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Evaluation of the incidence and age distribution of bronchiectasis from the Finnish hospital discharge register.

The incidence and age distribution of bronchiectasis is at present poorly known. The aim here was to describe the use made of hospital services by bronchiectasis patients in Finland over ten years and to estimate the incidence of this disease. Data on hospital admissions for bronchiectasis were obtained from the register maintained by the National Research and Development Centre for Welfare and Health. Admissions and new cases during the period 1983-1992 were calculated by age and sex in relation to the total population. There were 5,710 admissions for bronchiectasis and 1,928 new cases. The occurrence of cases treated in hospital was 4.9 per million person-years at age 0-14 years, 103.8 at 65 years or over and 38.9 in the total population. The estimated incidence of bronchiectasis is low in Finland, especially in childhood, and efforts aimed at its treatment should now be directed specifically at the elderly population, which continues to exert considerable pressure on even a relatively well developed health service in this respect.

Adolescent↗

[Primary ciliary dyskinesia in situs inversus without bronchiectasis].

BACKGROUND: In adults suffering from Kartagener's syndrome-which is found in 50% of patients with primary ciliary dyskinesia (PCD)-bronchiectasis is still one of three typical clinical features. In this condition it is caused by chronic bacterial inflammation as a result of impaired mucociliary clearance in congenital ciliary dysfunction. Little information is available on the incidence, age-related development and prophylactic therapy of bronchiectasis in children suffering from PCD. CASE REPORT/RESULTS: We describe the case of a 2-year old boy with clinical features of Kartagener's syndrome who showed impaired ciliary motility and typical ultrastructural defects of PCD. Bronchiectasis was excluded by bronchography. CONCLUSIONS: The diagnosis of PCD implies disturbed ciliary motility and abnormal ultrastructure of the cilia. In 50% of cases PCD is associated with situs inversus and is then referred to as kartagener's syndrome, whereas situs inversus itself does not define Kartagener's syndrome. Bronchiectasis is not necessarily present in children with PCD. Physiotherapy, inhalations, vaccinations and early antibiotic treatment may be of prophylactic value in preventing bronchiectasis. Early start of the life-long treatment depends on early diagnosis which should be based on well-defined criteria.

Aging↗

Inhaled steroids for bronchiectasis.

BACKGROUND: Bronchiectasis is a progressive condition characterised by irreversible destruction and dilatation of airways, generally associated with chronic bacterial infection. The two distinct therapeutic goals are: symptom control and reduction in morbidity; and prevention of progression of the underlying disease. OBJECTIVES: To determine whether regular inhaled corticosteroids produce improvement in symptom control and whether they beneficially influence the natural history of the disease. SEARCH STRATEGY: The Cochrane Airways Group RCT register and Cochrane Controlled Clinical Trials Register were searched using the following search terms; bronchiectasis AND [corticosteroid* OR beclomethasone OR budesonide OR fluticasone OR triamcinolone OR flunisolide]. Bibliographies of each included RCT was searched for additional trials. Pharmaceutical companies that manufacture inhaled corticosteroids were also contacted. SELECTION CRITERIA: Only randomised double blind studies controlled trials were included. Patients with radiographic evidence of bronchiectasis were included, but patients with cystic fibrosis were excluded. DATA COLLECTION AND ANALYSIS: Data was extracted by one of the reviewers (FR). Continuous outcomes were analysed as effect sizes (weighted mean difference or as standardised mean difference with 95% confidence intervals). MAIN RESULTS: Only two trials on a total of 54 patients could be included. The studies were of 4 and 6 weeks duration. Inhaled corticosteroids had no significant effect on any of the outcomes included in this review, however there was a trend towards improving: FEV1, FVC, PEFR, RV and DLco. REVIEWER'S CONCLUSIONS: In bronchiectasis, regular use of inhaled corticosteroids may improve lung function. The available studies were too short and too small to provide any clear evidence to guide practice. Larger and longer studies should include rate of decline of lung function, exacerbation frequency, hospitalisations and healthy status as outcomes.

