Abstract: BACKGROUND Chronic obstructive pulmonary disease (COPD) is common although often poorly characterised, particularly in primary care. However, application of guidelines to the management of such patients needs a clear understanding of the phenotype. In particular, the British guidelines for the management of COPD recommend that the diagnosis is based on appropriate symptoms and evidence of airflow obstruction as determined by a forced expiratory volume in one second (FEV 1 ) of 1 /VC ratio of METHODS A study was undertaken of 110 patients aged 40–80 years who had presented to their general practitioner with an acute exacerbation of COPD. The episode was treated at home and, when patients had recovered to the stable state (two months later), they were characterised by full lung function tests and a high resolution computed tomographic (HRCT) scan of the chest. RESULTS There was a wide range of impairment of FEV 1 which was in the normal range (⩾80%) in 30%, mildly impaired (60–79%) in 18%, moderately impaired (40–59%) in 33%, and severely impaired ( 1 /VC ratio was present in all patients with an FEV 1 of 1 of ⩾80% predicted. Only 5% of patients had a substantial bronchodilator response suggesting a diagnosis of asthma. Emphysema was present in 51% of patients and confined to the upper lobes in most (73% of these patients). HRCT evidence of bronchiectasis was noted in 29% of patients and was predominantly tubular; most (81%) were current or ex-smokers. A solitary pulmonary nodule was seen on 9% of scans and unsuspected lung malignancy was diagnosed in two patients. CONCLUSIONS This study confirms that COPD in primary care is a heterogeneous condition. Some patients do not fulfil the proposed diagnostic criteria with FEV 1 of ⩾80% predicted but they may nevertheless have airflow obstruction. Bronchiectasis is common in this group of patients, as is unsuspected malignancy. These findings should be considered when developing recommendations for the investigation and management of COPD in the community.

Abstract: Background—An obstructive defect is usual in bronchiectasis, but the pathophysiological basis of airflow obstruction remains uncertain. High resolution computed tomographic (CT) scanning now allows quantitation of static morphological abnormalities, as well as dynamic changes shown on expiratory CT scans. The aim of this study was to determine which static and dynamic structural abnormalities on the CT scan are associated with airflow obstruction in bronchiectasis. Methods—The inspiratory and expiratory features on the CT scan of 100 patients with bronchiectasis undergoing concurrent lung function tests were scored semiquantitatively by three observers. Results—On univariate analysis the extent and severity of bronchiectasis, the severity of bronchial wall thickening, and the extent of decreased attenuation on the expiratory CT scan correlated strongly with the severity of airflow obstruction; the closest relationship was seen between decreased forced expiratory volume in one second (FEV1) and the extent of decreased attenuation on the expiratory CT scan (Rs = ‐0.55, p<0.00005). On multivariate analysis bronchial wall thickness and decreased attenuation were consistently the strongest independent determinants of airflow obstruction. The extent of decreased attenuation was positively associated with the severity of bronchial wall thickness, but was not independently linked to gas transfer levels. Endobronchial secretions seen on CT scanning had no functional significance; the severity of bronchial dilatation was negatively associated with airflow obstruction after adjustment for other morphological features. Conclusions—These findings indicate that airflow obstruction in bronchiectasis is primarily linked to evidence of intrinsic disease of small and medium airways on CT scanning and not to bronchiectatic abnormalities in large airways, emphysema, or retained endobronchial secretions. (Thorax 2000;55:198‐204)

Abstract: The development and structure of the respiratory tract Functions of the respiratory tract Epidemiology and respiratory disease Lung defences and immunology The clinical manifestations of respiratory disease Some problems in radiological diagnosis Diagnostic procedures Drugs used in respiratory disorders Acute infections of the upper respiratory tract, trachea and bronchi Pneumonia Empyema, thoracis Lung abscess The pathogenesis, epidemiology and prevention of tuberculosis Clinical features of tuberculosis The treatment of tuberculosis Pulmonary disease caused by opportunist mycobacteria Fungal and actinomycotic diseases Parasitic diseases of the lung Chronic bronchitis and emphysema Respiratory failure Pulmonary embolism Pulmonary hypertension Pulmonary odema and the adult respiratory distress syndrome Bronchiectasis Sarcoidosis Asthma Hypersensitivity pneumonitis Pulmonary eosinophilia Pulmonary angiitis and granulomatosis Pneumothorax Cystic fibrosis Occupational lung diseases Drug-induced lung disease Lung disease in the immunocompromised patient Cancer of he lung Other pulmonary neoplasms and related conditions Cryptogenic fibrosing alveolitis Pulmonary manifestations of systemic disease Some less common lung diseases Developmental disorders of the lungs Diseases of the pleura Diseases of the mediastinum Diseases of the chest wall Abnormalities and diseases of the diaphragm Terminal care in respiratory disease

