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PubMed2005

New Zealand national incidence of bronchiectasis "too high" for a developed country.

AIMS: To prospectively estimate the incidence of bronchiectasis among New Zealand (NZ) children, to consider aetiology and severity, and to evaluate regional and ethnic variation. METHODOLOGY: NZ paediatricians were surveyed monthly for new cases of bronchiectasis during 2001 and 2002 via the NZ paediatric surveillance unit (with coverage of >94% of NZ paediatricians). Notified cases had their computed tomography scans reviewed and scored for severity. Confirmed cases were followed up by postal questionnaire one year after diagnosis. Demographic, aetiological, and severity data were collected. RESULTS: Ninety nine notifications were received. Sixty five cases were confirmed. An overall incidence of 3.7 per 100,000 under 15 year old children per year was estimated. Incidence was highest in Pacific children at 17.8 compared with 4.8 in Maori, 1.5 in NZ European, and 2.4 other per 100,000 per year. Incidence varied significantly by region. The median age at diagnosis was 5.2 years; the majority had symptoms for more than two years. Eighty three per cent had bilateral disease, with a median of three lobes affected, mean FEV1 of 77% predicted, and modified Bhalla HRCT score of 18. CONCLUSIONS: The incidence of bronchiectasis is high in NZ children, nearly twice the rate for cystic fibrosis and seven times that of Finland, the only other country reporting a childhood national rate. Incidence varied substantially between ethnicities. Most cases developed disease in early childhood and had delayed diagnosis.

Adolescent↗

PubMed2003

Management of bronchiectasis.

Around 1 in 1,000 adults in the UK has bronchiectasis, a potential outcome of various underlying lung diseases. It results from a 'vicious circle' of recurrent infection, inflammation, excessive mucus production, reduced mucociliary clearance, and dilatation and destruction of bronchi. Associated damage to blood vessels can lead to haemoptysis, which is usually slight, but may be severe. Bronchiectasis may be limited to one area (localised) or be more widespread (generalised). It can reduce lung function and quality of life, and may lead to respiratory failure and premature death. Here we review the management of patients with bronchiectasis.

Adult↗

PubMed2001

Anti-Pseudomonas aeruginosa antibody detection in patients with bronchiectasis without cystic fibrosis.

BACKGROUND: Pseudomonas aeruginosa is a frequent cause of infection in patients with bronchiectasis. Differentiation between non-infected patients and those with different degrees of P aeruginosa infection could influence the management and prognosis of these patients. The diagnostic usefulness of serum IgG antibodies against P aeruginosa outer membrane proteins was determined in patients with bronchiectasis without cystic fibrosis. METHODS: Fifty six patients were classified according to sputum culture into three groups: group A (n=18) with no P aeruginosa in any sample; group B (n=18) with P aeruginosa alternating with other microorganisms; and group C (n=20) with P aeruginosa in all sputum samples. Each patient had at least three sputum cultures in the 6 months prior to serum collection. Detection of antibodies was performed by Western blot and their presence against 20 protein bands (10-121 kd) was assessed. RESULTS: Antibodies to more than four bands in total or to five individual bands (36, 26, 22, 20 or 18 kd) differentiated group B from group A, while antibodies to a total of more than eight bands or to 10 individual bands (104, 69, 63, 56, 50, 44, 30, 25, 22, 13 kd) differentiated group C from group B. When discordant results between the total number of bands and the frequency of P aeruginosa isolation were obtained, the follow up of patients suggested that the former, in most cases, predicted chronic P aeruginosa colonisation. CONCLUSION: In patients with bronchiectasis the degree of P aeruginosa infection can be determined by the number and type of outer membrane protein bands indicating which serum antibodies are present.

Aged↗

PubMed2002

Randomised crossover study of the Flutter device and the active cycle of breathing technique in non-cystic fibrosis bronchiectasis.

BACKGROUND: Airway clearance techniques are an important part of the routine care of patients with bronchiectasis. The use of the Flutter, a hand held pipe-like device causing oscillating positive expiratory pressure within the airways, has been proposed as an alternative to more conventional airway clearance techniques. METHODS: A randomised crossover study was performed in 17 stable patients with non-cystic fibrosis bronchiectasis at home, in which 4 weeks of daily active cycle of breathing technique (ACBT) were compared with 4 weeks of daily physiotherapy with the Flutter device. RESULTS: No significant differences between the two techniques were found. Median weekly sputum weights were similar with a median treatment difference of 7.64 g (p=0.77) and there was no evidence of treatment order or order interaction effects (p=0.70). Health status (Chronic Respiratory Disease Questionnaire) and ventilatory function did not change significantly during either treatment period. There was no significant change in peak expiratory flow rate or in breathlessness (Borg score) after individual physiotherapy sessions with either technique. A questionnaire indicated subjectively that patients preferred the Flutter (11/17) to ACBT for routine use. CONCLUSIONS: Daily use of the Flutter device in the home is as effective as ACBT in patients with non-cystic fibrosis bronchiectasis, and has a high level of patient acceptability.