Administration, Inhalation↗

Anticholinergic therapy for bronchiectasis.

BACKGROUND: Anticholinergic agents block bronchoconstriction mediated by the vagus nerve and may also dry up bronchial secretions. They are effective in obstructive airways disease and may be beneficial in bronchiectasis OBJECTIVES: To determine the effect of anticholinergic therapy in acute exacerbations and stable bronchiectasis. SEARCH STRATEGY: The Cochrane Airways Group clinical trials register was searched using the terms bronchiectasis AND anticholinergic OR ipratropium bromide OR tiotropium OR atropine. SELECTION CRITERIA: Only randomised controlled trials were considered. DATA COLLECTION AND ANALYSIS: Two reviewers assessed the retrieved studies working independently. MAIN RESULTS: Twelve studies were identified, of which six were obtained for further scrutiny. One was translated from Italian. None met the inclusion criteria. REVIEWER'S CONCLUSIONS: No formal recommendations can be made about the use of anticholinergic therapy in acute or stable bronchiectasis based on the literature currently available.

Acute Disease↗

Surgery for non-localized bronchiectasis.

BACKGROUND: Resection for localized bronchiectasis is a well established therapy. However, there is little information on the role of surgery in non-localized bronchiectasis. METHODS: Between January 1992 and April 2001, 55 patients without cystic fibrosis underwent resection. Forty-eight patients (mean age 45 (range 23-74) years; 32 women) were available for long-term follow-up. Twenty-five patients underwent resection for localized disease (group 1) and 23 had bronchiectasis in at least two different lobes (group 2). RESULTS: Thirty-one of the 48 patients were treated by Video Assisted Thoracoscopic Surgery (VATS) resection. There was no 30-day mortality. Mean duration of hospital stay was 10.9 (range 6-31) days in group 1 and 11.1 (range 5-19) days in group 2. Three of 25 patients in group 1 required reoperation. Only minor complications occurred in group 2 (three patients). Mean follow-up for both groups was 37 (range 6-97) months. Twenty-three of 25 patients in group 1 and 16 of 23 in group 2 reported satisfaction at 6 months after the operation. Recurrent infection was noted in three patients in each group. Haemoptysis recurred in only one patient in group 2. CONCLUSION: The surgical treatment of selected patients with non-localized bronchiectasis was safe and most patients were satisfied with the outcome.

Adult↗

Nasal or oral oil application on infants: a possible risk factor for adult bronchiectasis.

Lipid pneumonia has been observed in infants exposed by inhalation of oil or butter applied into the nose or throat as part of an old custom. We performed a case-control study to test the hypothesis, that this ancient tradition may be a predisposing factor to bronchiectasis. A case group of 59 patients with bronchiectasis and three control groups -46 patients with COPD, 32 asthmatics, and 71 healthy Saudis-were questioned about possible risk factors of bronchiectasis including nasal or oral application of oil/butter at infancy. The risk of witnessed exposure to this old folk remedy was significantly higher among the cases than the controls (OR = 3.9 (1.7-8.8), (95% confidence interval) p < 0.001). Application of oil or butter into the nasal or oral cavity of infants may be a risk factor for bronchiectasis.

Administration, Intranasal↗

High-resolution CT in the assessment of bronchiectasis in children.