Abstract: Although bronchiectasis has become a rare condition in U.S. children, it is still commonly diagnosed in Alaska Native children in the Yukon Kuskokwim Delta. The prevalence of bronchiectasis has not decreased in persons born during the 1980s as compared with those born in the 1940s. We reviewed case histories of 46 children with bronchiectasis. We observed that recurrent pneumonia was the major preceding medical condition in 85% of patients. There was an association between the lobes affected by pneumonia and the lobes affected by bronchiectasis. Eight (17%) patients had surgical resection of involved lobes. We conclude that the continued high prevalence of bronchiectasis appears to be related to extremely high rates of infant and childhood pneumonia. Pediatr Pulmonol. 2000;29:182-187. Published 2000 Wiley-Liss, Inc.

Abstract: There has been intense research into the role nitric oxide (NO) plays in physiological and pathological mechanisms and its clinical significance in respiratory medicine Elevated levels of exhaled levels of exhaled NO in asthma and other inflammatory lung diseases lead to many studies examining NO as potential markers of airway inflammation, enabling repeated noninvasive and standardized monitoring of airway inflammation In airway inflammation, NO is not merely a marker but may have anti-inflammatory and pro-inflammatory effects Significant correlation has been found between exhaled NO and skin test scores in steroid naive asthmatic patients, allowing to discriminate patients with and without airway responsiveness Exhaled NO is significantly elevated in acute asthma, or steroid-resistant severe asthma, or when the maintenance dose of inhaled steroids is reduced, and quickly reduced down to the levels in patients with stable asthma after steroid treatment Exhaled NO has been successfully used to monitor anti-inflammatory treatment with inhaled corticosteroids in asthma Exhaled NO is extremely sensitive and rapid marker of the dose-dependent effect of steroid treatment, or asthma deterioration, which is increased to any changes in lung function, provocative concentration causing a 20% fall in forced expiratory volume, sputum eosinophilia or asthma symptoms Exhaled NO is not increased in stable chronic obstructive pulmonary disease (COPD), but patients with unstable COPD, or bronchiectasis have high NO levels Exhaled and nasal NO are diagnostically low in cystic fibrosis and primary pulmonary dyskinesia Analysis of exhaled air, including nitric oxide, is feasible and could provide a noninvasive method for use in monitoring and management of lung diseases

Abstract: The pulmonary associations of inflammatory bowel disease (IBD) are poorly characterized. The clinical, physiological and high-resolution computed tomographic thorax characteristics of the lung disease in patients with IBD presenting with respiratory symptoms are described. Detailed clinical information was obtained and standard pulmonary physiological tests and thorax high-resolution computed tomography performed on 14 patients with ulcerative colitis (UC) and three with Crohn's disease (CD), 10 male, aged 38-83 yrs. Respiratory symptoms had been present for 2-50 yrs and extraintestinal manifestations were present in three (17.6%). Normal pulmonary physiology (six patients) was associated with the high resolution computed tomographic changes of bronchiectasis, mosaic perfusion and air trapping suggestive of obliterative bronchiolitis and a pattern of centrilobular nodules and branching linear opacities ("tree in bud" appearance) suggestive of either cellular bronchiolitis or bronchiolectasis with mucoid secretions. Bronchiectasis was found in 13 patients (11 UC, 2 CD), 11 had air trapping and five had a "tree in bud" appearance on computed tomography. One patient had a predominantly peripheral reticular pattern at the lung bases similar to that found in cryptogenic fibrosing alveolitis and one patient had a mixed reticular and ground-glass pattern in the midzones with a patchy distribution in the central and peripheral portions of the lungs with air trapping. Eleven patients (three with alveolitis) exhibited a clinical and/or physiological response to steroids. Pulmonary abnormalities in ulcerative colitis and Crohn's disease can present years after the onset of the bowel disease and can affect any part of the lungs. Early recognition is important as they can be strikingly steroid-responsive.