Bronchiectasis↗

PubMed2005

Exercise training and inspiratory muscle training in patients with bronchiectasis.

BACKGROUND: Bronchiectasis is a chronic suppurative lung disease often characterised by airflow obstruction and hyperinflation, and leading to decreased exercise tolerance and reduced health status. The role of pulmonary rehabilitation (PR) and inspiratory muscle training (IMT) has not been investigated in this group of patients. METHODS: Thirty two patients with idiopathic bronchiectasis were randomly allocated to one of three groups: PR plus sham IMT (PR-SHAM), PR plus targeted IMT (PR-IMT), or control. All patients (except the control group) underwent an 8 week training programme of either PR or PR plus targeted IMT. Exercise training during PR was performed three times weekly at 80% of the peak heart rate. IMT was performed at home for 15 minutes twice daily over the 8 week period. RESULTS: PR-SHAM and PR-IMT resulted in significant increases in the incremental shuttle walking test of 96.7 metres (95% confidence interval (CI) 59.6 to 133.7) and 124.5 metres (95% CI 63.2 to 185.9), respectively, and in endurance exercise capacity of 174.9% (95% CI 34.7 to 426.1) and 205.7% (95% CI 31.6 to 310.6). There were no statistically significant differences in the improvements in exercise between the two groups. Significant improvements in inspiratory muscle strength were also observed both in the PR-IMT group (21.4 cm H2O increase, 95% CI 9.3 to 33.4; p = 0.008) and the PR-SHAM group (12.0 cm H2O increase, 95% CI 1.1 to 22.9; p = 0.04), the magnitude of which were also similar (p = 0.220). Improvements in exercise capacity were maintained in the PR-IMT group 3 months after training, but not in the PR-SHAM group. CONCLUSION: PR is effective in improving exercise tolerance in bronchiectasis but there is no additional advantage of simultaneous IMT. IMT may, however, be important in the longevity of the training effects.

Breathing Exercises↗

PubMed1980

Lung crackles in bronchiectasis.

The inspiratory timing of lung crackles in patients with bronchiectasis was compared with the inspiratory timing of the lung crackles in chronic bronchitis and alveolitis. In severe obstructive chronic bronchitis the lung crackles are typically confined to early inspiration while in alveolitis the lung crackles continue to the end of inspiration but may begin in the early or the mid phase of inspiration. In uncomplicated bronchiectasis on the other hand, the lung crackles typically occur in the early and mid phase of inspiration, are more profuse, and usually fade by the end of inspiration. In addition in bronchiectasis, crackles are also usually present in expiration, they are gravity independent and become less profuse after coughing.

Adult↗

PubMed1985

Bronchiectasis and oligospermia: two families.

Five patients from two families had a bronchiectasis syndrome; the men also had oligospermia with poor progressive sperm motility. None of the patients had cystic fibrosis, alpha 1 antitrypsin deficiency, immunoglobulin deficiency, structural abnormalities of the bronchi, or abnormalities of ciliary ultrastructure. Onset of pulmonary symptoms was in the neonatal period or infancy, and bronchitis and bronchiectasis were most severe in the middle and lower lobes. Two patients had sinusitis and two otitis. The two men old enough to be studied had 2-10 X 10(6) sperm/ml of ejaculate with less than 10% progressively motile. There were no other associated anomalies. These patients probably had a distinct genetic error predisposing to pulmonary infection leading to bronchiectasis and, in men, oligospermia with poor progressive motility.

Adolescent↗

PubMed1989

The immunological component of the cellular inflammatory infiltrate in bronchiectasis.

Immunohistological analysis of bronchial biopsy specimens from nine patients with bronchiectasis and four control subjects was performed with a panel of monoclonal antibodies selected to show lymphocyte and macrophage subsets and signs of cellular activation. The cells taking part in the inflammatory response in the bronchial wall of patients with bronchiectasis were almost exclusively mononuclear cells, most of them T lymphocytes. B lymphocytes were observed in biopsy specimens from only two out of nine patients. CD8+ T cells outnumbered CD4+ cells in all patients in a ratio ranging from 2:1 to 10:1. Most T lymphocytes also strongly expressed CD7 antigen and a proportion of them expressed HLA-DR. Most of the lymphocytic infiltration occurred just beneath the basement membrane of the epithelium, though intraepithelial and submucosal infiltration was also seen. Non-lymphoid mononuclear cells expressing the phenotype of dendritic cells and macrophages were found dispersed throughout the infiltrate, most of them expressing HLA-DR. These observations support the hypothesis that cell mediated immunological reactions contribute to the inflammation associated with bronchiectasis.