To assess the possibilities and limitations of high-resolution CT (HRCT) in the evaluation of bronchiectasis in children, we conducted a prospective study of 20 children with clinical and/or chest film findings suggestive of this diagnosis. The 2-mm collimation, 4.3-s HRCT scans with 10 mm interslice spacing were obtained in areas of suspected bronchiectasis; in nonsuspect areas 25-30 mm interslice spacing was used. No preparation for examination was required. Bronchiectasis was revealed in ten patients (50%), being bilateral in four cases and unilateral in six cases. All types of bronchiectatic patterns were found. Cooperation during the examination was the only difference when compared with an investigation of adults. It was not a serious problem in children aged 7 years and older; scans in 6-year-old children were diagnostic but not ideal. Nondiagnostic scans were obtained in a 3-year-old girl. At the time of the scans only one patient had undergone surgery. Preoperative bronchography confirmed the CT findings. The authors conclude that HRCT can limit the need for bronchography in children with a CT finding of focal bronchiectasis in whom surgery is contemplated. When using longer scanning times it is not possible to obtain good results without sedation of children younger than 6 years.

Adolescent↗

Reduced exercise capacity in non-cystic fibrosis bronchiectasis.

OBJECTIVE: Bronchiectasis not due to cystic fibrosis is usually a consequence of severe bacterial or tuberculous infection of the lungs, which is commonly seen in children in developing countries. Our aim was to study its functional sequelae and affect on work capacity in children. METHODS: Seventeen children (7-17 years of age) with clinical and radiological evidence of bronchiectasis of one or both lungs were studied at the Cardiopulmonary Unit of the Tuberculosis Research Centre. Pulmonary function tests including spirometry and lung volume measurements were performed. Incremental exercise stress test was done on a treadmill, and ventilatory and cardiac parameters were monitored. Control values were taken from a previous study. RESULTS: Children with bronchiectasis had lower forced vital capacity (FVC) (1.1 + 0.4 L versus 1.5 + 0.4 L, p = 0.003) and FEV1 (0.95 +/- 0.2 L versus 1.4 +/- 0.3 L, p < 0.002) compared to age- and sex-matched healthy controls. The patient group had significantly higher residual lung volumes (0.7 +/- 0.3 L versus 0.4 + 0.1 L, p < 0.02). At maximal exercise, they had lower aerobic capacity (28 +/- 6 ml/min/kg versus 38 +/- 5 ml/min/kg, p < 0.0001) and maximal ventilation (24 +/- 8 L/min versus 39 +/- 10 L/min, p < 0.001). At maximal exercise, while none of the controls desaturated, oxygen saturation fell below 88% in eight of 17 patients. CONCLUSION: The findings show that children and adolescents with non-cystic fibrosis bronchiectasis have abnormal pulmonary function and reduced exercise capacity. This is likely to interfere with their life as well as future work capacity. Efforts should be made to minimize lung damage in childhood by ensuring early diagnosis and instituting appropriate treatment of respiratory infections.

Adolescent↗

Surgical management of bronchiectasis.

BACKGROUND: The prevalence of bronchiectasis has decreased significantly over recent decades. We reviewed the morbidity, mortality, and outcome of surgical treatment for pulmonary bronchiectasis in the modern era of antibiotic therapy. METHODS: From January 1976 through January 1993, 134 patients (55 male and 79 female patients) underwent pulmonary resection for bronchiectasis. The mean age was 48.4 years (range, 4 to 89 years). The indication for operation was failure of medical therapy in 85 patients (63.4%), hemoptysis in 26 (19.4%), lung abscess in 12 (9.0%), and a nondiagnostic mass in 11 (8.2%). Mean duration of symptoms was 6 years (range, 1 to 60 years) and included a productive cough in 104 patients, fetid sputum in 91, recurrent infections in 81, and hemoptysis in 56. Thirteen patients (9.7%) had no prior symptoms and presented with either hemoptysis, lung abscess, or unresolved pneumonia. The disease was bilateral in 26 patients (19.4%) and mainly confined to the lower lobe in 75 (56.0%). The mean number of segments involved was 4.4 (range, one to 14 segments). Surgical treatment included lobectomy in 86 patients (64.2%), pneumonectomy in 21 (15.7%), wedge resection or segmentectomy in 18 (13.4%), and a combination of these approaches in 9 (6.7%). Disease was considered completely resected in 108 patients (80.6%). RESULTS: The operative mortality rate was 2.2% and the morbidity rate was 24.6%. The mean follow-up in 103 patients was 6 years (range, 1 to 16 years). Overall, 61 patients (59.2%) were asymptomatic after operation. Symptoms were improved in 30 patients (29.1%) and unchanged in 12 (11.7%). Complete resection resulted in a significantly better result than incomplete resection (p < 0.05). CONCLUSIONS: Pulmonary resection for bronchiectasis can be done with low mortality and morbidity. When possible, complete resection should be performed.

