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PubMed2000

Bronchiectasis in Alaska Native children: causes and clinical courses.

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.

Adolescent↗

PubMed2003

Prolonged antibiotics for purulent bronchiectasis.

BACKGROUND: The vicious cycle hypothesis for bronchiectasis predicts that bacterial colonisation of the respiratory tract perpetuates inflammatory change. This damages the mucociliary escalator preventing bacterial clearance and allowing the persistence of pro-inflammatory mediators. Conventional treatment with physiotherapy and intermittent antibiotics are felt to improve the condition of bronchiectasis patients although there are no conclusive data showing that these interventions influence the natural history of the condition. Various strategies have been tried to interrupt this cycle of infection and inflammation and one of these is to prolong antibiotic treatment in the hope of allowing the airway mucosa to heal. OBJECTIVES: This systematic review brings together the evidence and where possible presents a meta-analysis of the data available to answer the question 'Does treatment with prolonged courses of antibiotics influence the outcome in purulent bronchiectasis?' SEARCH STRATEGY: The Cochrane Airways Group trials register and reference lists of identified articles were searched. SELECTION CRITERIA: Randomised trials looking at the use of prolonged antibiotic therapy in the treatment of bronchiectasis. DATA COLLECTION AND ANALYSIS: Trial quality was assessed and data extraction was carried out by the reviewers independently. Study authors were contacted for missing information. MAIN RESULTS: 447 abstracts were found and reviewed for suitability. Six trials were included and 302 patients were randomised amongst these trials. 40% of the patients were contributed by one trial. Antibiotics were given for between 4 weeks and one year. There were 40 withdrawals due to treatment failure and intolerable side effects. Only limited meta-analysis was possible due to the diversity of the trials. Response rates showed significant effects in favour of prolonged antibiotic treatment (Peto OR (95% CI), 3.37 (1.60 to 7.09)). Conversely for exacerbation rates there was no significant difference between prolonged antibiotics and placebo (Peto OR (95% CI), 0.96 (0.27 to 3.46)). For withdrawals there was no significant difference between treatment and placebo management (Peto OR (95% CI), 1.06 (0.42 to 2.65)). Data for lung function showed no significant benefit in favour of antibiotic treatment (% predicted FEV1, WMD (95% CI) -1.05 (-6.93 to 4.83)). REVIEWER'S CONCLUSIONS: The evidence available shows a small benefit for the use of prolonged antibiotics in the treatment of bronchiectasis. This review is limited by the diversity of the trials. Further randomised controlled trials with adequate power and standardised end points are required.

Adult↗

PubMed2001

Long-acting beta-2-agonists for bronchiectasis.

BACKGROUND: Symptoms of bronchiectasis include chronic productive cough, wheeze, breathlessness and recurrent infections of the lower respiratory tract. Long-acting bronchodilators are being used more frequently in the management of people with this condition. OBJECTIVES: To determine the effectiveness of long-acting bronchodilators in the management of bronchiectasis that is not due to cystic fibrosis. SEARCH STRATEGY: We searched the Cochrane Airways Group Bronchiectasis Database made up of trials identified from searching the Cochrane Controlled Trials Register, MEDLINE, EMBASE and hand-searches of leading respiratory journals. The latest searches were performed in May 2001. SELECTION CRITERIA: Randomised controlled trials, with or without masking. DATA COLLECTION AND ANALYSIS: The results of searches were reviewed against pre-specified criteria. MAIN RESULTS: We were unable to identify any randomised controlled trials investigating the effectiveness of long-acting bronchodilator therapy in the management of bronchiectasis. REVIEWER'S CONCLUSIONS: Further research is needed to establish if long-acting bronchodilators have a role in the management of people with bronchiectasis.

Adrenergic beta-Agonists↗

PubMed2001

Oral steroids for bronchiectasis (stable and acute exacerbations).

