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PubMed2003

Sputum sol neutrophil elastase activity in bronchiectasis: differential modulation by syndecan-1.

The persistently dominant activity of neutrophil elastase in bronchial secretions replete with antielastases is crucial to the pathogenesis of bronchiectasis. We hypothesize that components in the bronchial secretions bind neutrophil elastase and compromise the inhibitory efficiency of prevailing antielastases. Zymographic analysis of sputum sols from patients with bronchiectasis found elastase activity in a polydisperse, alcian blue-stained zone of high molecular mass. This suggested that neutrophil elastase was complexed with polyanionic partners. Western blot analysis found not only the polyanionic partner, heparan sulfate/syndecan-1, but also the physiological antielastases, secretory leukoproteinase inhibitor and alpha1-antitrypsin, in the complex. Both dissociative density gradient ultracentrifugation and heparin displacement revealed that elastase dissociated from heparan sulfate/syndecan-1 was fully inhibited by the endogenous antielastases. This contrasts with the effects of exogenous antielastases on sputum neutrophil elastase activity-that of alpha1-antitrypsin was limited, but that of secretory leukoproteinase inhibitor was facilitated. Similarly, complexed elastase on blots of sputum sol zymographs was bound and inhibited by exogenous secretory leukoproteinase inhibitor but not by exogenous alpha1-antitrypsin. Taken together, the results bring a new focus to heparan sulfate/syndecan-1 complexed with neutrophil elastase in inflamed bronchial secretions as a target for modulating elastase susceptibility to physiological antielastases.

Blotting, Western↗

PubMed1997

Sodium chloride increases the ciliary transportability of cystic fibrosis and bronchiectasis sputum on the mucus-depleted bovine trachea.

Mucus retention in the lungs is an important feature of several respiratory diseases (Regnis, J.A., M. Robinson, D.L. Bailey, P. Cook, P. Hooper, H.K. Chan, I. Gonda, G. Bautovich, and P.T.P. Bye. 1994. Am. J. Respir. Crit. Care Med. 150:66-71 and Currie, D.C., D. Pavia, J.E. Agnew, M.T. Lopez-Vidriero, P.D. Diamond, P.J. Cole, and S.W. Clarke. 1987. Thorax. 42:126-130). On the mucus-depleted bovine trachea, the ciliary transport rate of sputum from patients with cystic fibrosis and bronchiectasis of other causes was slow, but the rate was doubled by increasing the sodium chloride content by 90 mM. Increasing the sputum osmolality by inspissation or by the addition of nonelectrolytes had a similar effect. The viscoelasticity of sputum, but not the bovine ciliary beat frequency, was markedly saline dependent over the pathophysiological range. This suggests that low mucus salinity, not (as is generally assumed) its under-hydration, contributes to its retention in bronchiectasis due to cystic fibrosis and other causes, probably by affecting its rheology. It also indicates how the genetic defect in cystic fibrosis might lead to impaired mucus clearance. Therapies that increase the osmolality of lung mucus might benefit patients with mucus retention.

Animals↗

PubMed2000

Carcinoid tumourlets associated with diffuse bronchiectasis and intralobar sequestration.

Innumerable carcinoid tumourlets may develop within pulmonary lobes should there be scarring from intralobar sequestration; these tumourlets may, in turn, be the cause of chronic lung disease. This report documents the incidental detection of multifocal carcinoid tumourlets in the lung of a 65-year-old man who had repeated episodes of lung infection, progressive dyspnea and haemoptysis; he lived at high altitude. The left lower lobe of the lung was resected surgically, during which procedure an aberrant systemic arterial supply was noticed. The patient had diffuse bronchiectasis and intralobar sequestration. The latter implies the development of abnormal lung tissue located within lobar tissue--but which does not communicate with the bronchial tree; it is supplied with arterial blood from a branch of the aorta--arising either above or below the diaphragm. There was loss of demarcation between the sequestered lung and the surrounding lower lobe lung parenchyma. The proliferation of pulmonary neuroendocrine cells in the form of tumourlets, had probably occurred as an adaptive response to the chronic hypoxia experienced. The combination of intralobar sequestration, bronchiectasis and carcinoid tumourlets, although uncommon, may arise when intralobar sequestration of the lung has not been resected at an incipient stage.

