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Changing paradigms in the diagnosis and management of bronchiectasis.

The face of bronchiectasis may have changed in recent years but individual cases continue to pose difficult challenges. As childhood infection becomes less of a problem, alternative causes of bronchiectasis are increasingly recognized which themselves offer new problems of diagnosis and management. Evolving concepts of pathogenesis suggest alternative strategies for treatment but as yet the evidence base on which to make firm decisions is lacking. Antibacterial regimens are not universally applicable and individualized protocols with parenteral, nebulized or continuous antibacterial therapy are increasingly used in the treatment of patients with bronchiectasis. Despite the theoretical appeal of using mucolytic or anti-inflammatory drugs their roles are still uncertain and have yet to be examined in adequate clinical trials. The factors determining disease progression are still poorly understood but in some patients worsening airflow obstruction heralds the onset of ventilatory failure. The management of the latter requires bronchodilators and controlled oxygen therapy, and strategies including non-invasive ventilation are increasingly an option. Changing indications for surgery are evident with fewer palliative resections but a developing role for transplantation.

Adrenal Cortex Hormones↗

Inflammatory bowel disease and the lung: is there a link between surgery and bronchiectasis?

PURPOSE: One-third of patients with inflammatory bowel disease (IBD) has extracolonic manifestations. Inflammatory bowel-associated pulmonary disease is one of the less commonly recognized and more recently described manifestations. Here, we report the experience of our patients with inflammatory bowel-associated bronchiectasis. METHODS: A retrospective analysis of case notes of patients with IBD and respiratory manifestations was undertaken. Relevant demographic, clinical, radiological, and pulmonary physiology laboratory results were reviewed. RESULTS: Ten patients with IBD and bronchiectasis were identified. Eight developed respiratory symptoms after surgery for IBD. Five of the ten had ulcerative colitis. Their lung function abnormality is mild to moderate in severity. Small airways disease (forced expiratory flow between 25-75% is <50%) was evident in seven of the ten patients. CONCLUSIONS: This preliminary study supports an association between surgery for IBD and development of symptomatic lung disease, particularly bronchiectasis, in susceptible patients. The pulmonary manifestations of IBD in some patients may only become clinically significant after surgery and the withdrawal of medical treatment.

Aged↗

Bronchiectasis masquerading as pulmonary embolus.

The diagnosis of bronchiectasis usually suggests a patient with a productive cough and shortness of breath. This report describes the case history of an otherwise healthy 43-year-old man who presented with repetitive episodes of pleuritic pain that led to several extensive, invasive evaluations in search of a pulmonary embolus. Only after a carefully taken history did the patient admit to an intermittent chronic cough, which suggested the diagnosis of bronchiectasis. This diagnosis was ultimately confirmed by bronchography. This report suggests that patients with bronchiectasis may present with unusual clinical manifestations, and that the correct diagnosis may be missed unless physicians are aware that "old" diseases may present in "new" ways.

Acute Disease↗

Failure of systemic antibiotic in preventing sequential endogenous endophthalmitis of a bronchiectasis patient.

PURPOSE: To report a rare presentation of bilateral, sequential Pseudomonas aeruginosa endogenous endophthalmitis in a woman with bronchiectasis. DESIGN: Interventional case report. METHODS: A 69-year-old woman with bronchiectasis developed right and then left endogenous endophthalmitis with the microbial of P. aeruginosa, even though a course of 3-week intravenous ceftazidime antibiotic was prescribed. RESULTS: The presentation of endogenous endophthalmitis in the right eye was late, and final visual acuity was hand movements. The condition was recognized much earlier in the left eye, and the infection was treated early on with vitrectomy and intravitreal antibiotics. Visual acuity at 1 year was stable at 20/40. CONCLUSION: Systemic antibiotic failed to eradicate the primary source of Pseudomonas infection in the bronchiectasis patient. Unusual, painful red eye may be the presenting feature of endogenous endophthalmitis. Early vitrectomy may be considered in endogenous endophthalmitis caused by virulent pathogens such P. aeruginosa.

Aged↗

Lung transplantation for non-cystic fibrosis bronchiectasis: analysis of a 13-year experience.

