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PubMed1998

Treatment of idiopathic bronchiectasis with aerosolized recombinant human DNase I. rhDNase Study Group.

STUDY OBJECTIVE: To study the safety and efficacy of aerosolized recombinant human DNase I in the treatment of idiopathic bronchiectasis. DESIGN: Double-blind, randomized, placebo-controlled, multicenter study. POPULATIONS: Three hundred forty-nine adult outpatients in stable condition with idiopathic bronchiectasis from 23 centers in North America, Great Britain, and Ireland. INTERVENTIONS AND MEASUREMENTS: Study patients received aerosolized rhDNase or placebo twice daily for 24 weeks. Primary end points were incidence of pulmonary exacerbations and mean percent change in FEV1 from baseline over the treatment period. RESULTS: Pulmonary exacerbations were more frequent and FEV1 decline was greater in patients who received rhDNase compared with placebo during this 24-week trial. CONCLUSIONS: rhDNase was ineffective and potentially harmful in this group of adult outpatients in stable condition with idiopathic bronchiectasis. This contrasts with previously published results that demonstrated efficacy of rhDNase in patients with cystic fibrosis bronchiectasis.

Administration, Inhalation↗

PubMed2002

Exhaled H(2)O(2) in steady-state bronchiectasis: relationship with cellular composition in induced sputum, spirometry, and extent and severity of disease.

STUDY OBJECTIVES: To determine the concentration of exhaled H(2)O(2) in patients with bronchiectasis, and to study the relationship between levels of exhaled H(2)O(2), extent of disease, symptoms score, spirometry, and cellular composition obtained from induced sputum; furthermore, to account for possible confounding effects of inhaled corticosteroids (ICS) usage, long-term oral antibiotic treatment, and chronic colonization with Pseudomonas aeruginosa. DESIGN: Cross-sectional study. PATIENTS: Thirty patients with steady-state bronchiectasis. RESULTS: Mean (95% confidence interval [CI]) exhaled H(2)O(2) levels were significantly elevated in patients with bronchiectasis compared to normal subjects: 1.1 (0.87 to 1.29) microM vs 0.3 (0.19 to 0.36) microM, respectively (p < 0.0001). Patients treated with ICS had similar values as steroid-naïve patients. The group of patients with P aeruginosa colonization showed a significantly increased concentration of H(2)O(2) compared to the group without P aeruginosa colonization. Patients receiving long-term oral antibiotic treatment had significantly higher values of H(2)O(2) compared to those not receiving antibiotics. There was a significant positive correlation between H(2)O(2) and either the percentage of neutrophils in induced sputum or the extent of the disease as defined by high-resolution CT. A significant negative correlation was found between H(2)O(2) and FEV(1) percent predicted. Finally, there was a significant positive correlation between H(2)O(2) and the symptoms score. CONCLUSIONS: Patients with bronchiectasis in stable condition showed increased levels of exhaled H(2)O(2). The above-mentioned levels were not decreased either by ICS or long-term oral antibiotic treatment, but were significantly affected by chronic colonization with P aeruginosa. H(2)O(2) levels could be an indirect index of neutrophilic inflammation, impairment of lung function, and extension and severity of the disease.

Administration, Inhalation↗

PubMed2005

A pilot study of the safety and efficacy of tobramycin solution for inhalation in patients with severe bronchiectasis.

