolytic agents in the treatment of bronchiectasis acute exacerbation was compared according to the symptom control as well as exacerbation duration. Through randomization, patients received Qingfei Yihuo capsule or placebo combine with routine antibiotics and mucopolytic agents treatment for 10 days. Symptom score of cough, sputum, short of breath pre- and post-treatment as well as the symptom score in daily card were compared between the two groups. The spirometry and St. George respiratory questionnaire (SGRQ) before and after treatment were compared.ResultsThis study was conducted from June 2017 to August 2018. One hundred and ninety patients from 7 centers in 6 hospitals with bronchiectasis acute exacerbation were enrolled in the study. There was statistically improvement of symptom score (including the 9th and 10th treatment days) according to the daily card recording in Qingfei Yihuo capsule group compared to the placebo group, but no statistically significant difference was found in spirometry results or SGRQ.ConclusionQingfei Yihuo capsule has assistant effect on improving respiratory symptoms of bronchiectasis exacerbation.
ObjectiveTo explore the risk factors for death in patients with bronchiectasis. MethodsTwo hundred and eighty-three patients diagnosed with bronchiectasis at Daxing Hospital of Capital Medical University from January 2011 to December 2013 were collected and followed up to October 2015 after discharge. Patients' age, gender, body mass index (BMI), smoking history, dyspnea score, image data, sputum culture, blood gas analysis and the results of spirometry were collected. The risk factors for death were analyzed with COX regression analysis. ResultsAmong 283 cases, 52 patients died. The 1-, 2-, 3-and 4-year cumulative survival rates were 97%, 86%, 71%, 45%, respectively. COX regression analysis showed that age≥70 years (RR=2.222, 95%CI 1.145-4.314), BMI < 18.5 kg/m2 (RR=2.328, 95%CI 1.205-4.497), bronchiectasis involving≥3 lobes in chest high-resolution computed tomography (RR=0.382, 95%CI 0.188-0.774) and FEV1% pred < 70% (RR=1.032, 95%CI 0.923-1.180) were the independent risk factors for death of patients with bronchiectasis (all P < 0.05). ConclusionsThere are multiple risk factors contribute to death of patients with bronchiectasis. Early identification of risk factors shall improve the prognosis of patients with bronchiectasis.
ObjectiveTo investigate the relationship between the red blood cell distribution width (RDW) and the severity of acute exacerbation of bronchiectasis in elderly patients.MethodsThe clinical data of 216 elderly patients with acute exacerbation of bronchiectasis admitted from January 2015 to October of 2018 were analyzed retrospectively. The severity of acute exacerbation of bronchiectasis in the elderly was evaluated by bronchiectasis severity index (BSI) score. Meanwhile, 50 elderly people receiving qualified medical examination were collected as a healthy control group in the same period. The distributions of BSI score, RDW, procalcitonin (PCT), neutrophil percentage (NEU%) and C-reactive protein (CRP) were described in the patients with different risk degree. The severity of acute exacerbation of bronchiectasis in the elderly was evaluated by BSI. The patients were divided into three groups by BSI score: a low risk group, a middle risk group, and a high risk group. The indexes were described including the distribution of stratified BSI score, RDW, PCT, NEU%, CRP at different risk levels. The correlation of each index was analyzed by Spearman correlation. The threshold value of RDW was calculated by general linear regression, and the influencing factors of BSI score were analyzed by multivariate linear regression analysis.ResultsThe higher the risk stratification, the higher the BSI score, RDW, PCT, NEU% and CRP were. RDW was positively correlated with PCT, NEU% and CRP (r values were 0.425, 0.311, 0.177, respectively, P<0.05). BSI score was positively correlated with RDW, PCT, NEU%, and CRP (r values were 0.425, 0.394, 0.650, 0.578, respectively, P<0.05). RDW was positively correlated with PSI score (r=0.425, P<0.05). The thresholds of RDW were 11.45% and 14.03%. Multiple linear regression showed that RDW, PCT, NEU% and CRP were all influential factors of BSI score and explained 52.3% of the total mutation rate.ConclusionRDW is related to the severity of acute exacerbation of bronchiectasis in the elderly, and can predict the severity of acute exacerbation of bronchiectasis in the elderly.
Abstract： Objective To compare clinical outcomes of complete video-assisted thoracoscopic surgery （cVATS） lobectomy with routine thoracotomy lobectomy for the treatment of bronchiectasis, and evaluate the feasibility, safety and specific considerations of cVATS lobectomy for the treatment of bronchiectasis. Methods We retrospectively analyzed the clinical data of 115 patients who underwent lobectomy for the treatment of bronchiectasis in Beijing Chaoyang Hospital from May 2009 to January 2012. According to the way of operation, these patients were divided into two groups （cVATS group and thoracotomy group）. In the thoracotomy group, there were 62 patients （28 males and 34 females with an age of 46.2±11.9 years） who underwent routine thoracotomy lobectomy. In the cVATS group, there were 53 patients （19 males and 34 females with an age of 45.7±12.2 years） who underwent cVATS lobectomy. Operation time, intra-operative blood loss, postoperative thoracic drainage, complications and hospitalization, and cost of hospitalization were compared between the two groups. Results There was no perioperative death in all patients. There was no statistical difference in operation time between the two groups. Intra-operative blood loss of thoracotomy group patients was significantly higher than that of cVATS group patients （228.8±121.7 ml vs. 157.1±123.8 ml, t=2.592, P=0.011）. Postoperative thoracic drainage （866.7±439.5 ml vs. 1 805.3±466.7 ml, t=9.003，P=0.000）, duration of chest tube drainage （6.6±3.3 d vs. 9.8±4.6 d,t=3.339，P=0.001）, and postoperative hospitalization （7.5±2.2 d vs. 11.2±5.4 d, t=3.424，P=0.001） of cVATS group were significantly lower than those of thoracotomy group. The cost of hospitalization of cVATS group was significantly higher than that of thoracotomy group （38 543.6±11 051.8 yuan vs. 30 523.4±10 028.5 yuan，t=3.423, P=0.001）. There was no statistical difference in postoperative complications between the two groups （P＞0.05）. Forty-five patients in cVATS group were followed up for 2 to 14 months. Among them, 42 patients completely had no sputum or haemoptysis and 3 patients still intermittently had some sputum. Conclusion cVATS lobectomy is safe and feasible for the treatment of bronchiectasis. Compared with routine thoracotomy lobectomy, cVATS lobectomy does not increase surgical risk, but can significantly reduce operation injury and length of hospital stay. During cVATS, the lung residual should be handled cautiously especially for patients with nonanatomic pulmonary resection, and the non-single-order operation is recommended.