Abstract: Degradation of extracellular matrix components is central to many pathological features of chronic destructive lung disorders. Desmosine and isodesmosine are elastin-derived cross-linked amino acids whose urine levels are considered representative of elastin breakdown. The aim of this study was to apply a novel methodology, based on high-performance capillary electrophoresis, to the quantification of desmosine and isodesmosine in 11 patients with stable chronic obstructive pulmonary disease (COPD), 10 with an exacerbation of COPD, nine with alpha1-antitrypsin deficiency, 13 with bronchiectasis, and 11 adults with cystic fibrosis, in comparison to 24 controls. It was found that, in patients with stable COPD, urinary desmosine levels were higher than in controls (p=0.03), but lower than in COPD subjects with an exacerbation (p< or =0.05). The highest desmosine levels were found in subjects with alpha1-antitrypsin deficiency, bronchiectasis and cystic fibrosis (p<0.001 versus stable COPD). In a short-term longitudinal study, five stable COPD patients showed a constant rate of desmosine excretion (mean coefficient of variation <8% over three consecutive days). In conclusion, the present method is simple and suitable for the determination of elastin-derived cross-linked amino acid excretion in urine, giving results similar to those obtained using other separation methods. In addition, evidence is presented that urinary desmosine excretion is increased in conditions characterized by airway inflammation, such as exacerbations of chronic obstructive pulmonary disease, bronchiectasis and cystic fibrosis. Results obtained in subjects with alphal-antitrypsin deficiency suggest that this method might be used to evaluate the putative efficacy of replacement therapy.

Abstract: The systemic autoimmune diseases include collagen vascular diseases, the systemic vasculitides, Wegener granulomatosis, and Churg-Strauss syndrome. They can cause a variety of thoracic abnormalities that are influenced by the pathophysiologic characteristics of the underlying disease process. Although many of the abnormalities can be detected at chest radiography, high-resolution computed tomography (CT) has been shown to be superior in depicting parenchymal, airway, and pleural abnormalities. Thoracic manifestations of collagen vascular diseases include pleural disease, pulmonary fibrosis, diaphragm weakness, aspiration pneumonia, bronchiolitis obliterans organizing pneumonia, bronchiolitis obliterans, and bronchiectasis. Wegener granulomatosis may be associated with multiple nodules or masses with irregular margins that are frequently cavitated. Patients with Churg-Strauss syndrome often have consolidation or ground-glass attenuation at chest radiography and CT. Goodpasture syndrome is associated with extensive bilateral air-space consolidation.

Abstract: Allergic bronchopulmonary aspergillosis (ABPA) is a hypersensitivity reaction to the fungus Aspergillus fumigatus that may progress to bronchiectasis. The aim of the present study was to characterize airway inflammation in patients with clinically stable ABPA and asthma, and to correlate this with bronchiectasis severity. Subjects with ABPA and central bronchiectasis (ABPA-CB; n=16) and ABPA with serological evidence alone (ABPA-S; n=10) were studied. Comparison groups were A. fumigatus-sensitized asthma (n=19), non-A. fumigatus-sensitized asthma (n=15) and healthy controls (n=8). Hypertonic saline challenge, sputum induction and high-resolution computed tomography (HRCT) of the chest were performed. Sputum eosinophil numbers were markedly elevated in ABPA-CB (median 8.4%) compared to ABPA-S (2.4%), A. fumigatus-sensitized asthma (1.8%), asthma (1.8%) and controls (0.3%) (p<0.01); sputum eosinophil cationic protein levels were higher in ABPA-CB (median 13,706 ng.mL(-1)), compared to ABPA-S (1,633.5 ng.mL(-1)), A. fumigatus-sensitized asthma (1,550.7 ng.mL(-1)), asthma (309.2 ng.mL(-1)), and controls (110 ng.mL(-1)) (p<0.001). ABPA-CB also showed increased sputum neutrophil number (median 60.3%) compared to the other groups (controls 29.3%) (p=0.01). The severity of bronchiectasis on HRCT correlated with sputum neutrophil (r=0.6) and eosinophil number (r=0.5) but not serum immunoglobulin-E levels. In conclusion, clinically stable allergic bronchopulmonary aspergillosis with bronchiectasis is characterized by an intense heterogenous inflammatory infiltrate consisting of eosinophils and neutrophils, which correlates closely with the severity of bronchiectasis on high-resolution computed tomography. Sputum analysis may be useful in monitoring the course of allergic bronchopulmonary aspergillosis.