BACKGROUND: Lung transplantation is a well-established treatment for end-stage cystic fibrosis, and there are considerable data on medium- and long-term results. However, less information exists about transplantation for non-cystic fibrosis bronchiectasis. METHODS: Between December 1988 and June 2001, 22 patients (12 men, 10 women) underwent transplantation for bronchiectasis not due to cystic fibrosis. Procedures were bilateral sequential single-lung transplants (BSSLTX) in 4 patients, en bloc double lung transplants (DLTX) in 5, heart-lung transplants (HLTX) in 6, and single-lung transplants (SLTX) in 7. Lifelong outpatient follow-up was continued at a minimum of every 6 months. RESULTS: One-year Kaplan-Meier survival for all patients was 68% (95% confidence interval [CI], 54%-91%), and 5-year survival was 62% (95% CI, 41-83%). One-year survival after SLTX was 57% (95% CI, 20%-94%) vs 73% (95% CI, 51-96%) for those receiving 2 lungs. At 6 months, mean forced expiratory volume in 1 second was 73% predicted (range, 58%-97%), and mean forced vital capacity was 68% predicted (range, 53%-94%) after receiving 2 lungs (n = 10); in the SLTX group at 6 months, mean forced expiratory volume in 1 second was 50% predicted (range, 34%-61%), and mean forced vital capacity was 53% predicted (range 46-63%) (n = 4). CONCLUSIONS: Survival and lung function after transplantation for non-cystic fibrosis bronchiectasis was similar to that after transplantation for cystic fibrosis. A good outcome is possible after single lung transplantation in selected patients.

Adult↗

Surgical management of childhood bronchiectasis due to infectious disease.

BACKGROUND: The purpose of this study was to estimate operative risk and to identify indicators of adverse prognosis in patients undergoing resection for childhood bronchiectasis. METHODS: From January 1985 to February 2001, patients undergoing resection for bronchiectasis were studied. The indications for operation were failure of medical therapy in 33 patients (94.2%) and hemoptysis in 2 (5.7%). The mean duration of symptoms was 4.2 years (range, 1-9 years). Surgical treatment included lobectomy in 17 patients (48.5%), pneumonectomy in 7 (20%), lobectomy plus segmentectomy in 5 (14.2%), bilobectomy in 2 (5.7%), and segmentectomy in 4 (11.4%). RESULTS: The operative mortality rate was 2.8%, and the morbidity rate was 17.6%. The mean follow-up in 34 patients was 5.4 years (range, 1-12 years). Overall, 22 patients (64.7%) were asymptomatic after surgery. Clinical improvement was noticed in 8 patients (23.5%), and no improvement was noticed in 4 (11.7%). Complete resection resulted in a significantly better clinical outcome than incomplete resection (P <.05). CONCLUSIONS: Surgery for childhood bronchiectasis can be performed with low mortality and morbidity. Complete resection should be performed when possible.

Adolescent↗

Non-cystic-fibrosis bronchiectasis: a perspective from South America.

Nowadays, bronchiectasis tends to be considered a rare disease. This is really the case in developed countries, where good standards of living have been prevalent for many decades. But it might not be the case in the developing world, where better sanitary conditions are still needed. This article reviews non-cystic fibrosis bronchiectasis, emphasising differences between developed and developing countries. Diagnostic methods and therapeutic issues are discussed as is the Latin American experience of postviral bronchiectasis.

Bronchiectasis↗

Elevated levels of transforming growth factor-beta(1) in serum of patients with stable bronchiectasis.

