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[Bronchiectasis following colectomy in a patient with ulcerative colitis and factor V Leiden mutation].

For some years it has been known that lung disease may be present in patients with inflammatory bowel disease (IBD). Diseases of the central airway, bronchi, and lung parenchyma are among the most common forms of lung involvement in IBD patients. Bronchiectasis is frequent and almost always appears after the onset of disease and in close association with inflammatory activity. However, reports of the appearance of bronchiectasis following colectomy for an exacerbation of IBD have been rare. We present the case of a 36-year-old man with severe ulcerative colitis who, following a total colectomy, developed bilateral bronchiectasis 12 months after surgery and for whom both preoperative and postoperative computed tomography scans were available.

Adult↗

[Inhaled antibiotic therapy in bronchiectasis?].

Antimicrobial therapy is an important aspect of disease management for patients with bronchiectasis. Delivery of an inhaled antibiotic is an appealing alternative to oral or intravenous administration because the antibiotic is delivered in high concentrations directly to the site of infection, eliminating the need for high systemic concentrations and reducing the risk of systemic toxicity. In recent controlled studies these potential benefits have been assessed in patients with bronchiectasis who became colonized by P. aeruginosa and the results support the use of nebulized antibiotics. In up to one-third of patients P. aeruginosa was eradicated from their sputum by inhaled antibiotic therapy and up to 62 % of patients showed improved medical condition. The further development of new aerosol devices supported by clinical testing will allow effective management of patients with bronchiectasis by an inhalation therapy that minimizes time constraints and drug loss which may improve health status and quality of life.

Administration, Inhalation↗

Lung resection for bronchiectasis in children.

Twenty children with bronchiectasis underwent pulmonary resections during an 8-year period. All patients suffered from significant symptoms that did not respond to medical treatment and had localised disease. In 7 patients bronchiectasis developed following foreign body aspiration and in two the aetiology was cystic fibrosis and immune deficiency, respectively. The lower lobes were most commonly involved. Atelectasis of the remaining ipsilateral lung was the most common postoperative complication occurring in four patients. One patient required reoperation for recurrent symptoms. Fifteen out of 18 patients who underwent curative resections were asymptomatic in the longterm follow-up. It is concluded that for children with significant symptoms due to localised bronchiectasis that fails to respond to medical treatment, resection of the affected lobes is indicated.

Adolescent↗

Current management of bronchiectasis: review and 3 case studies.

Bronchiectasis is the abnormal, irreversible dilatation of diseased bronchi. Permanently dilated airways, usually in the medium-sized bronchi, are inflamed and often obstructed with thick, purulent secretions. Known causative factors include postinfection bronchial damage, postinhalation injury, hypersensitivity reactions, and congenital airway obstructive disorders. Typical symptoms include sputum overproduction, fever, pleurisy, dyspnea, and chronic cough. Diagnosis involves radiographic studies and pulmonary function testing. Treatment includes oral, aerosolized, or intravenous antibiotic therapy according to the severity of the exacerbation, and mucus clearance by means of bronchial hygiene assistive devices, chest physiotherapy, postural drainage, and high-frequency chest compression. We present a review of bronchiectasis and offer 3 case studies illustrating current management of different presentations, including use of aerosolized antibiotics for patients infected with Pseudomonas aeruginosa. Although an adjunctive program of pulmonary rehabilitation may be useful for patients with bronchiectasis, no confirming studies have been performed to date, and additional research in this area is warranted.

Adult↗

Bronchiectasis due to lipid aspiration in childhood: clinical and pathological correlates.

We report the clinical and pathological features in six Arab children with bronchiectasis caused by ghee lipid aspiration. They all had a history of ghee administration followed by a history of chronic cough dating from early childhood. Chest radiographs showed consolidation/collapse of the right middle and left lower lobes in the majority, and bronchography and chest CT scan confirmed bronchiectasis. The children were treated medically, without any improvement, and five required surgery. The histology of the lung revealed dilated bronchi filled with vacuolated granular eosinophilic material and the peripheral lung tissue was mostly atelectatic with patchy lymphocytic bronchiolitis. Frozen sections of the lung tissue showed scattered lipogranulomas with fat-laden macrophages and fat droplets within peripheral and perivascular lymphatics. In communities where the traditional practice of force-feeding infants and children with ghee exists, it may be an important predisposing cause of bronchiectasis.

Anti-Bacterial Agents↗

Bronchiectasis and rheumatoid disease: is there an association?