Abstract: Primary ciliary dyskinesia (PCD) is a heterogeneous disease with impaired mucociliary transport leading to respiratory disorders, hearing impairment and male infertility. PCD can be diagnosed by clinical features together with functional and structural analysis of the cilia. To prevent bronchiectasis with a marked reduction in quality of life, early diagnosis is essential. The rarity of PCD and the costs of ultrastructural analysis of cilia require a rational diagnostic concept. We therefore reviewed the literature and compared clinical manifestations as well as functional and structural analyses of the cilia in 28 patients (23 children, 5 adults) investigated between 1990 and 1998. All were thoroughly examined for other possible diseases before biopsy, and ten patients (35.7%; eight children, two adults) were diagnosed as having PCD. From the literature review and our findings we conclude that ciliary investigation is indicated (a) in patients who remain suspected of having PCD despite thorough clinical examination and exclusion of other disorders such as cystic fibrosis, allergy, immunologic disorders and α1-antitrypsin deficiency; (b) in patients with situs inversus suffering from chronic and/or recurrent airway infections; and (c) in patients with neonatal respiratory distress syndrome of “unknown” cause (i.e. after exclusion of hyaline membrane disease, aspiration syndromes, neonatal pneumonia, and pneumothorax as well as cardiovascular and metabolic diseases). Conclusion The combination of extensive clinical examination with functional and ultrastructural analysis of the cilia results in a high degree of accuracy in diagnosing PCD.

Abstract: Purpose The purpose of this work was to evaluate the radiographic and serial high resolution CT (HRCT) findings in patients with nonspecific interstitial pneumonia/ fibrosis (NSIP). Method We identified 15 patients with biopsy-proven NSIP. Radiography and initial and follow-up CT findings were reviewed. Results Predominant radiographic findings were bilateral infiltrates distributing in the middle and lower lung zones and decreased lung volumes. At initial CT, predominant patterns were peribronchovascular interstitial thickening (n = 6), parenchymal bands (n = 8), intralobular interstitial thickening (n = 12), and traction bronchiectasis (n = 14). Mixed pattern of ground-glass opacity and consolidation (n = 11) were predominant findings of increased lung opacity. At follow-up CT in 14 cases, the abnormalities had disappeared completely in 3, improved in 9, persisted in 1, and worsened in 1. Conclusion The pulmonary abnormalities observed in NSIP on HRCT can disappear or be diminished in most cases after corticosteroid therapy. Intralobular interstitial thickening and traction bronchiectasis, which have been considered to be indicators of irreversible fibrosis, also show favorable responses.