Adolescent↗

Computed tomography of bronchiectasis in association with tuberculosis.

Eight patients with pulmonary tuberculosis proven by positive sputum culture were found to have various degrees of bronchiectasis by computed tomography (CT) but not by plain chest radiography. The CT is characterized by cystic dilatation of the thick-walled bronchi manifested as strings or clusters of pearls or grapes for cystic bronchiectasis and a "tram-line" appearance for cylindrical bronchiectasis. We stress that bronchiectasis remains quite common in association with pulmonary tuberculosis, and CT can replace bronchography for its detection.

Adult↗

Effect of azithromycin on bronchiectasis and pulmonary function in a heart-lung transplant patient with severe chronic allograft dysfunction: a case report.

Azithromycin has been shown to be beneficial in several diseases with chronic neutrophilic inflammation of the airways, such as cystic fibrosis and bronchiolitis obliterans syndrome (BOS) after lung transplantation. Up to now, however, its healing effect on bronchiectasis has never been demonstrated. We report a heart-lung transplant patient who developed chronic rejection (BOS stage 3) with the appearance of gross bronchiectasis on a spiral computed tomography (CT) chest scan. Within 2 weeks after starting azithromycin, the patient's forced expiratory volume in 1 second increased significantly and a repeat spiral CT chest scan 5 months later, showed a major improvement of the bronchiectasis. This case report illustrates that bronchiectasis may greatly improve after treatment with azithromycin and no longer needs to be considered an endstage finding in patients with severe BOS.

Adolescent↗

In vitro study of regulation of IL-6 production in bronchiectasis.

Persistent airway inflammation is an important pathogenetic factor in bronchiectasis, and interleukin (IL)-6 is among the mediators implicated in regulation of inflammation in bronchiectatic airways. We postulated that airway secretion with its constituents of cytokines and enzymes would provide an environment for perpetuation of inflammation in vivo. We aimed to determine the action of sputum from patients with bronchiectasis on IL-6 production from cultured normal human bronchial epithelial (NHBE) cells and its modulation by anti-inflammatory drugs in vitro. Cultures of NHBE cells were tested with (i) sputum of bronchiectatic patients, (ii) anti-tumor necrosis factor-alpha (TNF-alpha) pre-treated sputum, or (iii) recombinant human (rh)-TNF-alpha. Alternatively, NHBE cells were incubated with one of the anti-inflammatory drugs before treatment with sputum or rh-TNF-alpha. IL-6 produced into the medium was assayed by ELISA. Sputum in bronchiectasis stimulated IL-6 production from NHBE cells by 1.9 times. This was largely attributable to TNF-alpha as pre-incubation of sputum sol with anti-TNF-alpha almost neutralized the sputum effect. Apart from dexamethasone, the other drugs exerted inhibitory effects on IL-6 production. Ibuprofen suppressed sputum-stimulated IL-6 production to levels above control and effect levelled off at 50-100 microg/mi, contrasting the dose-dependent suppression to control level with MK-663 (0.1-10 microg/ml) and to sub-control levels with triptolide (20-1000 ng/ml). Our results support that sputum in bronchiectasis can stimulate IL-6 production from NHBE cells, and TNF-alpha is an important cytokine mediating the process. The suppressive effects observed with ibuprofen, triptolide and MK-663 warrant further study.

Anti-Inflammatory Agents↗

Inhaled steroids improve quality of life in patients with steady-state bronchiectasis.