Aged↗

PubMed2003

Tidal expiratory flow limitation, dyspnoea and exercise capacity in patients with bilateral bronchiectasis.

In this study the authors investigated whether expiratory flow limitation (FL) is present during tidal breathing in patients with bilateral bronchiectasis (BB) and whether it is related to the severity of chronic dyspnoea (Medical Research Council (MRC) dyspnoea scale), exercise capacity (maximal mechanical power output (WRmax)) and severity of the disease, as assessed by high-resolution computed tomography (HRCT) scoring. Lung function, MRC dyspnoea, HRCT score, WRmax and FL were assessed in 23 stable caucasian patients (six males) aged 56 +/- 17 yrs. FL was assessed at rest both in seated and supine positions. To detect FL, the negative expiratory pressure (NEP) technique was used. The degree of FL was rated using a five-point FL score. WRmax was measured using a cyclo-ergometer. According to the NEP technique, five patients were FL during resting breathing when supine but not seated, four were FL both seated and supine, and 14 were NFL both seated and supine. Furthermore, it was shown that: 1) in stable BB patients FL during resting breathing is common, especially in the supine position; 2) the degree of MRC dyspnoea is closely related to the five-point FL score; 3) WRmax (% pred) is more closely correlated with the MRC dyspnoea score than with the five-point FL score; and 4) HRCT score is closely related to forced expiratory volume in one second % pred but not five-point FL score. In conclusion, flow limitation is common at rest in sitting and supine positions in patients with bilateral bronchiectasis. Flow limitation and reduced exercise capacity are both associated with more severe dyspnoea. Finally, high-resolution computed tomography scoring correlates best with forced expiratory volume in one second.

Adult↗

PubMed2006

Increased mortality from lung cancer and bronchiectasis in young adults after exposure to arsenic in utero and in early childhood.

Arsenic in drinking water is an established cause of lung cancer, and preliminary evidence suggests that ingested arsenic may also cause nonmalignant lung disease. Antofagasta is the second largest city in Chile and had a distinct period of very high arsenic exposure that began in 1958 and lasted until 1971, when an arsenic removal plant was installed. This unique exposure scenario provides a rare opportunity to investigate the long-term mortality impact of early-life arsenic exposure. In this study, we compared mortality rates in Antofagasta in the period 1989-2000 with those of the rest of Chile, focusing on subjects who were born during or just before the peak exposure period and who were 30-49 years of age at the time of death. For the birth cohort born just before the high-exposure period (1950-1957) and exposed in early childhood, the standardized mortality ratio (SMR) for lung cancer was 7.0 [95% confidence interval (CI), 5.4-8.9; p<0.001] and the SMR for bronchiectasis was 12.4 (95% CI, 3.3-31.7; p<0.001). For those born during the high-exposure period (1958-1970) with probable exposure in utero and early childhood, the corresponding SMRs were 6.1 (95% CI, 3.5-9.9; p<0.001) for lung cancer and 46.2 (95% CI, 21.1-87.7; p<0.001) for bronchiectasis. These findings suggest that exposure to arsenic in drinking water during early childhood or in utero has pronounced pulmonary effects, greatly increasing subsequent mortality in young adults from both malignant and nonmalignant lung disease.

Adult↗

PubMed1992

Systemic hypersensitivity vasculitis associated with bronchiectasis.

Systemic hypersensitivity vasculitis developed in a 53-year-old man during acute exacerbation of bronchiectasis infected with Pseudomonas aeruginosa. High grade fever, mononeuropathy multiplex, cutaneous vasculitis, and biopsy specimen-proved mesangioproliferative glomerulonephritis with crescent formation and leukocytoclastic vasculitis associated with circulating immune complex occurred. Corticosteroid and cyclophosphamide therapy was effective for vasculitis and bronchiectasis.

Acute Disease↗

PubMed1996

Is resection of bronchiectasis beneficial in patients with primary ciliary dyskinesia?

A retrospective study of 21 patients with primary ciliary dyskinesia (PCD) was done. Thirteen had prior resection of bronchiectasis and eight had not. Information about present complaints was obtained by a questionnaire. The prevalence of present respiratory symptoms was the same in both groups. The surgical patients had more severe disease and 85% of them considered the operation beneficial. Selected patients with PCD may have improved conditions with resection of bronchiectasis.

