Ciliary assessment in bronchiectasis

Bronchiectasis is a common condition among the Oriental population and affected patients suffer from chronic sputum production punctuated by recurrent infective exacerbations. Cilia are minute structures present on the surface of respiratory and other epithelial cells that beat continuously to maintain a sterile mucosal surface in the respiratory tract. Patients with primary ciliary dyskinesia could potentially develop recurrent sinotrachrobronchitis, bronchiectasis, serous otitis media, hydrocephalus, and male infertility. The assessment of cilia has, however, received little attention until recently and generally involves elaborate methods that require complex and expensive technology. This brief article discusses application of the saccharine test, light microscopy assessment of ciliary beat, and transmission electron microscopy assessment of the ultrastructure of cilia. The rationale and indications for ciliary assessment are also listed along with illustrations showing ciliary structure, equipment required for sampling and assessment of cilia, and transmission electron micrographs of ciliary ultrastructural abnormalities.     

Airway nitric oxide output is reduced in bronchiectasis

BACKGROUND: Increased concentrations of exhaled nitric oxide (NO) have been detected in inflammatory lung diseases including asthma and have been attributed to increased expression and activity of inducible nitric oxide synthase (iNOS) within the airways. However, previous studies of exhaled NO in patients with bronchiectasis have yielded conflicting results, with reports of both increased and normal NO values. Recent evidence from animal models suggests that chronic airway infection reduces NO production within the lung, despite causing increased iNOS expression. We tested the hypothesis that, in human subjects with bronchiectasis, chronic airway infection reduces NO output from the conducting airways. METHODS: Using a recently described two-compartment model, we measured separately the contributions of the conducting airways and the alveoli to exhaled NO in nine patients with stable bronchiectasis and eight control subjects before and after inhaled glucocorticoid therapy. RESULTS: We found that airway NO output was significantly lower in bronchiectasis than in normal airways whereas NO output from the alveoli was similar to that of control subjects. High-dose inhaled glucocorticoid therapy did not alter airway or alveolar NO production. CONCLUSIONS: These findings demonstrate that, in patients with bronchiectasis, airway NO output is reduced and that iNOS does not contribute significantly to airway NO production.     

Early radiographic and clinical features associated with bronchiectasis in children

Bronchiectasis among children living in developing regions is associated with respiratory infections during early childhood, but specific risk factors that precede childhood bronchiectasis are not fully characterized. We hypothesized that severe respiratory syncytial viral (RSV) infection in infancy would increase the risk of bronchiectasis among Alaska Native children in rural Alaska. This was a follow-up cohort study of a 1993-1996 case-control study of RSV-hospitalized case patients and their controls. For each 5-8-year-old former case-patient and control subject, we reviewed medical records, interviewed parents, performed physical examinations and spirometry, collected sera, and analyzed all historical chest radiographs. Ten (11%) RSV cases and 10 (9%) controls had radiographic evidence of bronchiectasis. The mean age at radiographic diagnosis of bronchiectasis was 3.3 years (range, 1.2-6.1 years). Children were more likely to develop bronchiectasis if their chest radiographs, when they were < 2 years of age, showed lung parenchymal densities (RR = 3.9, P < 0.013), persistent parenchymal densities > 6 months' duration (RR = 3.0, P = 0.02), or infiltrates on multiple episodes (test for trend, P = 0.003). Radiographic features of hyperinflation and atelectasis among children < 2 years old were not associated with eventual bronchiectasis. A single severe infection with RSV alone did not predispose Alaska Native infants to bronchiectasis. Childhood bronchiectasis was associated with lung and hence airway injury, manifested on radiographs by parenchymal densities or "pneumonia" rather than by hyperinflation or atelectasis.     

支气管扩张症伴有气道阻塞的临床特点分析

目的:探讨支气管扩张症伴有气道阻塞的临床特点。方法:回顾性收集2007年1月至2012年12月间,在我院就诊住院的160例支气管扩张症急性加重患者的临床资料。根据其肺功能是否提示阻塞性通气功能障碍分为阻塞性组、非阻塞性组。比较两组患者在临床表现、胸部高分辨CT、肺功能结果及实验室检查等方面的差异。结果:肺功能提示阻塞性通气功能障碍的支扩患者病程长,出院后1年内发生急性加重的次数多,呼吸困难评分(m MRC)高(P<0.05),FVC%预计值、FEV1%预计值、FEV1/FVC%预计值及DLCO/VA%预计值较非阻塞性组低,而RV%预计值、TLC%预计值及RV/TLC%预计值高于非阻塞性组(P<0.05)。结论:合并气道阻塞的支气管扩张症患者病程更长,发生急性加重次数更多,呼吸困难更明显,肺功能更差。     

Ciliary neurotrophic factor prevents retrograde neuronal death in the adult central nervous system.

The neurocytokine ciliary neurotrophic factor (CNTF) was described originally as an activity that supports the survival of neurons of the chicken ciliary ganglia in vitro. The widespread expression of CNTF and its principal binding protein, CNTF receptor alpha, in the central and peripheral nervous systems suggests a broader trophic role for this peptide. In the present study, we report that CNTF prevents axotomy-induced cell death of neurons in the anteroventral and anterodorsal thalamic nuclei of the adult rat. Using the polymerase chain reaction, we also demonstrate the presence of CNTF and CNTF receptor alpha mRNA in these same thalamic nuclei. The coincidence of CNTF and its receptor in a population of neurons responding to the factor suggests a paracrine function for CNTF. The present findings establish that CNTF has significant effects on neurons of the central nervous system in vivo and demonstrate that neurocytokines can prevent cell death in the adult central nervous system.     