STUDY OBJECTIVE: To evaluate the efficacy and safety of tobramycin solution for inhalation (TSI) in patients with severe bronchiectasis. DESIGN: Open-label clinical trial consisting of three treatment cycles (14 days of drug therapy, and 14 days off drug) and an additional 40-week follow-up by chart review. SETTING: Nine clinical sites throughout the United States. SUBJECTS: Forty-one adult patients (>/= 18 years old) with diffuse bronchiectasis affecting two or more lung segments and a history of Pseudomonas aeruginosa infection. INTERVENTIONS: TSI, 300 mg tobramycin per dose bid. MEASUREMENTS AND RESULTS: During the 12-week treatment period, significant improvements (reduction of 1.5 U [p = 0.006]) occurred in mean pulmonary total symptom severity score, a composite score that assesses the severity of cough, shortness of breath, sputum production, fatigue, and wheezing. Significant improvements (reduction of 9.8 U [p < 0.001]) were also observed in St. George Respiratory Questionnaire scores, which measure health-related quality of life. Eradication or presumed eradication of P aeruginosa occurred in 6 of 27 evaluable subjects (22.2%). Tobramycin-resistant P aeruginosa developed in two subjects (minimal inhibitory concentration >/= 16 microg/mL). Ten subjects withdrew from the study due to adverse events; in nine of these subjects, adverse events were considered probably or possibly related to treatment. The most common adverse events were cough, wheezing, and dyspnea. CONCLUSIONS: TSI therapy resulted in significant improvements in respiratory symptoms and health-related quality of life in subjects with severe bronchiectasis, but some subjects did not tolerate TSI therapy. Bronchiectasis patients receiving this therapy should be monitored for signs of intolerance.

Administration, Inhalation↗

PubMed2005

The disease-modifying effects of twice-weekly oral azithromycin in patients with bronchiectasis.

INTRODUCTION: Bronchiectasis is a chronic pulmonary process characterized by recurrent respiratory infections leading to destruction of airways secondary to inflammation. We investigated whether the addition of 6-months' twice-weekly azithromycin to the existing treatment regimen in patients with pulmonary bronchiectasis decreased the number of exacerbations and improved pulmonary function compared with a similar period of time without concurrent azithromycin. METHODS: Thirty patients with high-resolution computed tomography scan-confirmed bronchiectasis were to be recruited. In random order, patients received usual medications for 6 months, and usual medications plus oral azithromycin 500mg twice weekly for 6 months. Patients receiving azithromycin first had a 1-month washout period prior to entering the second phase. Patients recorded weekly peak flow (PF) measurements. Pulmonary function tests (PFTs), 24-hour sputum volume, and needs for intervention with medication or ancillary support were collected at baseline and every 3 months. Exacerbation incidence and sputum volume measurements were compared from baseline to the end of each study phase. RESULTS: Twelve patients were enrolled; 11 were included in the analysis. Owing to randomization, most patients received the azithromycin first, which was fairly well tolerated. PFTs did not change significantly during either study phase and PFs appeared to remain stable during azithromycin therapy and throughout the subsequent control phase. Azithromycin significantly decreased the incidence of exacerbations compared with usual medications (5 vs 16; p = 0.019). Mean 24-hour sputum volume significantly decreased (15% [p = 0.005]) during the active treatment phase, and remained decreased during the control phase (p = 0.028). Subjectively, patients reported increased energy and quality of life while receiving treatment with azithromycin. CONCLUSIONS: The addition of twice-weekly azithromycin significantly decreased the incidence of exacerbation and 24-hour sputum volume and may have stabilized the PFTs and PFs in this 11-patient pilot study. The results of this study justify further investigation of adding azithromycin to the treatment regimens of patients with bronchiectasis for its disease-modifying effects.

Aged↗

PubMed1978

Bronchography in the recognition of congenital cystic bronchiectasis.

Congenital cystic bronchiectasis is an uncommon disease. Most of the reported cases have been in infants and young children. This paper presents four cases of older children and younger adults who had congenital cystic bronchiectasis. Distribution of the disease is lobar or may even involve an entire lung. Bronchography plays an important role in the diagnosis of congenital cystic bronchiectasis. The three important bronchographic signs of congenital cystic bronchiectasis are: (1) absence of contrast filling of the bronchial glands duct; (2) contrast filling of numerous cysts which communicate directly with the bronchial tree; and (3) nonvisualization of normal lung parenchyma within the area of involvement.

Adolescent↗

PubMed1994

Volumetric high-resolution CT in the diagnosis of interstitial lung disease and bronchiectasis: diagnostic accuracy and radiation dose.