Rheumatoid arthritis is associated with a number of pleuropulmonary disorders. A retrospective study of the frequency of rheumatoid disease in patients with bronchiectasis and pulmonary fibrosis was performed. The results suggest that the frequency of bronchiectasis and rheumatoid disease is similar to that of the well established, but rare, association between pulmonary fibrosis and rheumatoid arthritis. We therefore suggest that bronchiectasis should be remembered as a pulmonary association of rheumatoid disease which occurs as commonly as pulmonary fibrosis.

Aged↗

HLA associations in subjects with rheumatoid arthritis and bronchiectasis but not with other pulmonary complications of rheumatoid disease.

We have examined HLA-DR, DQA and DQB variants in 72 controls, 153 subjects with RA without extra-articular features and in subjects with the rheumatoid pulmonary complications of interstitial fibrosis (23) peripheral airways disease (13) and in 41 subjects with RA and bronchiectasis. Subjects with RA alone showed the expected association with HLA-DR4 (79%) but those with RA and co-existent pulmonary fibrosis were less likely to be DR4 positive (61%). No other HLA-DR variants were significantly increased in the different disease groups. HLA-DQB1*0501 which types serologically as DQw1 was increased in subjects with RA and peripheral airways disease as compared to rheumatoid subjects with normal lung function, but these differences were not statistically significant. DQB1*0601 was increased in subjects with bronchiectasis with or without RA (but only significantly so in RA-BR subjects) DQB1*0301, DQB1*0201 and DQA1*0501 frequencies were also increased in subjects with RA and bronchiectasis as compared to those with RA alone.

Aged↗

Air cysts and bronchiectasis prevail in nondependent areas in severe acute respiratory distress syndrome: a computed tomographic study of ventilator-associated changes.

OBJECTIVE: To investigate prevalence and spatial distribution of air cysts and bronchiectasis associated with mechanical ventilation in patients with severe acute respiratory distress syndrome. DESIGN: Retrospective observational study. SETTING: University hospital intensive care division. SUBJECTS: A total of 21 patients with severe acute respiratory distress syndrome requiring prolonged mechanical ventilation and undergoing thoracic computed tomographic scanning. MEASUREMENTS AND MAIN RESULTS: Lung fields were anatomically divided according to functional bronchial divisions in ten segments on each side. Air cysts, bronchiectasis, and the percentage of normal and abnormal tissue were quantified for each segment and correlated to the duration and variables of mechanical ventilation. Air cysts prevailed in nondependent compared with dependent areas (38% +/- 5% vs. 9% +/- 3%, respectively; p <.01). With mechanical ventilation longer than 10 days, the odds ratio was 13.1 (95% confidence interval, 1.4-118.7) for air cysts and for bronchiectasis 17.6 (95% confidence interval, 2.8-109.6). The amount of abnormal parenchyma correlated with duration of mechanical ventilation on high end-inspiratory pressure but not with tidal volume or minute ventilation. The presence of pneumothorax was not correlated with ventilation settings but with the amount of consolidated tissue revealed by the computed tomographic scan. CONCLUSIONS: These findings suggest predominant ventilator-induced lung damage in better ventilated areas (i.e., nondependent regions). Severity of changes revealed by computed tomographic imaging seems to be associated mainly with the high inspiratory pressures required and the length of mechanical ventilation.

APACHE↗

Chronic lung sepsis: lung abscess, bronchiectasis, and empyema.

Chronic lung infections including lung abscess, bronchiectasis, and empyema remain serious medical diseases despite the availability of antimicrobial agents. Recent publications contribute to our understanding of disease pathogenesis and management of these entities. The microbial pathogen of lung abscess may be different comparing immunocompetent with immunocompromised patients, supporting a concept for aggressive diagnostic investigations and targeting of specific potential pathogens. The use of surgery in bronchiectasis may be declining, but surgical intervention remains an important strategy with favorable outcomes, especially in cases of focal bronchiectasis. For empyema, maintaining a high index of clinical suspicion may allow for diagnostic thoracentesis in patients with pleural effusions and allow initiation of appropriate tube thoracotomy drainage and use of fibrinolytics. With appropriate management, morbidity and mortality may be limited and outcomes generally favorable.

Anti-Bacterial Agents↗

Bronchiectasis in adult patients: an expression of heterozygosity for CFTR gene mutations?

While all patients with cystic fibrosis (CF) have mutations in both CFTR alleles, often only one CFTR change is detected in patients with other lung disorders. The aim of this study was to investigate whether heterozygosity for CFTR mutations could be a determinant risk factor in the development of bronchiectasis in adult patients. We have performed the CFTR gene analysis in a cohort of 55 bronchiectasis adult patients with unknown etiology. The 5T variant (TG)m and the M470V polymorphisms were also analyzed. A general population in which the same molecular analysis was previously performed was used as the control group. The mutational spectrum of patients was also compared with that found in our CF population. CFTR mutations/variants were found in 20 patients (36%), 14 with only one mutant gene (25%). All six patients colonized by Staphylococcus aureus presented with at least one CFTR change (p = 0.001). No statistical significance was observed between patients with and without mutations for other clinical features. The 5T variant was found in four patients. Additionally, 90% of patients with mutations had the more functional M470 allele (p < 0.001). These results suggest the involvement of the CFTR gene in bronchiectasis of unknown etiology in adult patients.