Adult↗

PubMed1992

Sputum sol phase proteins and elastase activity in patients with clinically stable bronchiectasis.

BACKGROUND: Inflammatory and proteolytic activity occurs in sputum from patients with stable purulent bronchiectasis and has been proposed as the main pathogenetic mechanism of the disease. This study was designed to define further the role of inflammation and proteolysis in bronchiectasis. METHODS: Neutrophil elastase activity, sputum concentrations of the serum derived inhibitors alpha 1 antiproteinase and alpha 2 macroglobulin, and the sputum to serum ratios of albumin and C reactive protein concentration were measured in 26 patients with bronchiectasis. RESULTS: Free elastase activity was found in 15 sputum samples. A trend to higher proteolytic and inflammatory activity was found between mucoid and purulent sputum samples, suggesting that inflammatory and proteolytic activities are related to the macroscopic degree of purulence. Purulent sputum had a high sputum to serum ratio of C reactive protein, suggesting local production or active transport of this protein into bronchial secretions. C reactive protein was more sensitive than albumin in detecting a higher degree of inflammation in elastase positive samples. CONCLUSION: The finding of greater concentrations of alpha 2 macroglobulin in purulent and elastase positive samples than in mucopurulent, mucoid and elastase negative sputum samples suggests that this inhibitor may have a role in the proteolysis-antiproteolysis balance in bronchial secretions.

Albumins↗

PubMed1994

Localised pulmonary resection for bronchiectasis in hypogammaglobulinaemic patients.

BACKGROUND: Bronchiectasis and pulmonary infections are common in patients with hypogammaglobulinaemia. Despite intravenous gammaglobulin treatment and appropriate antibiotics, a subgroup of patients remains with persistent localised pulmonary infection in segments where bronchiectasis had developed before appropriate treatment. As such localised pulmonary suppuration (segmental or lobar) may serve as a focus for progression of bronchiectasis, surgical resection of the involved segments may be considered. The outcome of pulmonary resection in four such patients is reported. RESULTS: Surgery was well tolerated except for one postoperative empyema. Information on follow up is available from 3.5 to 5 years. All patients experienced considerable reduction of symptoms including cough, sputum production, antibiotic use, and hospital admissions. CONCLUSIONS: Surgical resection of localised bronchiectatic segments should be considered in patients with hypogammaglobulinaemia with persistent localised suppuration and symptoms refractory to medical treatment.

Adolescent↗

PubMed1998

Bronchiectasis following colectomy for Crohn's disease.

Bronchiectasis developing following colectomy for ulcerative colitis has been reported in a few cases. This may be the first report of bronchiectasis developing after colectomy for Crohn's disease. The close temporal relationship to colectomy, lack of bacterial pathogens in the sputum, and an impressive response to oral steroids suggest a difference in pathogenesis from idiopathic bronchiectasis.

Adult↗

PubMed1998

Hypertrophied bronchial artery at thin-section CT in patients with bronchiectasis: correlation with CT angiographic findings.

PURPOSE: To evaluate hypertrophied bronchial arteries on thin-section computed tomographic (CT) scans in patients with bronchiectasis by using CT angiographic correlation. MATERIALS AND METHODS: Spiral CT angiography was performed prospectively in 14 patients (eight men, six women; age range, 34-71 years) with bronchiectasis who were suspected of having bronchial arterial hypertrophy at thin-section CT (performed without contrast medium). The inclusion criteria were tubular (in six patients) or nodular (in 14 patients) areas of soft-tissue attenuation that had an appearance unlike that of lymph nodes at thin-section CT and that were within the mediastinum and around the central airway. These findings were subsequently correlated with the spiral CT angiographic findings. RESULTS: At comparative analysis of thin-section CT scans and CT angiograms, seven of the eight (88%) tubular lesions and 19 of the 36 (53%) nodular lesions in the mediastinal soft tissue were proved to be hypertrophied bronchial arteries. All of the six (100%) tubular and 19 of the 21 (90%) nodular lesions around the walls of the main (primary) and lobar bronchi were hypertrophied bronchial arteries. In eight (57%) patients, CT angiograms showed 11 intraluminal protrusions caused by hypertrophied bronchial arteries in the main bronchi, lobar bronchi, or both. CONCLUSION: Nodular and tubular structures in the mediastinum and around the central airway on thin-section CT scans in the patients with bronchiectasis are suggestive of hypertrophied bronchial arteries. Recognition of the hypertrophied bronchial artery can be critical for the bronchoscopist.