BACKGROUND: Inflammation plays a significant role in the pathophysiology of bronchiectasis. Two small studies have shown small benefits from inhaled corticosteroids and oral corticosteroids may be of benefit in bronchiectasis OBJECTIVES: To determine the efficacy of oral corticosteroids in acute and stable bronchiectasis SEARCH STRATEGY: The Cochrane Airways Group clinical trials register, derived from MEDLINE, EMBASE and hand searching of major journals, was searched using the terms bronchiectasis AND corticosteroid* OR (beclomethasone, cortisone, deflazacort, hydrocortisone, methylprednisolone, prednisolone, dexamethasone and triamcinolone SELECTION CRITERIA: Only randomised controlled trials were considered DATA COLLECTION AND ANALYSIS: No trials met the inclusion criteria for the review MAIN RESULTS: No randomised controlled trials were identified REVIEWER'S CONCLUSIONS: There are no randomised trials upon which to make recommendations about the use of oral corticosteroids in acute or stable bronchiectasis.

Acute Disease↗

PubMed2001

Oral methyl-xanthines for bronchiectasis.

BACKGROUND: Bronchiectasis is characterised by chronic sputum production,bronchial wall dilation,recurrent infection and airflow limitation. Methylxanthines are used in the management of airflow limitation associated with asthma and COPD, where they are also purported to have anti-inflammatory properties. In theory they may be of use in bronchiectasis. OBJECTIVES: To determine the efficacy of methylxanthines in the treatment of bronchiectasis. SEARCH STRATEGY: The Cochrane Airways Group clinical trials register derived from MEDLINE,EMBASE and hand searches using the terms bronchiectasis, aminophylline, theophylline and methyl- xanthine SELECTION CRITERIA: Only randomised controlled trials were to be considered. DATA COLLECTION AND ANALYSIS: The results of the searches were reviewed by two authors. Searches yielded seven trials none of which met the inclusion criteria. MAIN RESULTS: No randomised controlled trials were identified. REVIEWER'S CONCLUSIONS: Further research is required to establish if the methylxanthines have a role in the treatment of bronchiectasis.

Administration, Oral↗

PubMed2006

Inhaled hyperosmolar agents for bronchiectasis.

BACKGROUND: Mucus retention in the lungs is a prominent feature of bronchiectasis. The stagnant mucus becomes chronically colonised with bacteria, which elicit a host neutrophilic response. This fails to eliminate the bacteria, and the large concentration of host-derived protease may contribute to the airway damage. The sensation of retained mucus is itself a cause of suffering, and the failure to maintain airway sterility probably contributes to the frequent respiratory infections experienced by many patients. Hypertonic saline inhalation is known to accelerate tracheobronchial clearance in many conditions, probably by inducing a liquid flux into the airway surface, which alters mucus rheology in a way favourable to mucociliary clearance. Inhaled dry powder mannitol has a similar effect. Such agents are an attractive approach to the problem of mucostasis, and deserve further clinical evaluation. OBJECTIVES: To determine whether inhaled hyperosmolar substances are efficacious in the treatment of bronchiectasis SEARCH STRATEGY: The Cochrane Airways Group Specialised Register was searched, and leaders in the field were contacted. Searches were current as of October 2005. Search updates will be run annually. SELECTION CRITERIA: Any trial using hyperosmolar inhalation in patients with bronchiectasis not caused by cystic fibrosis. DATA COLLECTION AND ANALYSIS: Two reviewers assessed studies for suitability. MAIN RESULTS: Two small studies met the inclusion criteria of the review (28 participants). One study reported tracheobronchial clearance of a particulate radio aerosol after inhalation of dry mannitol on a single occasion, with appropriate control. Airway clearance doubled in the central and intermediate regions of the lung, but not in the peripheral region, after mannitol administration. No side effects were observed, but two patients were premedicated with nedocromil to prevent bronchospasm. Findings from one further trial indicated that one domain of a sensitive health status instrument showed a favourable response to mannitol. AUTHORS' CONCLUSIONS: Dry powder mannitol has been shown to improve tracheobronchial clearance in bronchiectasis, as well as cystic fibrosis, asthmatics, and normal subjects. Hypertonic saline has not been specifically tested in bronchiectasis, but improves clearance in these other conditions and in chronic bronchitis. The measurement of health status in one of the studies should be repeated in future longer term randomised controlled studies of mannitol and hypertonic saline. Consideration should also be given to exacerbations and symptom scores, as well as drug-related adverse events.