OBJECTIVE: The purpose of this study was to evaluate the diagnostic accuracy and radiation dose of volumetric high-resolution CT in the diagnosis of interstitial lung disease and bronchiectasis when four contiguous sections were acquired at each of three levels. The potential benefits were weighed against the increased radiation dose of multiple scans. SUBJECTS AND METHODS: High-resolution CT scans of four contiguous sections were obtained at each of three locations (the aortic arch, the carina, and 2 cm above the diaphragm) in 50 consecutive patients (mean age, 44 years old) with known or suspected interstitial lung disease or bronchiectasis who were referred for evaluation with high-resolution CT. Each individual scan was analyzed for the presence of motion-induced streaking, blurring, or doubling. The diagnostic information contained in each set of four scans was compared with that contained in the first of the four scans in the set. RESULTS: Motion degraded at least one of the four images in each set in 69 (46%) of 150 volumetric acquisitions. When the full set of four images was considered instead of just the first scan from the set, the number of motion-free studies in patients with suboptimal respiratory suspension was increased by 40% (from 99 to 139). Diagnostic accuracy was improved as more features were identified on contiguous scans: the sensitivity of the first scan compared with that of the complete set of four scans was 84% for the detection of bronchiectasis, 97% for ground-glass opacity, 88% for honeycombing, 88% for septal thickening, and 86% for nodular opacities. Although the integral radiation exposure for a set of four CT scans was 2.8 times that of a single scan obtained with standard technique, peak skin exposure was unchanged. Slightly increased image noise with the reduced technique compromised diagnostic ability in 6% of studies. CONCLUSION: The use of volumetric high-resolution CT increased diagnostic accuracy, particularly for bronchiectasis at the lung bases, without increasing peak skin radiation exposure. With the availability of four contiguous scans per anatomic level, the subjective confidence in interpretation and number of motion-free studies also increased.

Adult↗

PubMedDate not supplied

Effect of obstructive airway disease in patients with non-cystic fibrosis bronchiectasis.

BACKGROUND: Extensive research has been devoted to cystic fibrosis-related brochiectasis, compared with non-cystic fibrosis bronchiectasis but the latter is more common and results in significant morbidity and mortality. We assessed the relationship between pulmonary function test (PFT) findings and sputum bacteriology, blood gases, number of hospital admissions and mortality in patients with non-cystic fibrosis bonchiectasis (NCFB). METHODS: We conducted a retrospective review of 88 consecutive patients admitted with exacerbation of bronchiectasis over 5 years from 1996 to 2001. Demographic and clinical data collected included gender, age, pulmonary functions, arterial blood gases, sputum bacteriology during stable and exacerbation periods, and number of hospital admissions due to exacerbation of bronchiectasis. A comparison was made between patients having obstructive airway disease (OAD group) and patients with normal or restrictive pulmonary functions (non-OAD group). RESULTS: OAD in patients with NCFB adversely affected clinical outcome. There was a significant increase in Pseudomonas colonization (60.3% vs. 16%; P<0.0003), hypercapnic respiratory failure (63.4% vs. 20%; P<0.0003), and mean number of admissions due to exacerbation (6 vs. 2; P<0.0001) in the OAD group as compared with the non-OAD group. Although mortality was increased in the OAD group, the difference was not statistically significant. CONCLUSION: Patients with NCFB who have OAD have a significantly higher rate of colonization with Pseudomonas aeruginosa (PSA), hypercapnic respiratory failure, a greater number of hospital admissions due to exacerbation of bronchiectasis, and a higher mortality compared with patients with restrictive or normal pulmonary functions.

Adolescent↗

PubMed2002

Bronchiectasis in indigenous children in remote Australian communities.