Bronchiectasis is a chronic inflammatory and infective airway disease characterized by irreversible dilatation of the bronchi and persistent purulent sputum. Transforming growth factor-beta(1) (TGF-beta(1)) has been found to be increased in the lungs or bronchoalveolar lavage fluid of patients with inflammatory lung diseases. However, little is known on the serum TGF-beta(1) levels in patients with bronchiectasis. We aimed to determine the serum TGF-beta(1) concentrations in 95 patients with stable bronchiectasis (63 women; mean+/-sd age, 58.9+/-14.1 years) and 68 control subjects (23 women; 48.9+/-12.8 years) by ELISA, and to correlate with clinical parameters. The serum TGF-beta(1) levels were significantly higher in bronchiectatic patients compared with control subjects (median [range], 1812.5 pg/ml [1226.4-4114.5 pg/ml] vs. 1342.4 pg/ml [940.3-2371.7 pg/ml]; P<0.001). There was, however, no correlation between serum TGF-beta(1) levels with FEV(1) (% predicted), FVC (% predicted), 24h sputum volume, the number of bronchiectatic lung lobes or total white blood cell count (P>0.05). Our findings support previous indications that TGF-beta(1) may contribute to bronchiectatic airway inflammation. Further studies on the potential mechanisms and pathogenesis implications of this elevation should also be pursued in future.

Adult↗

The impact of bronchiectasis associated to sinonasal disease on quality of life.

BACKGROUND: Bronchiectasis (BQs) is an uncommon disease with the potential to cause devastating complications. All patients with BQs have cough and chronic sputum production that may have a great impact on patient's quality of life. Upper airway symptoms are also frequent in patients with BQs. Associations between upper and lower airways diseases have been demonstrated in allergic rhinitis and asthma, nasal polyposis and asthma, chronic obstructive lung disease and chronic rhinosinusitis. OBJECTIVE: (1) To investigate the impact of bronchiectasis and nasal symptoms on quality of life. (2) To evaluate the added impact of nasal polyposis on quality of life in patients with BQs. METHODS: Sixty patients with bronchiectasis and upper airway symptoms were included. Patients were evaluated for nasal symptoms, nasal polyp size by endoscopy, and quality of life using the SF-36 generic questionnaire. RESULTS: In comparison with the Spanish general population, patients with BQs had worse scores in all SF-36 domains (P<0.05). Males reported significantly higher quality of life scores on physical functioning and social functioning than females did. Although the age, pulmonary function, presence of nasal polyps, upper airway symptoms, tobacco smoking history, and disease duration was similar between them. Males with BQs had worse quality of life than males from the Spanish general population on body pain, general health, and vitality (P<0.05). Females with BQs had worse quality of life than females from the Spanish general population on physical function, body pain, general health, vitality, social function, and emotional role (P<0.05). Nasal polyps were found in 25 (41.6%) of 60 patients with BQs. No significant differences were observed on quality of life outcomes between patients with BQs with and without nasal polyposis. CONCLUSION: These results suggest that BQs has a considerable impact on quality of life while nasal polyposis has no additional impact on the quality of life of patients with BQs.

Adult↗

[Differential diagnosis of bronchiectasis: high-resolution CT as a valuable aide].

Bronchiectasis is defined as localized irreversible dilatation of the bronchial tree. Brochiectasis has been associated with a wide variety of causes, but it is mostly caused by acute, chronic or recurrent infections. This paper should give a review about the manifestation of bronchiectasis and bronchioloectasis in HR-CT and discuss the causing entities. However, integration of bronchiectasis and other HR-CT findings may enable a narrower differential diagnosis, in some cases it is possible to give the correct diagnose directly.

Bronchiectasis↗

Limited operation for severe multisegmental bilateral bronchiectasis.

BACKGROUND: Some patients exhibiting severe multisegmental bilateral bronchiectasis are no longer improved with antibiotic treatment and drainage and, most of the time, operation is contraindicated. In our institution, limited operation has been offered to select patients for this indication. We report our data regarding the feasibility and utility of such a procedure. METHODS: We studied 16 patients who underwent surgical removal of nonlocalized disease between 1990 and 1999. We report the mortality and morbidity rates of this surgical procedure and the clinical, bacteriological, and functional data for each patient. RESULTS: There was no mortality and the morbidity was low (18%, all with favorable outcome). Symptoms such as hemoptysis, sputum production, or dyspnea were also improved. The recurring infections decreased in frequency in 8 patients and disappeared completely in 5 others. The bacteriological data assessment revealed disappearance of germs in 4 patients and persistence of chronic colonization in others. Postoperative spirometric data were not worsened and postoperative computed tomographic scans did not show progression of lesions not removed. CONCLUSIONS: These results suggest that, in properly selected patients, lasting symptomatic improvement can be achieved by resection. Limited operation may be indicated in nonlocalized bilateral bronchiectasis, provided that a target can be identified. This procedure is supported by physiopathologic arguments and is particularly relevant to patients with bronchiectasis with cystic and functionless territories.