Objective To evaluate the clinical effects of bronchial artery embolization ( BAE) for massive hemoptysis due to bronchiectasis.Methods 205 patients with massive hemoptysis were treated with bronchial artery embolization using coils, polyvinyl alcohol ( PVA) microspheres, line segmen, and gelatin sponge after the site of bleeding or the abnormal arteries were identified by arteriography. Super selective bronchial artery embolization was performed with a coaxial microcatheter inserted into the bronchial artery. Results BAE was successfully performed in 205 cases with massive hemoptysis ( left and right bronchial artery embolization in 35 cases, left bronchial artery embolization in 20 cases, right bronchial artery embolization in 126 cases, common bronchial artery embolization in 22 cases, right inferior phrenic artery embolization in 2 cases) . Of 205 patients, 169 were cured, 24 were relieved with a success rate of 94.1% . Long termcumulative hemoptysis nonrecurrence rates was 82.4% . 23 patients developed post embolization syndrome characterized by mild fever and chest pain and ended with spontaneous recovery without special management. No severe complications including spinal cord injury or dystopia embolization were observed. Conclusions Bronchial arterial embolization interventional therapy is a rapid, safe and effective method in the treatment of massive hemoptysis due to bronchiectasis.
ObjectiveTo describe the clinical characteristics of pulmonary nocardiosis associated with bronchiectasis and to evaluate the methods of diagnosis and treatment.MethodsClinical data of two patients with pulmonary nocardiosis and bronchiectasis were analyzed and the literature on the subject were reviewed.ResultsTwo female patients with bronchiectasis were respectively 55 and 62 years old, both of them presented with fever, cough, expectoration, and leukocytosis. Case 1 also complicated with respiratory failure and leukemoid reaction. Chest CT showed bilateral pulmonary multi-patchy consolidations with cavities in case 1 and unilateral pulmonary local consolidation without cavities in case 2. Sputum smears on modified Ziehl-Neelsen staining of both patients showed typical Nocardia hyphe. Sputum culture of case 1 showed Nocardia otitidiscaviarum, which was sensitive to sulfamethoxazole/trimethoprim (SMZ/TMP), amikacin and moxifloxacin while resistant to ceftriaxone, imipenem and meropenem. Sputum culture of case 2 was negative. Case 1 was treated with SMZ/TMP + moxifloxacin + amikacin for 6 months, and case 2 treated with SMZ/TMP for 3 months. Both patients were clinically cured. Six case reports including 7 patients were searched, including 4 Chinese reports and 2 English reports. Most of them were reported individually. ConclusionsBronchiectasis is a risk factor for pulmonary nocardiosis, and the treatment of nocardiosis should accord with drug susceptibilities and severities of diseases. The prognoses of patients with pulmonary nocardiosis and bronchiectasis are relatively good.
Objective To investigate the cardiovascular events (CVE) and survival status of patients with bronchiectasis (BE) during follow-up after acute exacerbation. Methods Prospective cohort study was used. Clinical data of 134 BE patients with acute exacerbation who were hospitalized from July 2016 to September 2020 were collected. The patients were followed up after discharge by phone or respiratory clinic every 3 months until November 2022. CVE or death was the endpoint event. Result During the follow-up period, 41 patients developed CVE, while 93 patients did not. Fifty-one patients died during the follow-up period, with a mortality rate of 38.06%. Among them, 41 cases of CVE resulted in 21 deaths, with a mortality rate of 51.22%; 30 cases died in 93 non-CVE patients, with a mortality rate of 32.26%. Logistic regression results showed significant influencing factors for CVE in BE patients were age, hypertension, chronic obstructive pulmonary disease (COPD), and moderate to severe illness. The significant influencing factors for the death of BE patients were age, COPD, moderate and severe illness, and CVE events. The significant influencing factors for the death of CVE patients were age and receiving CVE treatment. The area under ROC curve (AUC) and 95%CI was 0.858 (0.729 - 0.970) for the warning model for CVE in BE patients. The AUC (95%CI) was 0.867 (0.800 - 0.927) for the warning model for death in BE patients. The AUC (95%CI) was 0.811 (0.640 - 0.976) for the warning model for death of CVE patients. Conclusions Population factors and comorbidities are risk factors for CVE in BE patients after acute exacerbation. The appearance of CVE worsens the long-term prognosis of BE patients. The corresponding warning models have high warning effectiveness with AUC>0.8.