The rates of bronchiectasis for Indigenous children from remote Australian communities are unacceptably high, with one study showing 14.7/1000 Aboriginal children. Children with bronchiectasis need to be identified early for optimisation of medical treatment. Under-reporting of cough is common. Bronchiectasis should be suspected in children with recurrent bronchitis or pneumonia, and when, despite appropriate therapy, pulmonary infiltrates or atelectasis persist 12 weeks beyond the index illness. During acute infective episodes, oral antibiotics and chest physiotherapy to clear the airways should produce prompt resolution; otherwise, hospitalisation is necessary. Management follows the cystic fibrosis model of regular review, encouragement of physical activity, optimising nutrition, maintenance of immunisation and avoidance of environmental toxicants, including passive smoke exposure. Successful management and prevention of bronchiectasis will require improvements in housing, nutrition, and education, as well as access to comprehensive healthcare services, with coordination between primary and hospital-based healthcare providers.

Adolescent↗

PubMed1998

133Xenon ventilation scan as a functional assessment in bronchiectasis.

BACKGROUND: Mucus impaction in the airways impairs ventilation and exercise tolerance in patients with bronchiectasis. Parameters for evaluating the ventilatory dynamic change have been limited by variable cofactors. We developed a tool to evaluate the changes directly on images of a ventilation scan. MATERIALS AND METHODS: We used a 133Xenon ventilation washout scan to assess the time of half clearance (T1/2) of the regions of interest (ROIs) corrected by that of a control area (CA) as T1/2ROI/CA. We then compared the ventilation washout scan with high-resolution computer tomography (HRCT) scoring to assess the severity of bronchiectasis, as well as conducting 6-minute walking tests or spirometry for the evaluation of the clinical response to a 3-day course of chest physiotherapy. Nine patients with bronchiectasis and mucus hypersecretion were enrolled in this study. RESULTS: The functional impairments by mucus impaction or air trapping were well documented in the ventilation washout scan, which not only provided an anatomical image but also dynamic profiles. The ratios of T1/2ROI/CA were significantly correlated to the corresponding scoring of HRCT (3.45 +/- 0.85 vs 7.50 +/- 1.51, r2 = 0.61, p = 0.023, n = 8). The improvement in T1/2ROI/CA (from 3.45 +/- 0.85 to 2.60 +/- 0.59, p = 0.022, n = 9) was paralleled by an increase in the 6-minute walking test (from 310.4 +/- 43.2 m to 352.4 +/- 45.1 m, p = 0.028, n = 7). CONCLUSION: The 133Xenon scan may be used to evaluate the heterogeneity of ventilation abnormalities and the efficacy of clinical therapy directly in patients with bronchiectasis.

Bronchiectasis↗

PubMed1999

Lung resection for the treatment of severe localised bronchiectasis in cystic fibrosis patients.

BACKGROUND: A small proportion of cystic fibrosis patients develop severe localised bronchiectasis. When this persists despite maximal medical therapy it presents a difficult management problem. Lung transplantation cannot be justified. We report encouraging results in six patients with severe localised bronchiectasis and cystic fibrosis who underwent pulmonary resection. METHODS: Each child had severe localised bronchiectasis despite maximal medical therapy. Intensive preoperative toilet was instituted and pulmonary resection undertaken when lung function was optimal. RESULTS: There was a marked improvement in symptoms in every case. No significant long-standing morbidity was associated with the resection. There was no significant decrease in pulmonary function following resection. CONCLUSION: Pulmonary resection should be considered in the management of severe localised bronchiectasis unresponsive to maximal medical therapy in cystic fibrosis patients.

Bronchiectasis↗

PubMed1999

[Bronchiectasis in a patient with ulcerative colitis].

Bronchopulmonary involvement is uncommon in ulcerative colitis. Bronchiectasis is a rare manifestation of the disease. We report a case of a 65-year-old patient with ulcerative colitis in whom we diagnosed bronchiectasis. The evidence of a relationship between bronchiectasis and ulcerative colitis was established on the basis of 3 criteria: the late occurrence of bronchiectasis in a patient without pulmonary history, their appearance after the ulcerative colitis developed and their improvement after inhaled corticosteroid treatment. This observation points out that ulcerative colitis investigations should be extended to the respiratory structures. The respiratory involvement is not always concomitant with the bowel disease. This observation suggests the efficiency of the inhaled corticosteroids in this disease.