Adolescent↗

Immune-deficient bronchiectasis associated with X-linked lymphoproliferative disease.

Bronchiectasis may occur with various congenital and acquired immunodeficiency diseases. The association of bronchiectasis and the X-linked lymphoproliferative disease (XLP), also known as Duncan's disease is unknown. We describe the case of a 39-year-old man with XLP, the oldest surviving, who developed chronic bronchiectasis with hemoptysis and required a pneumonectomy to control his symptoms.

Adult↗

The value of computed tomography in the diagnosis and management of bronchiectasis.

In order to evaluate the usefulness of computed tomography (CT) in the diagnosis and management, as distinct from only the diagnosis, of bronchiectasis, we retrospectively reviewed the clinical, lung function, and radiological data of 38 patients suspected of having the disease. All had chest radiographs, CT scans, and bronchograms. The approach was to examine the radiological investigations with the clinical data in three stages: I, chest radiograph; II, CT; III, bronchography (BG), as if they were newly presenting cases. At the end of each stage, a decision was made either to proceed to the next stage or to stop because further investigation was considered unlikely to alter management. Apparent normality, equivocal abnormality, or unilateral abnormality were criteria for proceeding. Unfitness for surgery, unequivocal bilateral disease, or mild disease were criteria for stopping after a firm diagnosis had been made. We stopped at stage I in four patients (11%) because the chest radiograph showed bilateral bronchiectasis and two had poor lung function. We stopped at stage II in 15 patients (39%): 12 had bilateral disease on CT; three had unilateral disease on CT but their clinical features were so mild that BG was considered unjustified. Scrutiny of the CT and BG films of those patients who were judged not to require CT or BG in the retrospective review confirmed that these would not have altered their management. Of the remaining 19 patients who proceeded to stage III, BG was useful in 15 (39%) by confirming or refuting CT findings, but was not useful in four (11%) because of underfilling. We conclude that optimal use of chest radiographs and CT in patients suspected of having bronchiectasis can significantly reduce the necessity of performing BG.

Adult↗

[Pulmonary function and quality of life in relation to bronchial colonization in adults with bronchiectasis not caused by cystic fibrosis].

BACKGROUND: We aimed at assessing the pulmonary function and life quality of patients with bronchiectasis not caused by cystic fibrosis (CF) on the basis of the presence or absence of colonization (with Pseudomonas or other microorganisms). PATIENTS AND METHOD: Prospective, randomised control-case study of patients with bronchiectasis who came to the Pneumology area of the Hospital Universitario de Canarias between January 1999 and December 2000 in a stable clinic situation. Patients must had no antibiotic therapy over last six weeks before the study. Patients with CF and patients who had an acute respiratory disease were excluded. We obtained two sputum samples for culture with an interval of six weeks between both. We determined the expiratory flow in the first second (FEV1), the forced vital capacity (FVC) and arterial gases. Quality of life was measured by the St. George respiratory questionnaire. RESULTS: We included 70 patients, 25 males (35%) and 45 females (64%), with a mean (SD) age of 56 (17) years. There were 14 patients who had Pseudomonas (Ps-group), 10 who had other microorganisms (another-group) and 46 patients who had no microorganism in sputum culture (no-group). The pulmonary function of the no-group (FEV<SUB<1%: 73 [24] and FVC%: 79 [21]) was better than that of the Ps-group (FEV1%: 47 [27]; p = 0.00; FVC%: 61 [28]; p = 0.04). The quality of life of the no-group (total score (TS): 33.2 [18.9]) was better than that of the Ps-group (TS: 54 [23.2]; p = 0.00) and that of the another-group (TS: 52.2 [20.4]; p = 0.02). CONCLUSIONS: Non CF patients whose bronchiectasis are colonized with Pseudomonas have worse pulmonary function and quality of life than uncolonized ones. Moreover, patients having microorganisms other than Pseudomonas have a worse quality of life than those without microorganisms.