Bronchiectasis↗

PubMed1995

Presence, activities, and molecular forms of cathepsin G, elastase, alpha 1-antitrypsin, and alpha 1-antichymotrypsin in bronchiectasis.

The presence, activities, and molecular forms of the serine proteinases, elastase, and cathepsin G, and their endogenous inhibitors, alpha 1-antitrypsin and alpha 1-antichymotrypsin, were investigated in bronchoalveolar lavage (BAL) fluid of bronchiectasis patients divided into mild, moderate, and severe disease subgroups and compared to BAL fluid from healthy controls. Immunochemical characterization and quantitation were performed by Western immunoblot. The activities of elastase and cathepsin G were recorded spectrophotometrically using synthetic substrates. The results showed a significant difference in elastase and cathepsin G activities in BAL fluid of the three subgroups, revealing the following data--mild subgroup, 0.21 +/- 0.09 mU/g and 57.35 +/- 20.9 U/g; moderate subgroup, 1.87 +/- 1.12 mU/g and 89.24 +/- 31.4 U/g; and severe subgroup, 2.64 +/- 1.63 mU/g and 139.18 +/- 58.3 U/g, respectively--compared to those of the healthy control group, 0.09 +/- 0.03 mU/g and 50.96 +/- 16.5 U/g. Evidently, the protective shield of plasma-derived antiproteinases was sufficient in healthy subjects and, also, in mild cases of bronchiectasis, but not in cases of severe and moderate forms of bronchiectasis, in which free and catalytically active elastase and cathepsin G were detected. The serine proteinases inhibitors (serpins), alpha 1-antitrypsin and alpha 1-antichymotrypsin, have evidently been oxidized and/or proteolytically cleaved in the cases of moderate and severe bronchiectasis. The results indicate that insufficient endogenous downregulation of catalytically active elastase and cathepsin G in BALF leads to tissue injury, resulting in alterative and deformative processes in the bronchiectasis lung.

Adolescent↗

PubMed2000

Bronchiectasis: causes and management.

Bronchiectasis is a condition representing abnormal and permanent dilatation and distortion of medium sized bronchi, usually accompanied by destruction of the airway wall. Post inflammatory bronchiectasis remains very common in the developing countries as a sequel to pulmonary tuberculosis, whooping cough, and severe measles (among other causes). Cystic fibrosis is the most common cause of generalized bronchiectasis in developed countries. Symptoms primarily are chronic cough and expectoration of foul smelling sputum. Bronchography was, until recently, the investigation of choice for the diagnosis of bronchiectasis and the gold standard against which the current best imaging technique HRCT (high resolution computed tomography) has been compared. Treatment includes prompt attention to acute exacerbations, management of airway secretions and control of airway hyperreactivity. Treatment is aimed at the non progression of the disease and complete cure if possible. The role of surgical therapy has evolved from early curative resection for all patients to a more palliative approach. Patients with advanced generalized bronchiectasis should be considered for lung transplantation.

Anti-Bacterial Agents↗

PubMedDate not supplied

Clinical value of ciliary assessment in bronchiectasis.

Although ciliary dysfunction and numerous ultrastructural defects have been described, and these could be etiologically important in the development of bronchiectasis, their correlation with relevant clinical parameters have not been systematically evaluated. We have prospectively evaluated the prevalence and clinical significance of ciliary beat frequency and ultrastructural defects of nasal respiratory mucosa obtained from 152 stable patients with idiopathic bronchiectasis (100F, 57.7 +/- 15.2 yrs) and 127 control subjects (58F, 56.0 +/- 24.2 yrs). Bronchiectasis patients had significantly slower ciliary beat frequency (p < 0.05), and a greater percent of patients had central and peripheral microtubular defects (OR 14.4, 95% CI 5.6-36.8), namely, extra peripheral microtubules, "9 + 1", "8 + 2", and compound cilia (p < 0.05), but not microtubular disarrangement, extra matrix or ciliary tail abnormalities (p > 0.05), than controls. Bronchiectasis patients also had a greater proportion of cilia with any ultrastructural microtubular defects, compound cilia, and ciliary tails than controls (p < 0.05). Ciliary beat frequency did not correlate with clinically relevant parameters (p > 0.05). However, the percent of cilia with central, but not peripheral, microtubular defects correlated with 24 h sputum volume (r = 0.40, p = 0.001, and r = -0.04, p = 0.70, respectively) and FEV1 (r = -0.24, p = 0.01, and r = 0.00, p = 0.99 respectively). Our results strongly suggest a pathogenic role for central microtubular defects in the development of idiopathic bronchiectasis.