Abstract: Two Hundred patients with bronchial asthma were studied to identify the prevalence of allergic bronchopulmonary aspergillosis (ABPA). The patients selected required intermittent short courses of steroids and their mean duration of illness was 12 years. Absolute eosinophil count was > 500/mm3 in 53% of the cases. Chest X-rays showed small homogenous shadows with patchy infiltrations in 25% and fluctuating pneumonic shadows in 14% of the cases. Raised specific IgG and positive serum precipitin against Aspergillus fumigatus (AF) were present in 24% and 13%, respectively. Cases with radiological and immunological suspicion were further investigated for ABPA. Skin tests for Type-I and Type-III reactivity were positive with AF extract in 87% (n = 47) and 36% (n = 47) of the cases. A thorax CT of 31 patients showed central bronchiectasis in 24 cases, labeling these patients as ABPA-CB (ABPA with central bronchiectasis) and an other 7 as ABPA-S (serological positive). CT was not done in one case who, because of other positive findings, was also labeled as ABPA-S. Thus, these 32 asthmatics were found to have ABPA. Among them, there was raised specific IgG (100%) and raised specifc IgE against AF (100%), positive skin test for Type-I and Type-III reactivity (100% and 53%) against AF. There was elevated total IgE (100%, n = 29), a positive family history of asthma (63%), peripheral eosinophilia (100%) and a history of passage of brownish plugs (31%). Radiological findings suggested soft shadow with infiltration in 31% and fluctuating pneumonic shadows in 69% of cases. CT Thorax (n = 31) showed central bronchiectasis in 78% of theses patients. Based on the present data, the prevalence of ABPA in bronchial asthma patients is 16% (12% with central bronchiectasis and 4% only serologically positive). Therefore, patients should be investigated and diagnosed in an early phase of ABPA (ABPA-S) and should be treated to prevent permanent lung damage.

Abstract: Objective: Recent surgical literature has highlighted the dangers of pneumonectomy for inflammatory lung disease; therefore the assessment of the risk/benefit ratio of our departmental policy. Methods: Patients undergoing pneumonectomy for inflammatory lung disease during two 2-year periods, 1991‐1992 and 1996‐1997 inclusive, were retrospectively analyzed. Clinical indications for investigation and surgery, and radiographic findings were determined. Some comparisons between the two periods were drawn. Rates of morbidity and mortality were the principle outcome measures. Results: One hundred and fifty-five patients, 116 males, 39 females, with an average age of 30.2 years ranging from 1‐68 years, underwent pneumonectomy for ongoing features of productive cough, haemoptysis (two emergencies) and chronic empyema all with either bronchographic or computed tomography (CT) evidence of destroyed lung. One hundred and fourteen (72%) had or had had tuberculosis at time of surgery. Histology showed bronchiectasis in 53 (34%), end-stage disease in 49 (31.6%) and active tuberculosis in 48 (30.9%). Over 90% of the patients were free of disease at discharge. Mortality was two (1.2%). Morbidity (23%) included post-pneumonectomy empyema 23 (14.8%), bleeding three (1.9%), broncho-pleural fistula three (1.9%), with wound sepsis in one (0.6%) and thoracic duct injury in one (0.6%). Three groups were identified, (1) pneumonectomy through empyema ‐ a risk group, (2) pneumonectomy in active tuberculosis and (3) pneumonectomy in children. Twenty-three post-pneumonectomy empyemas (PPE) occurred with 21 of these following pneumonectomies through empyema (PTE), six PPEs followed 27 PTEs for active tuberculosis. Fourteen of the 21 empyemas following pneumonectomy through empyema were initially sterilized. Finally 15/23 (65%) of all PPEs were sterilized. Pneumonectomy in active tuberculosis did not carry the mortality or morbidity experienced by others. Pneumonectomy in children was remarkably uncomplicated, with one PPE occurring. Conclusions: This ongoing study shows pneumonectomy for inflammatory lung to be safe, with good results. Tuberculosis, being so common, adequate pre-operative and operative cover with anti-tuberculosis drugs may enhance results. q 2000 Elsevier Science B.V. All rights reserved.

Abstract: The purpose of this paper was to quantitate the distribution and severity of computed tomography (CT) and radiographic findings in patients with allergic bronchopulmonary aspergillosis (ABPA), probable ABPA, and asthmatic controls. Chest radiographs and high-resolution CT images were evaluated in 19 patients with documented ABPA and 18 asthmatic controls. Ten patients with probable ABPA were also evaluated. On CT examination 17 patients (89%) with ABPA had central cystic or varicoid bronchiectasis in at least one lobe. One patient had no evidence for bronchiectasis. Three asthmatic patients (17%) had findings of cylindrical bronchiectasis. All 10 patients with probable ABPA had evidence of bronchiectasis on high-resolution CT (HRCT). The majority of patients with ABPA have diffuse disease at the time of diagnosis, manifested by central cystic and/or varicoid bronchiectasis in four or five lobes. Evaluation with HRCT can facilitate a diagnosis of ABPA and probable ABPA, allowing for earlier treatment which may prevent progression to fibrosis.