慢性阻塞性肺疾病合并支气管扩张患者的临床特点分析

目的分析慢性阻塞性肺疾病(简称慢阻肺)合并支气管扩张患者的临床特征,提高对该病的认识和诊断水平。方法选择我院2011~2013年间住院的112例慢阻肺患者为研究对象,分为慢阻肺合并支气管扩张组(n=48)和慢阻肺组(n=64),比较两组患者的临床特征、胸部高分辨率CT(HRCT)、肺功能、血气分析表现,随访6个月记录两组患者的急性加重次数。结果慢阻肺合并支气管扩张组BMI低于慢阻肺组;日常咳脓性痰者比例、入院前一年的住院次数均明显高于慢阻肺组(P值分别0.01与0.05)。慢阻肺合并支气管扩张组患者FEV1%pred、FEV1/FVC、FVC%pred、DLCO%pred降低较单纯慢阻肺组更为显著(P0.05)。慢阻肺合并支气管扩张组6个月内平均每人发生急性加重次数(1.47±0.85次);高于慢阻肺组患者(1.06±0.54次),两者比较差异有统计学意义(P0.05)。结论慢阻肺患者中合并支气管扩张者较为常见,合并支气管扩张的慢阻肺患者肺功能下降更为明显,发生急性加重的风险增加。     

GDF11 level in patients with myelodysplastic syndrome and is clinical significance

<正>Objective To detect the expression level of growth differentiation factor 11(GDF11)in patients with myelodysplastic syndrome(MDS),and to evaluate the relationship between GDF11 level and erythropoiesis functions.Methods A total of 44 MDS patients(18 low-risk group patients and 26 high-risk group patients)in the Department of Hematology in Tianjin Medical University     

支气管扩张症合并慢性肺源性心脏病临床特点分析

目的:分析支气管扩张症合并慢性肺源性心脏病患者的临床特点。方法:回顾首都医科大学附属北京安贞医院2013年1月至2014年12月,就诊住院的支气管扩张症急性加重患者125例,分为支气管扩张症不合并慢性肺源性心脏病组(n=62),支气管扩张症合并慢性肺源性心脏病组(n=63),比较两组患者在临床资料、超声心动图参数、急性加重期病原学、实验室检查及胸部高分辨CT等方面的差异。结果:两组患者在年龄、性别、BMI、糖尿病、结核病病史等方面,差异均无统计学意义(P0. 05)。与不合并慢性肺源性心脏病相比,支气管扩张症合并慢性肺源性心脏病患者的病程长,吸烟指数高,呼吸困难评分(mMRC)高(P0. 05),BNP水平、超声估测的肺动脉收缩压力较高(P0. 05),而超声心动图LVEF、血气分析氧分压低于不合并慢性肺源性心脏病组。急性加重病原学方面,两组差异无统计学意义(P0. 05),但病毒感染在两组急性加重病原学中均占有重要比重(10%)。两组患者胸部CT表现差异无统计学意义(P0. 05)。结论:支气管扩张症合并慢性肺源性心脏病患者较不合并患者的病程长,临床指标偏重,需要临床医生早期预警及筛查。     

Localized bronchiectasis is a definite association of coronaro-bronchial artery fistula

An 81-year-old smoker presented with acute coronary syndrome. Coronary angiography revealed severe double-vessel disease, as well as the incidental finding of a coronaro-bronchial artery fistula that arose from the left circumflex artery. Percutaneous coronary intervention was performed on the culprit lesion in the left anterior descending artery. A subsequent high-resolution computed tomography of the thorax revealed mild bronchiectic change in the corresponding area supplied by the coronaro-bronchial artery fistula. The patient had a normal lung function test and never had any chest symptoms. From the literature, the association of the coronaro-bronchial artery fistula and localized bronchiectasis is very definite, but the pathophysiology is still controversial.     

Characterisation of the onset and presenting clinical features of adult bronchiectasis

BACKGROUND: There is little information available on the features of initial presentation of bronchiectasis and documentation of the onset and progress of symptoms leading up to this. Therefore a study was performed on a large cohort of adult patients presenting to Monash Medical Centre (MMC) to survey the course of their disease up to the time of diagnosis. OBJECTIVES: To characterise the onset and presenting clinical features of bronchiectasis in adults. METHODS: A cross-sectional study of 103 adults presenting to a tertiary referral hospital with newly diagnosed bronchiectasis. Clinical features of bronchiectasis and results of spirometry, sputum microbiology and radiology were assessed and correlated. RESULTS: Most patients had idiopathic bronchiectasis (74%) and did not have other significant disease. The dominant symptom was chronic productive cough present in 98% of patients with other important symptoms being chronic rhinosinusitis (70%), dyspnoea (62%), and fatigue (74%). Most patients had had a chronic productive cough for over 30 years prior to diagnosis and over 80% of patients had chronic respiratory symptoms from childhood. The dominant finding on physical examination was the presence of crackles which were generally bi-basal. Spirometry showed mild airway obstruction with an average forced expiratory volume in 1s of the cohort of 76% predicted. Radiologic imaging generally showed multilobar disease (80%). CONCLUSIONS: The typical profile of bronchiectasis in this group of patients was of longstanding productive cough, rhinosinusitis and fatigue in non-smokers with crackles on chest auscultation.