Adult↗

PubMed2005

Ciliary central microtubular orientation is of no clinical significance in bronchiectasis.

It has been suggested that patients with bronchiectasis might have increased central microtubular orientation angle (CMOA), which leads to poor coordination of ciliary beating, and consequently impairment of airway defence. We have employed transmission electron microscopy to assess CMOA of ciliated nasal mucosa in a cohort of 133 (81F, 56.8+/-16.1yr) stable bronchiectasis and 59 healthy subjects (30F, 49.3+/-22.1yr). There was no significant difference in CMOA between bronchiectasis (13.2 degree) and control subjects (13.0 degree, P=0.82). There was no significant difference in CMOA among patients according to the etiology of bronchiectasis, presence of nasal symptoms, or sputum status of Pseudomonas aeruginosa infection. Patients with more severe bronchiectasis, i.e. those with FEV(1) <60%, FVC <60%, or more than 4 bronchiectatic lung lobes, had significantly lower CMOA than their counterparts (P<0.05). There was no correlation between CMOA with age, 24h sputum volume, exacerbation frequency, FEV(1), FVC, or the number of bronchiectatic lung lobes (P>0.05). CMOA correlated with ciliary beat frequency (negative), and the percent of cilia showing ultrastructural or microtubular defects (P<0.05). Central microtubular orientation angle does not correlate with clinically important parameters, in contrary to the results reported by previously published smaller scale studies.

Adolescent↗

PubMed2006

Impact of bronchiectasis on right and left ventricular functions.

Diffuse systemic-pulmonary anastomoses and chronic hypoxemia may result in increase in ventricular work in bronchiectasis. We aimed to assess right ventricular (RV) and left ventricular (LV) functions in patients with bronchiectasis by using tissue Doppler-derived myocardial performance index (MPI), which is a novel and more sensitive parameter than conventional ventricular function parameters. To assess the possibility of RV and LV dysfunctions occurring in bronchiectasis, we studied 25 patients with bronchiectasis, and compared them with 22 age- and gender-matched control subjects. MPI, which is a combined index of both systolic and diastolic ventricular function, was calculated for both ventricles. RV and LV MPIs were also significantly different in patients and the controls. RV MPI was associated with the number of involved lobe, arterial blood oxygen pressure, and acceleration time/ejection time of pulmonary flow. LV MPI was not related to any clinical parameter, but it was correlated only with RV MPI. Ventricular functions are impaired in bronchiectasis. The impairment of RV function is related to involved lung lobe number, arterial oxygen pressure, and acceleration time/ejection time of pulmonary flow. LV dysfunction was correlated only with RV function.

Adult↗

PubMed2007

Aetiology in adult patients with bronchiectasis.

BACKGROUND: Bronchiectasis has a number of causes. Their prevalence is not well documented. The aim of this study was to identify aetiology in a population of patients referred to a specialist clinic with symptoms suggestive of bronchiectasis, to determine the proportion of patients in whom knowing the aetiology altered management. In addition we wished to describe in detail those patients who remained idiopathic to facilitate future studies of this group; and establish the diagnosis in those without bronchiectasis. METHODS: A total of 240 consecutive patients referred to the Royal Brompton Hospital with a history of recurrent chest infections, chronic cough and regular sputum production underwent a 3 day program of investigation. RESULTS: A total of 165 patients had bronchiectasis on CT scan, an underlying cause was identified in 122 (74%) and this affected management in 61 (37%). The common aetiologies were: post-infection (52), primary ciliary dyskinesia (17), allergic bronchopulmonary aspergillosis (13), and immune deficiency (11). Fourty-three patients had idiopathic bronchiectasis. They had symmetrical predominant lower lobe disease with onset of chronic chest and sinus symptoms in middle age. CONCLUSION: Full investigation of problematic cases should occur in a specialist centre because results affect management in a third of cases.