Abstract: Asthma is common among older persons, affecting approximately 4 to 8% of those above the age of 65 years. Despite its prevalence, late onset asthma may be misdiagnosed and inadequately treated, with important negative consequences for the patient's health. The histopathology of late onset disease appears to be similar to that of asthma in general, with persistent airway inflammation a characteristic feature. It is less clear, however, that allergic exposure and sensitisation play the same role in the development of disease in adults as they do in children. Atopy is less common among those with late onset asthma, and the prevalence of elevated immunoglobulin E levels is lower among those aged over 55 years of age than younger patients. Occupational asthma is an aetiological consideration unique to adult onset disease, with important implications for treatment. The differential diagnosis for cough, wheeze, and dyspnoea in the elderly is broad, and includes chronic obstructive bronchitis, bronchiectasis, congestive heart failure, lung cancer with endobronchial lesion and vocal cord dysfunction. Keys to accurate diagnosis include a good history and physical examination, the demonstration of reversible airways obstruction on pulmonary function tests and a favorable response to treatment. Inhaled corticosteroid therapy is recommended for patients with persistent disease, and careful instruction in the use of metered-dose inhalers is particularly important for the elderly.

Abstract: BACKGROUND—The purpose of this study was to describe findings on sequential high resolution computed tomographic (CT) scans of nine patients with non-specific interstitial pneumonia. METHODS—Thin section CT scans of nine patients with pathologically proven non-specific interstitial pneumonia were evaluated retrospectively. All patients underwent sequential CT scanning (mean follow up 3.1 years (range 1-8)). RESULTS—The predominant finding on the initial CT scans in seven patients was patchy areas of ground glass opacity in both the central and peripheral lung, with (n = 5) or without (n = 2) irregular areas of consolidation. In another two patients areas of consolidation in both the central and peripheral lung were seen as the predominant abnormality. The initial parenchymal abnormalities had resolved completely in four patients with predominant ground glass opacity without bronchiolectasis. Some of the bronchiectasis and bronchiolectasis resolved. In two patients bronchiectasis and bronchiolectasis occurred at one year and two years of follow up, respectively. In two patients with predominant consolidation the consolidation decreased but persisted, and in one patient the consolidation evolved into honeycombing. In the other patient bronchiectasis progressed over the course of seven years, forming varicoid bronchiectasis. CONCLUSION—Patients with non-specific interstitial pneumonia may recover completely after treatment with corticosteroids, but as many as half of these patients will have persistent pulmonary abnormalities on CT scans including bronchiectasis and honeycomb lung.

Abstract: Background: Pulmonary inflammation in bronchiectasis, pneumonia and idiopathic pulmonary fibrosis (IPF) is dominated by neutrophils. Pathophysiologic differences are seen in the deg

Abstract: Objective To determine clinical, radiographic, and pathologic features of bronchiectasis in cats. Design Retrospective study. Animals 12 cats with histologic evidence of bronchiectasis. Procedure Information on signalment, body weight, clinical signs of respiratory disease, concurrent diseases, method by which lung tissue specimens were obtained (surgical biopsy or necropsy), and histopathologic findings was obtained by reviewing medical records from January 1987 to June 1999 for cats with confirmation of bronchiectasis by histologic examination. Available thoracic radiographs were reviewed by a board-certified radiologist. Results Bronchiectasis was most commonly identified in older male cats. Clinical signs referable to the lower portion of the respiratory tract were detected in only 5 cats but, when evident, were usually chronic (duration > 1 year). Radiographic pattern of bronchiectasis was cylindrical in 4 cats, and in 1 of these cats, a saccular pattern was also identified. For most cats, bronchiectasis was detected in a single lung lobe. Radiographic evidence of bronchiectasis was not detected in 2 cats. Concurrent respiratory diseases included chronic bronchitis and bronchiolitis, neoplasia, bronchopneumonia, endogenous lipid pneumonia, and emphysema. Conclusions and clinical relevance Bronchiectasis appears to be an uncommon respiratory tract disorder that predominantly affects older male cats. Thoracic radiography may not be sensitive for the diagnosis of bronchiectasis in cats. Bronchiectasis in cats appears to be a sequela of chronic inflammatory bronchopulmonary diseases, especially chronic bronchitis, neoplasia, and bronchopneumonia.