Adult↗

PubMed2005

[Nocardiosis and bronchiectasis. An uncommon association?].

OBJECTIVE: Nocardiosis is difficult to diagnose and infrequently associated with bronchiectasis or cystic fibrosis. This study analyzes the clinical and microbiological characteristics of 40 patients in whom microorganisms belonging to the Nocardia asteroides complex were isolated. METHODS: We studied 27 males and 13 females, with a mean age of 45 years (8-82). Nocardia spp. were isolated in 129 samples. Cultures were performed with conventional methods; additionally, when branching Gram-positive bacilli were visualized on microscopic examination, modified BCYE-alpha (m) medium was used. RESULTS: In 77.5% of the cases, investigation for Nocardia spp. was based on microscopy findings, in six patients it was a fortuitous finding in BCYE-alpha (m) medium, and in only three patients nocardiosis was clinically suspected. Twenty-five patients had nocardiosis (5 disseminated infection and 20 lung infection) and the remaining 15 were colonized. The most frequent risk factor in the infections, whether disseminated or not, was systemic glucocorticoid use (P 5 .001). The most frequent underlying pathology was bronchiectasis (80%), associated or not with cystic fibrosis. There were seven deaths and two patients had neurological sequelae. CONCLUSIONS: Bronchiectasis was an important risk factor for colonization by Nocardia spp. (P 5 0.01) in all the patients studied, and for infection (P 5 0.05) in patients without cystic fibrosis. The use of BCYE-alpha (m) medium in processing respiratory secretions highly facilitated the isolation of Nocardia spp.

Adolescent↗

PubMed1993

Multivariate analysis of factors affecting pulmonary function in bronchiectasis.

Impaired pulmonary function is of prognostic importance in bronchiectasis. To assess the factors affecting pulmonary function in bronchiectasis, we studied the clinical features, atopic status, bronchial responsiveness, systemic inflammatory indices, and sputum characteristics including volume, purulence, leucocyte count, neutrophil chemotactic activity, elastolytic activity (EA) and bacteriology in 82 Chinese patients. The majority of patients had impaired spirometry with airflow obstruction but normal carbon monoxide diffusing capacity. Multivariate analysis showed that factors significantly associated with worse lung function were: bronchial hyperresponsiveness, concomitant asthma, higher serum globulin, higher peripheral leucocyte count, lower serum albumin, greater sputum volume, diffuse disease and older age. When subjects with clinical asthma were excluded, similar factors were identified, and in addition, longer duration of disease, presence of eosinophils in sputum and higher sputum EA were significantly related to worse spirometry. Most of the identified factors reflect a state of inflammation. Thus the findings suggest that persistent inflammation plays an important role in the deterioration of lung function in bronchiectasis.

Bronchial Provocation Tests↗

PubMed1999

Inhalation of dry powder mannitol improves clearance of mucus in patients with bronchiectasis.

Bronchiectasis is a disease characterized by hypersecretion and retention of mucus requiring physical and pharmacologic treatment. Recently we reported that inhalation of dry powder mannitol markedly increases mucociliary clearance (MCC) in asthmatic and in healthy subjects (Daviskas, E., S. D. Anderson, J. D. Brannan, H. K. Chan, S. Eberl, and G. Bautovich. 1997. Inhalation of dry-powder mannitol increases mucociliary clearance. Eur. Respir. J. 10:2449-2454). In this study we investigated the effect of mannitol on MCC in patients with bronchiectasis. Eleven patients 40 to 62 yr of age inhaled mannitol (approximately 300 mg) from a Dinkihaler. MCC was measured over 90 min, in the supine position, on three occasions involving: mannitol or control or baseline, using a radioaerosol technique. On the control day patients reproduced the breathing maneuvers and the number of coughs induced by the mannitol. Mannitol significantly increased MCC over the 75 min from the start of the intervention compared with control and baseline in the whole right lung, central, and intermediate region. Mean (+/- SEM) clearance with mannitol was 34.0 +/- 5.0% versus 17.4 +/- 3.8% with control and 11.7 +/- 4.4% with baseline in the whole right lung (p < 0.0001). The mean number of coughs induced by mannitol was 49 +/- 11. In conclusion, inhalation of dry powder mannitol increased clearance of mucus and thus has the potential to benefit patients with bronchiectasis.

