Objective To improve the knowledge of kartagener syndrome and the understanding of primary ciliary dyskinesia ( PCD) . Methods Three cases of Kartagener syndrome were reported and analyzed on clinical manifestations, symptoms, imaging appearances of chest, and family history. Meanwhile the related literatures were reviewed. Results The three patients deserved special recognition because of recurrent upper and lower respiratory tract infection. Of them, the third case showed mainly the symptoms of nasosinusitis at an early age, and the distinct pulmonary symptoms appeared in his adulthood. Further examination showed that the triad of chronic sinusitis, bronchiectasis, and situs inversus is present in all cases. The parents of the two cases were consanguineous marriaged. Examination of the bronchial mucosal biopsy specimen of the first case under transmission electron microscopy showed cilia with the absence of inner dynein arms. The three cases got improvement after treatment of anti-infection and expectorant. Conclusion For patients with recurring infection in the upper and lower respiratory tract from infancy, the possibility of PCD should be considered no matter whether a situs inversus exists.
Objective To evaluate the efficacy of long-term inhaled salmeterol / fluticasone combined with low-dose oral erythromycin in patients with bronchiectasis. Methods Sixty-two patients with bronchiectasis after exacerbation and maintained stable were randomly divided into three groups. Group A was treated with low-dose oral erythromycin, group B inhaled salmeterol/fluticasone, and group C inhaled salmeterol/fluticasone plus low-dose oral erythromycin. The study duration lasted for 6 months. The clinical symptoms, dyspnea scale, exacerbation frequency, and pulmonary function parameters were measured and compared. Results Fifty-four patients completed the whole study and 8 cases withdrew. The results showed that 6 months of low-dose erythromycin therapy can improve the clinical symptoms, whille exacerbation frequency was also decreased. Inhaled salmeterol/fluticasone improved lung function, however, had no effect on cough, expectoration and exacerbation frequency. Inhaled salmeterol/fluticasone combined with erythromycin was more significantly effective in improving lung functions as well as symptoms. Conclusions Long-terminhaled salmeterol/fluticasone combined with low-dose oral erythromycin can improve the clinical symptoms and lung function, decrease the frequency of exacerbation in patients with bronchiectasis. It may be as an alternative to the maintenance treatment of 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.
Bronchiectasis is a heterogeneous disease characterized by abnormal expansion of the bronchi, manifested by cough, sputum, and recurring lung infections. As one of the common fungi of lung infection, aspergillus can not only appear as the outcome of the disease in bronchiectasis, but also as an inducement to participate in the disease progression, and ultimately complicate the course of bronchiectasis. This article describes the susceptibility factors and pathogenic mechanisms of aspergillus in bronchiectasis, and further introduces the diagnosis and treatment status of bronchiectasis combined with aspergillus infection, aiming to clarify the effect of aspergillus infection on bronchiectasis and provide new thinking directions for its clinical diagnosis and treatment.
Objective To explore the distribution of bacteria among community acquired lower respiratory tract infection (LRTI) inpatients with underlying chronic respiratory tract diseases.Methods The clinical data，sputum culture and drug susceptibility results of 212 community acquired LRTI patients who were hospitalized during the period 2001-2005 were retrospectively analyzed.All patients had various underlying chronic respiratory tract diseases.Results A total of 229 strains of pathogens were detected，with the majority being gram negative bacteria.In pathogens of acute exacerbation of chronic obstructive pulmonary disease，gram negative bacteria occupied 73.9%.And Pseudomonas aeruginosa and Klebsiella pneumoniae were the most common pathogens，with each occupying 18.2% and 13.6% respectively.Gram positive bacteria occupied 23.8%，mainly Staphylococcus aureus (10.2%) and Streptococcus pneumoniae (9.1%).In patients with bronchiectasis exacerbated by bacterial infection，86.2% were caused by gram negative bacteria，the top three being，in descending order，Pseudomonas aeruginosa (27.5%)，Haemophilus parainfluenzae (13.7%)，and Haemophilus influenzae (11.8%).Bronchiectasis was the major risk factor of getting Pseudomonas aeruginosa infection (OR=5.590，95%CI 2.792~11.192).The risk factors of getting Acinetobacter baumanii infection were antacid usage within 1 month (OR=9.652，95%CI 2.792~11.192) and hypoalbuminemia (OR=2.679，95%CI 1.108~6.476).For enterobacters infections，including Klebsiella pneumoniae，Enterobacter cloacae and Escherichia coli，the risk factors were antibiotic usage within 1 month (OR=4.236，95%CI 1.982~9.057)，having renal diseases (OR=4.305，95%CI 1.090~17.008) and diabetes mellitus (OR=2.836，95%CI 1.339~6.009).Conclusions Gram negative bacteria were the main pathogens of community acquired LRTI in hospitalized patients with underlying chronic respiratory tract diseases.The pathogens were influenced by underlying diseases，severity of diseases and drug usage history of patients.
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.
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.
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 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.