Adult↗

Investigation of Helicobacter pylori in bronchoscopic lung specimens of young male patients with bronchiectasis but without gastrointestinal symptoms.

Anti-Helicobacter pylori IgG serum levels were measured in 31 young male bronchiectasis patients without gastrointestinal symptoms, and 56 healthy males. The possible presence of H. pylori was also investigated by rapid urease tests, culture and histopathological examination of protected catheter brush and biopsy specimens from the bronchiectatic site. No serological, microbiological or histological evidence of H. pylori infection was found in the bronchiectasis patient group. H. pylori did not appear to be an agent of infection or chronic colonisation in bronchiectasis, and may not have a significant role in the progression of this disease.

Adult↗

Allergic bronchopulmonary aspergillosis and sensitization to Aspergillus fumigatus in chronic bronchiectasis in adults.

Fifty adult subjects for whom a diagnosis of idiopathic bronchiectasis (excluding those secondary to tuberculosis or hypogammaglobulinaemia) had been confirmed previously were investigated by: questionnaire; blood eosinophil count; sputum culture for Aspergillus fumigatus and eosinophil count; chest radiography; skin-prick tests with several aeroallergens and four preparations of A. fumigatus, including a reference extract; measurement of specific IgE antibodies; precipitin testing and self-crossed immunoelectrophoresis with A. fumigatus. Five subjects were possible cases of allergic bronchopulmonary aspergillosis in whom the condition had been previously misdiagnosed or in whom sensitization to A. fumigatus had occurred after the onset of bronchiectasis. These five subjects had positive immediate skin reactions to A. fumigatus and a history of recurrent pneumonias. Four had a previous history of asthma and the others showed increased bronchial responsiveness to inhaled methacholine. At the time of the survey, A. fumigatus grew in the sputum of one out of five subjects. These subjects had increased levels of specific IgE. Two had precipitins by double diffusion and three subjects were positive on self-crossed immunoelectrophoresis. It is concluded that allergic bronchopulmonary aspergillosis or evidence of sensitization to A. fumigatus can be identified in a significant proportion of adult subjects with so-called idiopathic bronchiectasis.

Adult↗

Subjective benefit of inhaled therapies in patients with bronchiectasis: a questionnaire study.

The objectives of this study were to obtain information about the use and subjective benefits of inhaled therapies in patients with bronchiectasis and to determine if these benefits were related to the presence of airflow obstruction. One hundred and twenty patients completed a questionnaire on inhaled therapies and performed spirometry. Inhalers were used daily by 85/120 patients with bronchiectasis and 42/120 patients had a nebuliser. Inhaled therapies were used extensively in patients with (FEV1 < 80%) and without (FEV1 > 80%) evidence of airflow obstruction. The majority of patients reported subjective benefits from their inhaled therapies. Chi2 analysis showed that there was no association between perceived benefits of short acting bronchodilators (SAB), long acting bronchodilators (LAB), corticosteroids or nebulisers and degree of airflow obstruction. This study provides information about the subjective benefits of inhaled therapies which could facilitate a more comprehensive assessment of the net benefit of inhaled therapies in patients with bronchiectasis with and without airflow obstruction.

Administration, Inhalation↗

Links between hospital diagnoses of bronchiectasis and asthma.

In view of the conflicting notions of the relationship between bronchiectasis and asthma, we have analysed the use of hospital services by bronchiectasis and asthma patients and evaluated the links between these diseases, employing data from the Finnish Hospital Discharge Register of over 21 million hospitalization periods recorded in 1972-92. We conclude that asthma is common in hospitalized bronchiectasis patients and appears to be consequent upon this disease.

Asthma↗

Familial middle lobe bronchiectasis.

A rare familial occurrence of middle lobe bronchiectasis is reported in two siblings of a family with seven children. No definite cause for the development of bronchiectasis could be established. Enlarged hilar lymph nodes were found in the resected middle lobe which were compressing the middle lobe bronchus, and this may have contributed to the development of bronchiectasis.

Adolescent↗

IFN gamma and CXCR-1 gene polymorphisms in idiopathic bronchiectasis.