Aged↗

PubMedDate not supplied

[Bilateral pulmonary resections for bilateral bronchiectasis].

The present paper reports on 6 cases of bilateral bronchiectasis in patients aged 11 to 63 years, treated by simultaneous bilateral pulmonary resection and 1 case of bilateral bronchiectasis in which 14 segments were resected successively. Surgery by simultaneous bilateral approach and associated resection of the affected areas is a procedure that should be indicated, in the authors' opinion, only in carefully selected cases in which not more than 13 segments have to be resected. The indications and contraindications of surgery in bilateral bronchiectasis are discussed, preference being given to the simultaneous approach. In the cases reported at most 11 1/2 segments of the four pulmonary lobes affected were resected in a single stage; in 1 case 14 segments were resected successively, the resection exceeding in amplitude all similar one published until now. The immediate and late postoperative evolution (lesional and functional) were favourable, which pleads for a reconsideration of the approach to bilateral bronchiectasis and the application of the surgical treatment recommended, within the limits described.

Adolescent↗

PubMed2000

Lung resections in bronchiectasis due to lipoid pneumonia: a custom-design approach.

OBJECTIVE: To see the effect of "custom-designed" surgical resection of some severely damaged parts of the lungs in children with bronchiectasis caused by forced feeding of children early in their lives with animal fat "ghee". DESIGN: All children with bronchiectasis--post lipoid pneumonia--who failed to respond to medical treatment had surgical removal of most affected parts of their lungs. SETTINGS: Asir Central Hospital Abha, a referral hospital in Asir region of Saudi Arabia and a Teaching Hospital for College of Medicine and Health Sciences, King Khalid University, Saudi Arabia. SUBJECTS: Six children aged between seven and 12 years with lipoid bronchiectasis were referred to paediatric surgical service for surgical treatment. RESULTS: There was no surgical mortality. Apart from mild cough, all the pre-operative symptoms of the children disappeared. Two children died more than six months post-operatively due to the extent of the original disease. CONCLUSION: Surgical resection designed to remove the most affected parts of the lungs in diffuse-type bronchiectasis is recommended when there is failure of medical treatment.

Bronchiectasis↗

PubMed2003

[Analysis of clinicopathologic features and morphogenesis of carcinoid tumorlets in the lung with bronchiectasis].

OBJECTIVE: To describe the clinicopathologic features and immunophenotypes of carcinoid tumorlets in the lung with bronchiectasis, and to study the morphogenesis of these tiny tumors. METHODS: The histopathologic characteristics of 3 bronchiectasis cases with carcinoid tumorlets, 11 bronchiectasis and 2 normal lungs were studied. Specific markers of the tiny tumors and the number of neuroendocrine cells (NECs) in the airway mucosa were immunohistochemically detected by EnVision method. RESULTS: The tumorlets in the lungs presented as multi-focal nodules and most were displayed only under microscopy. These cells were arranged in clusters and foci of fascicles which were situated in the surrounding bronchial wall and bronchioles adjacent to bronchiectatic lesion, or in the scar tissues. The tiny tumors were consisted of short fusiform cells and small ovoid cells. Their nuclei were circular, oval or long fusiform and the cells were strongly argyrophilic on Grimelius staining. Intensive positive immunostaining for calcitonin, chromogranin A, NSE and gastrin were detected. Weak positive for CK, EMA, S-100 and focal positive for HC, ACTH, 5-HT were also observed. Proliferative NECs in airway mucosa adjacent to the tiny tumors increased significantly in number, compared with those in the airway mucosa of bronchiectasis without tumorlets and normal lungs (P < 0.001, respectively). CONCLUSIONS: The clinicopathologic features and immunophenotypes of carcinoid tumorlets resemble carcinoid tumors. They are the early stage of carcinoid development. Their development may be related to the chronic pulmonary damage resulting in hypoxia and stimulating the proliferation of NECs. These pulmonary carcinoid tumorlets can be used as a model to study the tumorigenesis of carcinoid carcinoma of the lung.

Adult↗

PubMed2004

[Typing of bronchiectasis according to syndrome differentiation].