Abstract: Adhesion molecules are expressed on the surface of endothelial cells and leukocytes and are responsible for mediating the migration of intravascular leukocytes into inflamed tissue. Intensive recruitment of neutrophils into the airways occurs in bronchiectasis, although little is known about the role of adhesion molecules in this process. The authors, therefore, determined serum levels of E-selectin, intercellular adhesion molecule (ICAM)-1 and vascular adhesion molecule (VCAM)-1 in stable bronchiectasis patients (n=37) and healthy control subjects (n=17), and evaluated their relationship with clinical markers of disease severity in bronchiectasis. Serum levels of E-selectin, ICAM-1 and VCAM-1 in bronchiectasis patients were significantly higher than those in control subjects (p=0.02, <0.0001 and 0.0002 respectively). Both E-selectin and ICAM-1 levels were inversely related to forced expiratory volume in one second (FEV1)% predicted (r=-0.57, p<0.001; and r=-0.53, p=0.001 respectively), and FVC% predicted (r=-0.52, p=0.002; and r=-0.46, p=0.005). This was not the case for VCAM-1 levels. There was a correlation between serum ICAM-1 levels and 24 h sputum volume (r=0.34, p= 0.04). Serum E-selectin and ICAM-1, but not VCAM-1, levels showed correlation with the number of lung lobes affected by bronchiectasis (r=0.35, p=0.04 and r=0.34, p=0.04 respectively). These original observations strongly suggest that E-selectin, intercellular adhesion molecule-1 and Vascular adhesion molecule-1 could play a significant role in the pathogenesis of bronchiectasis.

Abstract: Mycobacterium avium-intracellulare (MAI) pulmonary infection may occur in subjects with no preexisting lung disease and no known immunodeficiency, showing radiologically nodular bronchiectasis. There have remained some unresolved problems in the pathogenesis of the disorder, including the predominance in elderly women and the presence of not deteriorated or deteriorated disease. In the present study, we examined whether immunogenetic susceptibility is present in the disorder. We evaluated 64 cases of MAI disease and analyzed their short-term natural history by assessing symptoms, sputum bacteriology, and chest computed tomographic findings. The frequencies of human leukocyte antigen (HLA) alleles in patients were compared with those in 100 healthy Japanese control subjects. We assayed the HLA-A, -B, -C, -DR, and -DQ antigens serologically. Among 64 patients, 37 (35 females) did not show deterioration, whereas 27 (24 females) showed deterioration after an interval of 30 +/- 15 mo. There was no significant frequency of HLA-B and -C alleles in either group. In 37 not deteriorated patients, DR-6 was positive in 14 (37.8%) patients but in only 16 (16%) control subjects (p = 0.0061, odds ratio [OR] = 3.20). DQ-4 was positive in 10 (27.0%) patients but in only 10 (10%) control subjects (p = 0. 0122, OR = 3.33). In 27 deteriorated patients, HLA-A26 was positive in 14 (51.9%) patients but in only 21 (21.0%) control subjects (p = 0.0015, OR = 4.05). MAI pulmonary infection with nodular bronchiectasis shows two types of outcome, deteriorated and not deteriorated. The subjects with A-26 antigen might indicate the deterioration of MAI infection.

Abstract: Background Leukotriene receptor antagonists are a new class of drug that were initially identified for use in asthma. As they have an effect on neutrophil mediated inflammation, they may be of benefit in bronchiectasis. Objectives To determine whether leukotriene receptor antagonists have any additive benefit over and above conventional treatment for bronchiectasis (usually consisting of antibiotics and postural drainage). Search strategy The Cochrane Airways Group clinical trials register derived from MEDLINE, EMBASE and hand searching of major journals was searched using the terms:Bronchiec* AND leukotrien* OR anti-leuk* OR cysteinyl, Bronchiec* AND monteluk*, Bronchiec* AND zafirluk* Selection criteria Only randomised, controlled trials were considered Data collection and analysis The results of searches were analysed by both authors Main results No randomised, controlled trials were identified Reviewer's conclusions Further research is required to establish any benefit from the use of leukotriene antagonists in bronchiectasis.