Adolescent↗

PubMed1995

Accuracy of CT in predicting the cause of bronchiectasis.

There are many reports that state that the distribution and pattern of bronchiectasis may be sufficiently characteristic for a specific cause to be suggested. The aim of this study was to determine whether experienced chest radiologists could confidently and accurately diagnose various aetiologies of bronchiectasis from the computed tomography (CT) pattern of disease alone. CT scans of 108 patients with bronchiectasis of various causes (67 with idiopathic bronchiectasis, 10 with allergic bronchopulmonary aspergillosis, 12 with syndromes of impaired mucociliary clearance, 12 with hypogammaglobulinaemia and seven with adult cystic fibrosis) were assessed by three chest radiologists without knowledge of clinical data. Each observer listed the three most likely diagnoses in order of probability. In addition, a level of confidence on a 3-point scale was assigned to the first choice diagnosis. A correct first-choice diagnosis was made in 45% of readings. A high confidence level was given in only 9% of the first choice readings. Of these, a correct diagnosis was reached in 35%. There was poor interobserver agreement (mean kappa = 0.20). In conclusion, we found that the causes of bronchiectasis cannot be reliably diagnosed on the basis of CT appearances alone.

Adult↗

PubMed1998

Bronchiectasis.

Imaging has a key role in the identification of bronchiectasis. Over the years, imaging tests have become increasingly accurate in depicting the features of early bronchiectasis. This article covers the clinical, pathologic, and functional features of bronchiectasis; high-resolution CT scan technique; the signs of bronchiectasis on chest radiography and high-resolution CT scan; and the causes of misdiagnosis of bronchiectasis.

Bronchi↗

PubMed2006

[Functional results of surgical treatment of bronchiectasis in a developing country].

BACKGROUND: The prevalence of bronchiectasis has decreased significantly over recent decades in developed countries. However, resection for bronchiectasis still plays an important part in thoracic surgery practice in developing countries such as Turkey. This study was designed to evaluate the outcomes of surgical treatment for bronchiectasis, particularly in aspects related to the effects on functional well-being. PATIENTS AND METHOD: From January 1995 through December 2003, operations for bronchiectasis were performed in 81 patients. Demographic features, type of resection, and operative morbidity and mortality were evaluated. The outcomes related to overall "social" or nonpulmonary functional status were classified and compared according to a scale constructed to assess patients' well-being preoperatively and at the 6th postoperative month. RESULTS: The mean age was 24.4 years and 47 patients (58%) were male. Surgical treatment was lobectomy in 37 (45%), pneumonectomy in 10 (12%), segmentectomy in 13 (16%), and lobectomy plus segmentectomy in 22 (27%) of the operations. Complete resection of disease was achieved in 69 patients (85%). There was no operative mortality. The rate of morbidity was 18.3%. Improvement to a functional status of excellent was observed in 81.7% and improvement to a status of good was seen in 12.7% of patients; 5.6% experienced no change. The results of complete resection were significantly better than those of incomplete resection (P=.0015). CONCLUSION: Functional results of surgical treatment for bronchiectasis in this series suggest that the outcomes are favorable and promising, particularly in selected patients with sufficient pulmonary reserves and localized disease who are suitable for complete resection.

Adolescent↗

PubMed2002

Humoral immunity and bronchiectasis.