BACKGROUND: The effects of inhaled steroids upon the quality of life of patients with bronchiectasis remain unknown. STUDY OBJECTIVE: To analyze the effect of inhaled fluticasone propionate (FP) for 6 months upon the clinical, functional, microbiological and outcome parameters of patients with steady-state bronchiectasis not due to cystic fibrosis, and its repercussions for patient health-related quality of life (HRQoL). DESIGN: Prospective, randomized, double-blind (for effective doses) study. PATIENTS AND INTERVENTIONS: The diagnosis of bronchiectasis was made by high-resolution computed tomography. Ninety-three patients (mean age: 68.5 [8.4]) were randomized to receive 250 microg bid, 500 microg bid or no treatment with inhaled FP for 6 months. Data were collected at baseline and at 1, 3 and 6 months after the start of treatment. HRQoL was assessed using the validated Spanish version of the St. George's Respiratory Questionnaire. RESULTS: The group administered FP 1000 microg daily showed significant improvement in dyspnea (1.03 [2.1]-1.24 [2.2] points; P = 0.01-0.04), sputum production (P = 0.001), days without cough (P = 0.02) and short-acting beta-2 agonists used (P = 0.01) from the first month of treatment, with no changes in pulmonary function, number or severity of exacerbations, or microbiological profile of the sputum. As a result, an improvement in HRQoL was seen in this group after 3 months of treatment (45.4 [14.2] vs. 40.5 [13.9]; P = 0.01). CONCLUSIONS: Inhalatory FP 500 microg bid is effective from the first month of treatment for controlling the symptoms of patients with steady-state bronchiectasis-thus ensuring a significant improvement in HRQoL.

Administration, Inhalation↗

The association of tracheomegaly and bronchiectasis.

This study set out to determine the frequency of tracheomegaly in brochiectasis as judged by high resolution computed tomography (HRCT) while determining whether the published normal tracheal dimensions derived from radiographic data can be used for CT diagnosis. Seventy-five consecutive adults referred for CT assessment of possible bronchiectasis were studied and compared with a control group of 75 adults being staged for lymphoma. The internal tracheal diameters at aortic arch level of the control group correspond with published radiographic data and using these measurements, 7/42 (17%) patients with bronchiectasis were found to have tracheomegaly, while two of the 33 'symptomatic' patients (i.e. those patients not found to have bronchiectasis) had tracheomegaly. Further analysis confirmed that the bronchiectatic group's tracheal dimensions were significantly different from those of the control group while the 'symptomatic' group are an overlap population. We conclude that tracheomegaly is a frequent finding in bronchiectasis.

Adolescent↗

Indium-111-labelled granulocyte accumulation in respiratory tract of patients with bronchiectasis.

18 patients with severe bronchiectasis and persistent purulent sputum production were investigated, by the use of gamma scans, between acute exacerbations of their symptoms to determine localisation and traffic of 111In-labelled granulocytes in the lungs. Labelled granulocytes appeared in the respiratory tract of 16 patients within 24 h of their injection. Uptake occurred almost exclusively in areas demonstrated by computed tomography (CT) to be bronchiectatic. However, 111In uptake was not demonstrated in 21 of the 55 segments showing bronchiectasis on CT, and these were therefore thought to be not actively inflamed. The whole-body loss of radioactivity up to 4-7 days following injection of the labelled cells ranged from 0 to 53% and correlated with 24 h purulent sputum volume (r = 0.45, p less than 0.01) and with CT extent of moderate and severe bronchiectasis (r = 0.40, p less than 0.05), but not with overall CT extent of bronchiectasis, age, forced expiratory volume in one second, or duration of purulent sputum production in years.

Adult↗

Alpha 1-antitrypsin serum levels in widespread bronchiectasis.