Abstract: Airway inflammation in chronic obstructive pulmonary disease (COPD) is currently the subject of rapidly increasing research interest, investigating both the nature of the inflammatory cells and the cytokines present. The data are being used to define and assess the severity, cause, prognosis, and response to treatment of this disease. The collection of spontaneous sputum or the induction of sputum has been used to study inflammation in the larger airways. It remains unclear, however, how these data relate to the pathological processes at the site of airflow obstruction (the small airways and alveolar region). Nevertheless, even the interpretation of the results from the large airways depends on the many factors that will influence the inflammation. The initial process of sputum collection, sample processing, and the performance of assays in the biological fluids being tested may all influence the results. In addition, airways inflammation may be altered by the patient's clinical state, current treatment, and the nature of the disease (asthma, COPD, or bronchiectasis). Other factors including smoking, α1-antitrypsin (AAT) deficiency, and bacterial colonisation may also affect airways inflammation. Despite these many factors that may influence airways inflammation, many papers currently in the literature do not address them. The purpose of this review is to indicate the influence of these factors on airways inflammation to assist interpretation of the data currently in the literature. ### EOSINOPHIL Airway inflammation in asthma is classically related to the eosinophil. Eosinophil granules contain a variety of mediators capable of causing inflammatory damage to the airway epithelium including eosinophil peroxidase (EPO), major basic protein (MBP), eosinophil cationic protein (ECP), metalloproteinases, platelet activating factor, and cysteinyl leukotrienes.1 In addition, the immunological basis of asthma is distinct and reflected in the nature of the cytokines released into the airway. However, there is some overlap with COPD which may …

Abstract: Endothelin (ET)-1 has been suggested to promote neutrophil adhesion to endothelium, migration to inflamed areas, and release of elastase. ET-1 might therefore play a role in the pathogenesis of bronchiectasis, a chronic inflammatory and infective airway disease which is still poorly understood. Thirty five patients with stable bronchiectasis (20 females, mean age+/-SD 49.1+/-15.0 yrs) and 18 control subjects (8 females, 49.4+/-11.3 yrs) were recruited prospectively. The ET-1 levels in serum and sputum were measured by commercially available enzyme linked immunosorbent assay (ELISA) kits. Patients with Pseudomonas aeruginosa in their sputum had a significantly higher serum level of ET-1 (median 25.8, interquartile range 13-43.9 pg x mL(-1)) than patients without P. aeruginosa (0, 0-10.5 pg x mL(-1); p=0.0004) and healthy control subjects (4.6, 0-16.3 pg x mL(-1); p=0.002). However, patients with and without P. aeruginosa infection had no significant difference in sputum ET-1 level (p=0.15). There was no correlation between serum or sputum ET-1 levels with the serum and sputum levels of the interleukin (IL)-1beta, IL-8 and tumour necrosis factor (TNF)-alpha; the number of bronchiectasis lung lobes; and spirometry. Serum ET-1 level correlated with 24 h sputum volume for the bronchiectasis patients (r=0.51, p=0.002). The results, therefore, suggest a significant pathogenic role for endothelin-1 among Pseudomonas aeruginosa-infected patients with bronchiectasis. Further studies should be performed to evaluate the clinico-pathological correlation and expression of endothelin-1 in bronchiectasis.

Abstract: The yellow nail syndrome is characterized by slowly growing yellow discolored nails and lymphoedema, with onset generally after puberty. We report on a newborn infant who, at 23 weeks, was found to have hydrops on antenatal ultrasonography and bilateral chylothorax at delivery. His mother has the yellow nail syndrome, with typical nail changes, and bronchiectasis. There seemed to be no other etiology for the non-immune hydrops, and this is the second documented case of the prenatal manifestation of this condition.

Abstract: A 39-year-old woman with cystic bronchiectasis had repeated pulmonary infections from 1996 to 1999, and 6 of a total of 28 isolates of Escherichia coli from sputum specimens were studied. Their identical antibiotype and randomly amplified polymorphic DNA patterns indicated a single clone of E. coli, which persistently colonized the respiratory tract, causing recurrent infections.