Administration, Inhalation↗

PubMed2000

An investigation into causative factors in patients with bronchiectasis.

Bronchiectasis is a pathologic description of lung damage characterized by inflamed and dilated thick-walled bronchi. These findings may result from a number of possible causes and these may influence treatment and prognosis. The aim of this study was to determine causative factors in 150 adults with bronchiectasis (56 male, 94 female) identified using high-resolution computerized tomography. Relevant factors were identified in the clinical history; cystic fibrosis gene mutation analysis was performed; humoral immune defects were determined by measuring immunoglobulins, IgG subclasses and functional response to Pneumovax II vaccine; assessment was made of neutrophil function (respiratory burst, adhesion molecule expression, and chemotaxis); ciliary function was observed and those likely to have allergic bronchopulmonary aspergillosis (ABPA) were identified. Causes identified were: immune defects (12 cases), cystic fibrosis (4), Young's syndrome (5), ciliary dysfunction (3), aspiration (6), panbronchiolitis (1), congenital defect (1), ABPA (11), rheumatoid arthritis (4), and early childhood pneumonia, pertussis, or measles (44). Intensive investigation of this population of patients with bronchiectasis led to identification of one or more causative factor in 47% of cases. In 22 patients (15%), the cause identified had implications for prognosis and treatment.

Adolescent↗

PubMed2002

Elastase-mediated phosphatidylserine receptor cleavage impairs apoptotic cell clearance in cystic fibrosis and bronchiectasis.

Cystic fibrosis is characterized by an early and sustained influx of inflammatory cells into the airways and by release of proteases. Resolution of inflammation is normally associated with the orderly removal of dying apoptotic inflammatory cells through cell recognition receptors, such as the phosphatidylserine receptor, CD36, and alpha v integrins. Accordingly, removal of apoptotic inflammatory cells may be impaired in persistent inflammatory responses such as that seen in cystic fibrosis airways. Examination of sputa from cystic fibrosis and non-cystic fibrosis bronchiectasis patients demonstrated an abundance of apoptotic cells, in excess of that seen in patients with chronic bronchitis. In vitro, cystic fibrosis and bronchiectasis airway fluid directly inhibited apoptotic cell removal by alveolar macrophages in a neutrophil elastase-dependent manner, suggesting that elastase may impair apoptotic cell clearance in vivo. Flow cytometry demonstrated that neutrophil elastase cleaved the phosphatidylserine receptor, but not CD36 or CD32 (Fc gamma RII). Cleavage of the phosphatidylserine receptor by neutrophil elastase specifically disrupted phagocytosis of apoptotic cells, implying a potential mechanism for delayed apoptotic cell clearance in vivo. Therefore, defective airway clearance of apoptotic cells in cystic fibrosis and bronchiectasis may be due to elastase-mediated cleavage of phosphatidylserine receptor on phagocytes and may contribute to ongoing airway inflammation.

Adolescent↗

PubMed2005

Impaired pneumococcal antibody response in bronchiectasis of unknown aetiology.

As a defective anti-polysaccharide response can exist in the absence of an immunoglobulin deficiency, a series of 26 patients with bronchiectasis of unknown aetiology was vaccinated with a 23-valent pneumococcal polysaccharide vaccine. All patients suffered from recurrent respiratory tract infections. When measuring total antibody levels to pneumococcal serotypes 3, 4 and 9, a normal polysaccharide antibody response was found in 22 patients. However, only 11 of these subjects showed a normal pneumococcal antibody response within the IgA and/or IgG2 subclass, and thus could be classified as true responders, while 15 patients did not respond in either the IgA class or in the IgG2 subclass. When analysing differences between the responder (n = 11) and nonresponder (n = 15) groups, the latter demonstrated higher frequencies of respiratory tract infections and more severe lung pathology, as revealed by the presence of more bronchi visualised in the peripheral third of the lung by high-resolution computed tomography scanning. Moreover, nonresponders needed extensive lung surgery more often in order to control their disease (number of resected segments eight versus five). In conclusion, an important fraction of patients presenting with idiopathic bronchiectasis is associated with a selective anti-polysaccharide response deficiency and this subgroup appears to represent a more severe clinical phenotype. Therefore, it can be regarded as a separate clinical entity with possible therapeutic targets. In order to identify IgA and IgG2 anti-polysaccharide nonresponders, all patients presenting with bronchiectasis of unknown aetiology should be immunised with a pneumococcal polysaccharide vaccine, and IgA and IgG2 isotype responses should be evaluated as well as the total antibody response.

Bronchiectasis↗