Bronchiectasis is a common complication of primary antibody deficiency but the incidence of antibody deficiency as an underlying cause of bronchiectasis is largely undefined. In this study the humoral immune status of a cohort of bronchiectatic patients was investigated to detect the frequency of significant antibody deficiency and to determine the extent of immunological investigation which is appropriate for routine assessment of bronchiectasis patients. Fifty-six out-patients (with a mean age of 59.6 years) had serum immunoglobulins, IgG subclasses and specific antibodies to capsular polysaccharides of Haemophilus influenzae and Streptococcus pneumoniae measured. Where specific antibody -levels were low, where possible, appropriate immunization with pneumococcal or conjugated Haemophilus polysaccharide vaccines was offered and the responses quantified. Three of 56 patients had low total serum IgG levels. Thirteen of 56 had deficiencies of either a single IgG subclass or combinations of two or more subclasses, with IgG4 being most frequently implicated (9/56). Twenty-nine of 56 had low basal specific polysaccharide antibody levels. Test immunization, where performed, produced satisfactory responses in all cases except one, where a specific defect of responsiveness to pneumococcal polysaccharide was identified. This study indicates that antibody deficiency is an uncommon aetiological/underlying factor in the causation of bronchiectasis beyond the fourth decade and that detailed investigation of humoral immune status as a routine in bronchiectasis patients, at least at this age, is not generally justified.

Adult↗

PubMed1999

Bronchodilator response to inhaled beta-2 agonist and anticholinergic drugs in patients with bronchiectasis.

OBJECTIVE: An increase in incidence of reversible airflow obstruction and bronchial hyperresponsiveness occurs in patients with bronchiectasis. We conducted a study to assess the efficacy of bronchodilators in the treatment of bronchiectasis. METHODOLOGY: Twenty-four patients with confirmed bronchiectasis were studied. Each patient inhaled fenoterol 400 microg administered by metered dose inhaler via a spacer after a baseline lung function and a lung function test was repeated 30 min later. This was followed by a second dose of fenoterol 5 mg via nebulizer and another lung function test 30 min later. A repeat study was done at least 24 h later with ipratropium bromide 40 microg by metered dose inhaler and 500 microg by a nebulizer. RESULTS: The results showed a significant improvement from baselines (mean percentage change +/- SD) of peak expiratory flow rate (PEF) by 8.5 +/- 8.72% and 15.3 +/- 11.63%, forced expiratory volume in 1 s (FEV1) by 8.77 +/- 9.69% and 10.2 +/- 12.2% and forced vital capacity (FVC) by 10.25 +/- 11.61% and 10.09 +/- 10.88% after low- and high-dose fenoterol, respectively. The improvements after low- and high-dose ipratropium bromide for PEE FEV1 and FVC were 9.89 +/- 9.35% and 14.39 +/- 12.82%, 9.38 +/- 10.41% and 13.52 +/- 17.09%, and 8.03 +/- 10.85% and 9.63 +/- 13.85%, respectively. Eleven patients (45.8%) responded to one or both bronchodilators significantly (> 15% improvement in FEV1). Five patients (20%) responded to both, three (12%) to fenoterol alone and another three (12%) to ipratropium bromide alone. CONCLUSION: There is significant bronchodilator response in a subset of patients with bronchiectasis and patients with bronchiectasis should therefore undergo bronchodilator testing. Skin prick testing against a panel of nine allergens done on each individual yielded a positive result in 13 patients (54.2%).

Administration, Inhalation↗

PubMed2002

Regression of bilateral bronchiectasis with inhaled steroid therapy.

Bronchiectasis is defined as pathological and permanent dilatation of the bronchial tree. Affected patients suffer from chronic sputum production and usually slowly progressive airway destruction as a result of continued airway infection and inflammation. Regression of bilateral bronchiectasis has never been reported in the English literature. We report the case of a 60-year-old woman with longstanding progressive idiopathic bilateral bronchiectasis whose respiratory symptoms, including sputum, rapidly disappeared after commencement of inhaled budesonide. Repeat computed tomography assessment 40 months after commencement of inhaled steroid therapy, showed partial regression of bronchial dilation and resolution of small airways sepsis. In the absence of other possible explanations for the partial resolution of the bronchiectasis, the present case suggests a possible benefit of inhaled steroid therapy in bronchiectasis.

Administration, Inhalation↗