Idiopathic bronchiectasis is a disease of chronic, bacterial lung infection, unresolving inflammation and progressive lung damage. Bronchiectasis can be associated with autoimmune diseases including ulcerative colitis. Defects of both innate and adaptive immunity have been proposed. The airway inflammation is characterized by interleukin-8 (IL-8) expression and infiltration by neutrophils and T cells. Here we investigated two candidate gene polymorphisms that may contribute to disease susceptibility: a CXCR-1 (+2607 G/C) gene polymorphism that is implicated in IL-8 binding and neutrophil trafficking as well as the interferon-gamma (IFNgamma) (+874 T/A) polymorphism which is linked to levels of IFNgamma production. These polymorphisms were distributed similarly in the idiopathic bronchiectasis group and controls, suggesting that these two candidate gene polymorphisms are not associated with disease susceptibility.

Alleles↗

Inhaled mannitol for the treatment of mucociliary dysfunction in patients with bronchiectasis: effect on lung function, health status and sputum.

OBJECTIVE: Inhaled mannitol increases mucus clearance in patients with bronchiectasis by an unclear mechanism. The effect of mannitol on lung function, health status and sputum properties was investigated. METHODOLOGY: Nine patients with bronchiectasis inhaled 400 mg of mannitol once daily for 12 days. Health status was assessed using the St George's Respiratory Questionnaire (SGRQ). Sputum was analysed for viscosity, elasticity, spinnability, surface tension, contact angle, solids, mucociliary transportability (MCTR) on a frog palate, and cough transportability (CTR) on a simulated cough machine. RESULTS: Lung function was unchanged with treatment (baseline FEV1 82.0 +/- 16.2%) apart from an improvement in FEF from 85.4 +/- 13% (baseline) to 90.7 +/- 14.4% (P < 0.05; 12th treatment; visit 7). The total SGRQ score (mean +/- SD) of 49.3 +/- 13.8 at baseline, decreased by 12.4 +/- 10.2 (P < 0.01; visit 7) and 10.1 +/- 9.4 units (P < 0.02) 6-10 days after treatment cessation. The baseline subscores for symptoms (72.9 +/- 11.8), activity (44.7 +/- 20.9) and impact (44.4 +/- 14.3) were reduced by 0.8 +/- 9.1 (P > 0.7), 8.4 +/- 16.0 (P > 0.1) and 19.2 +/- 13.7 (P < 0.005) units, respectively (visit 7). Mannitol reduced the baseline (mean +/- SE) surface tension from 94.5 +/- 1.4 to 84.7 +/- 2.1 mN/m (P < 0.0001), contact angle from 51.1 +/- 2.8 to 33.2 +/- 2.4 degrees (P < 0.0001), spinnability from 11.6 +/- 0.4 to 10.0 +/- 0.2 mm (P < 0.005), and solids from 5.7 +/- 0.4 to 4.3 +/- 0.7% (P < 0.02), acutely (visit 7). Viscosity, elasticity and MCTR did not change significantly, while CTR was increased from 25.8 +/- 1.0 to 34.1 +/- 2.7 mm (P < 0.003). CONCLUSION: Mannitol significantly improved the health status over 12 days and this improvement was maintained for 6-10 days after cessation of treatment. In addition, mannitol reduced the tenacity, increased the hydration of mucus acutely and improved cough clearability in patients with bronchiectasis.

Administration, Inhalation↗

Genome macrorestriction analysis of sequential Pseudomonas aeruginosa isolates from bronchiectasis patients without cystic fibrosis.

The respiratory tracts of bronchiectasis patients may be persistently colonized with Pseudomonas aeruginosa, despite intensive chemotherapy. The organism may undergo phenotypic changes in these patients, providing misleading typing results by conventional methods. We prospectively studied eight bronchiectasis patients without cystic fibrosis over a period of 1 year. A high microbial load of P. aeruginosa was found in 70% of sputum samples collected. Of these, 55 sequential P. aeruginosa isolates were characterized by a genotyping method, pulsed-field gel electrophoresis, to overcome the problem of differentiating the P. aeruginosa strains during chemotherapy. Genome macrorestriction fingerprinting patterns were analyzed after digestion with XbaI restriction endonuclease. Of the eight patients, six harbored a single dominant strain of P. aeruginosa, with an intrapatient macrorestriction similarity pattern range of 96 to 100%. The other two patients were infected with mixed bacterial isolates including P. aeruginosa. However, diversity was observed in the P. aeruginosa isolates from all eight patients, with a relatedness of only 55 to 65%. The study further strengthens the fact that pulsed-field gel electrophoresis can be used efficiently and effectively to differentiate P. aeruginosa strains in bronchiectasis patients without cystic fibrosis.

Adult↗