OBJECTIVE: To study the general law of typing of bronchiectasis according to syndrome differentiation. METHODS: We collected the symptoms, conditions of tongue and pulse in patients of bronchiectasis, using frequencies procedure, discriminant analysis and K-means cluster analysis in SPSS statistical software as research medium. RESULTS: Five hundred and sixty three patients with bronchiectasis were studied. It suggested that accumulation of phlegm-heat in the lungs (45.65%), liver fire attacking the lungs (24.51%), asthenia of pulmonosplenic qi (22.38%), asthenia of both qi and yin (7.46%) were the main types. CONCLUSION: Clinical epidemiology provided scientific basis for further studying of the typing of bronchiectasis according to syndrome differentiation. Building up differentiation of syndromes through differentiation and analysis of main symptoms can be used in clinical diagnosis.

Adolescent↗

PubMed2004

Exhaled nitric oxide in bronchiectasis: the effects of inhaled corticosteroid therapy.

SETTING: While exhaled nitric oxide (eNO) levels are reduced by inhaled corticosteroid therapy in asthma, such treatment effect is unclear in bronchiectasis. DESIGN: Stable non-smoking bronchiectasis patients were randomised to receive either fluticasone (1 mg/daily) or identical placebo via the Accuhaler device. RESULTS: Sixty non-smoking patients (38 women; mean age 56.4 +/- 12.7 years) were recruited. Of these, half received inhaled fluticasone and half placebo therapy. eNO was measured using a chemiluminescence analyser at 0, 4, 12, 24, 36 and 52 weeks. There was no significant difference in eNO levels between fluticasone and placebo patients over the study period. There was no correlation between baseline eNO with age, FEV1, FVC, 24 h sputum volume or number of bronchiectatic segments. Patients with Pseudomonas aeruginosa (PA) infection, but not their counterparts, displayed a correlation between 0- and 52-week eNO levels. PA infection was associated with significantly lower eNO levels among the patients. CONCLUSIONS: Inhaled fluticasone therapy, despite being an effective anti-inflammatory agent, has no significant effect on eNO production, either at individual time points or over the entire 52-week profile, in bronchiectasis. It appears that eNO might not reflect the extent of airway inflammation in bronchiectasis.

Administration, Inhalation↗

PubMed1995

An update on bronchiectasis.

Bronchiectasis is associated with heterogeneous predisposing conditions that cause abnormal dilatation and persistent inflammation in the bronchial tree and lung parenchyma. The disease remains a common cause of significant morbidity and mortality, especially when associated with hereditary disorders such as cystic fibrosis, ciliary dyskinesia, and immunodeficiency states. Recent investigations have focused on the inflammatory mediators involved in the pathogenesis of bronchiectasis. High-resolution computed tomography is now the diagnostic modality of choice and may also contribute to clinical management. Computed tomography and high-resolution computed tomography have identified bronchiectasis in individuals with HIV and alpha-1-antitrypsin deficiency. Early identification of predisposing disorders and aggressive management of symptoms has already been demonstrated by prolonged survival and decreased morbidity in cystic fibrosis patients, and similar management may benefit other populations with bronchiectasis. New treatments such as recombinant human DNase have been shown to improve pulmonary function and quality of life in cystic fibrosis patients and may prove useful in other chronic inflammatory lung disease.

Bronchiectasis↗

PubMed1991

High resolution CT and bronchography in the assessment of bronchiectasis.

To elucidate the reliability of CT in the assessment of bronchiectasis, a retrospective study of high resolution CT and bronchography was carried out. A segment by segment comparison of 259 segmental bronchi from 70 lobes of 27 lungs in 19 patients was performed using bronchography as standard. CT was positive in 87 of 89 segmental bronchi with bronchiectasis giving a false-negative rate of 2%. CT was negative in 169 of 170 segmental bronchi without bronchiectasis at bronchography, giving a false-positive rate of 1%. There was agreement between the two modalities in identifying the different types of bronchiectasis.

Adult↗