Adult↗

A morphologic study of nodular lung carcinomas and their possible pathogenesis from a cluster of non-obstructive bronchiectasis.

A very simple procedure has enabled us to show that nodular lung carcinomas correspond, to a high degree, to neoplasias arising in a cluster of bronchiectasis of the non-obstructive type. This pathogenesis explains their peculiar features: the round shape with a sharp borderline on the surrounding lung, the frequent cavitation, the prevailing histologic type of epidermoid carcinomas. At present, when thin-section CT allows discovery of bronchiectasis with no need for bronchography, this interpretation suggests the possibility of carrying out a prevention program which should consist of identifying the high-risk group of patients with bronchiectasis, 'sputum producers' and smokers, in which an early diagnosis of nodular lung carcinoma might be realized by periodical diagnostic cytology.

Adult↗

Long-term antibiotics in the management of non-CF bronchiectasis--do they improve outcome?

In addition to bacteria and inflammatory cells, the sputum of patients with bronchiectasis contains mediators that damage the airway epithelium and promote inflammatory change. The deleterious effects of these mediators, such as neutrophil elastase, reduce host defences and consequently perpetuate the propensity to recurrent infection. This 'vicious cycle' of infection and inflammation in bronchiectasis suggests that long-term antibiotic therapy might be beneficial in these patients by reducing microbial load and, in doing so, inhibit inflammation in the lung allowing tissue repair to occur. Short courses of antibiotics achieve clinical improvements and also have been shown to reduce the levels of harmful mediators in the sputum. This article will cite the studies reported for long-term antibiotic treatment in bronchiectasis and overall there seems to be benefits for patients with chronic sputum purulence. The evidence that supports the postulated pathological mechanisms will also be discussed. Important issues in clinical practice such as the usefulness of antibiotic sensitivities, the evolution of resistance patterns, and drug delivery will also be discussed.

Anti-Bacterial Agents↗

Surgery for unilateral bronchiectasis: results and prognostic factors.

SETTING: King Khalid University Hospital referral centre for thoracic surgery, Riyadh, Saudi Arabia. OBJECTIVE: To assess the results of surgery and factors influencing its outcome in patients with unilateral bronchiectasis. DESIGN: A retrospective analysis of 40 patients with unilateral bronchiectasis who were operated upon consecutively at King Khalid Hospital, between July 1987 and May 1993. RESULTS: Left-sided disease was seen in 60% (n = 24) and right-sided in 40% (n = 16) of the patients. The entire lung was involved in 30% of cases (n = 12). Of these, the left lung was totally involved in 22.5% (n = 9) and the right in 7.5% (n = 3). A lobectomy was performed on 21 patients, basal segmentectomy with preservation of apical segment on 7, and pneumonectomy on 12. There was no operative mortality in this series. Six patients (15%) developed postoperative complications, bleeding (n = 4) and prolonged air leak (n = 2). During an average follow-up period of 30.7 months (+/- 15.4 months), 29 patients (72.5%) were cured and the remaining 11 (27.5%) improved. No patients with Pseudomonas aeruginosa infection (n = 3) or obstructive airway disease (n = 5) were cured (P = 0.02 and P = 0.002 respectively). CONCLUSION: Curative resection for selected patients with unilateral bronchiectasis can be performed safely with good results and low morbidity. Pseudomonas aeruginosa infection and obstructive airway disease have an adverse effect on postoperative cure.

Adolescent↗

[Relation between rhinosinusitis and bronchiectasis].

The nose and lungs have both histological and functional similarities and differences. Sinonasal and bronchial involvement are associated in many diseases. Cystic fibrosis, primary ciliary dyskinesia, Young's syndrome, and alpha-1 antitrypsin deficiency are diseases in which bronchiectasis and rhinosinusitis are both present. This review considers the diseases in which bronchiectasis occurs along with sinonasal manifestations. We propose examining sinonasal disease from a new perspective by observing it in patients with bronchiectasis.

Bronchiectasis↗