This study found significantly higher serum levels of alpha 1-antitrypsin (alpha 1-AT) in 35 patients with widespread bronchiectasis compared with healthy controls. The results are in contradiction with a previous study and four case reports which found an association between alpha 1-AT deficiency and bronchiectasis. However, the validity of the association has been questioned in the literature on the grounds that most of the patients in those reports had other possible aetiologies for their bronchiectasis. We believe the raised level of alpha 1-AT in our patients represents a non-specific acute phase response to bronchial infection. Further studies are needed to clarify whether bronchiectasis, like emphysema, can be a manifestation of alpha 1-AT deficiency.

Adolescent↗

Bronchiectasis in Finland: trends in hospital treatment.

The incidence of bronchiectasis has probably declined in developed countries in recent years, but no reliable statistical data on this are available. The present paper describes the use made of hospital services by bronchiectatic patients in Finland. Data on a total of 12,539 treatment periods for bronchiectasis that had occurred between 1972 and 1992 were collected from the discharge register maintained by the National Research and Development Centre for Welfare and Health (diagnosis 518 in the International Classification of Diseases up to 1986, and 494 from 1987 onwards). The number of admissions, new occurrences of bronchiectasis and days in hospital were calculated by sex and age in relation to the total population at the end of each year. There were 143 and 87 admissions per million inhabitants in 1972 and 1992, respectively. The admissions, new occurrences and the days in hospital all decreased, at annual rates of 1.3, 4.2 and 5.7%, respectively. Thus, where the number of new occurrences was 50 per million persons in 1977, it was 27 per million in 1992. In summary, bronchiectasis-related hospital treatment declined markedly between 1972 and 1992. Trend is attributed to effective treatment of pulmonary infections and the reduction in tuberculosis.

Bronchiectasis↗

Surgery for bronchiectasis.

OBJECTIVE: The incidence of bronchiectasis has declined markedly in developed countries. However, a reasonable number of patients still need surgery, despite aggressive physiotherapy and antibiotic therapy. We have reviewed our patients to clarify the benefits from surgery and to analyse the complications. MATERIAL AND METHODS: Between 1988 and 1999, we have operated on 119 patients with bronchiectasis, 71 female and 48 male, with a mean age of 42.2 years (range 11--77 years). Surgery was indicated because of unsuccessful medical therapy in 66 patients (55%), 31 (26%) had haemoptysis, 11 (9.2%) had lung abscess, 10 (8.4%) had lung masses, and three (2.5%) had pneumothorax. The most common manifestations were cough with sputum in 90 patients (76%), haemoptysis in 45 (38%) and recurrent infections in 57 (48%). The mean duration of the symptoms was 4 years (range 1--40 years). The lower lobes were diseased in 61 patients and bilateral disease was found in ten. The mean number of involved pulmonary segments was five (range 1-15). A lobectomy was performed in 75 patients (62%), a segmentectomy in 12 (10%), a pneumonectomy in nine (7.4%) and a bilobectomy in four (3.3%). Complete resection of the disease was achieved in 108 cases (91%). RESULTS: There was no operative mortality and perioperative morbidity occurred in 15 patients (15%), including temporary broncho-pleural fistulae in 7 (5.8%), and post-operative haemorrhage and atrial arrhythmias in four (3.3%) each. After a mean follow-up was 4.5 years, 73 patients (68%) of this group were asymptomatic, and 31 (29%) had meaningful clinical improvement, while only four (3.7%) maintained or worsened prior symptoms. The best clinical improvement occurred in patients with complete resection of the disease (P=0.008). There were no differences in the respiratory function, comparing pre- and post-operative data, with a 2-year of minimum interval. The VC was 91 and 89% and the FEV1 was 83% and 81% of expected, respectively before and after surgery, (P=NS). CONCLUSION: Surgery of pulmonary bronchiectasis has few complications and markedly improves symptoms in the great majority of patients, especially when complete resection of the disease is achieved. Pulmonary resection of bronchiectasis does not alter respiratory function.

Adult↗