Adult↗

PubMed2002

Validation of the Hong Kong Chinese version of the St. George Respiratory Questionnaire in patients with bronchiectasis.

STUDY OBJECTIVES: To validate the Hong Kong Chinese version of the St. George Respiratory Questionnaire (SGRQ-HK) in patients with bronchiectasis. DESIGN AND SETTING: Outpatients (93 patients; 61 women; mean age [+/- SD], 59.0 +/- 14.2 years) were assessed at baseline by the SGRQ-HK, the Hong Kong Chinese version of the 36-item short form health survey (SF-36-HK), and the hospital anxiety and depression scale (HADS). Forty randomly selected patients also were reassessed at 2 weeks for repeatability. Seventy-two patients were further reassessed at 6 months for responsiveness. MEASUREMENTS AND RESULTS: Cronbach alpha coefficients, which reflected internal consistency, were > 0.7 for all SGRQ-HK components except for symptoms (alpha = 0.59), and the intraclass correlation coefficients between baseline and the 2-week follow-up visits were between 0.80 and 0.94 (p > 0.05). SGRQ-HK component scores and total scores correlated with all the component scores of the SF-36-HK and the HADS (p < 0.02). SGRQ-HK component scores and total scores correlated with the scores of the SF-36-HK and the HADS, confirming the concurrent validity. All SGRQ-HK scores correlated negatively with FEV(1), FVC, and arterial oxygen saturation (p < 0.005), while the activity score correlated with the Karnofsky performance scale and the number of bronchiectatic lobes (p < 0.001). SGRQ-HK scores positively correlated with Borg scale scores, exacerbation frequency, and 24-h sputum volumes (p < 0.03). Patients with 24-h sputum volumes of > or = 10 mL or < 10 mL had significantly different SQRG-HK component scores and total scores (p < 0.002), although this sensitivity was not displayed by scores on the HADS or the SF-36-HK. Patients with 25% reductions in 24-h sputum volumes had significant improvements in SGRQ-HK activity scores, impact scores, and total scores (p < 0.02), but not in other quality-of-life measures or clinical parameters, indicating the responsiveness of the SGRQ-HK. CONCLUSION: The SGRQ-HK is a valid and sensitive instrument for determining quality of life in bronchiectasis patients.

Bronchiectasis↗

PubMed1976

Bronchiectasis following heroin-induced pulmonary edema. Rapid clearing of pulmonary infiltrates.

We observed a patient who developed diffuse bronchiectasis subsequent to heroin-induced pulmonary edema. Unlike the previously reported cases, there was rapid clearing of pulmonary infiltrates and little evidence of severe aspiration. The development of bronchiectasis was attributed to a bronchial infection subsequent to clearing of the pulmonary edema. Physiologic dysfunction was characterized by marked obstruction, pulmonary hypertension, and mild hypoxemia.

Adult↗

PubMed1977

Alpha1-antitrypsin deficiency with diffuse bronchiectasis and cirrhosis of the liver.

The medical literature has emphasized repeatedly the association of alpha1-antitrypsin deficiency with panacinar emphysema and cirrhosis of the liver. Previous reports have linked bronchiectasis with alpha1-antitrypsin deficiency. The present case confirms this association and adds the presence of an hepatic abnormality characteristics of alpha1-antitrypsin deficiency to complete the syndrome. The patient's phenotype was found to be MDuarteZ. It has been suggested that patients with diffuse bronchiectasis should be screened for alpha1-antitrypsin deficiency. We would add that a complete assessment of hepatic function, including liver biopsy, should be carried out in those individuals with reduced levels of alpha1-antitrypsin.

Adolescent↗

PubMed1985

Chest percussion and postural drainage in patients with bronchiectasis.

Patients with chronic obstructive pulmonary disease have been treated routinely with chest physical therapy for many years in spite of a lack of scientific validation of this procedure. Only recently have the indications for chest physical therapy been clarified. It is currently believed that such therapy is especially beneficial in patients with copious secretions, and that it is less effective in patients with scanty secretions. No study has specifically evaluated the efficacy of chest physical therapy in patients with bronchiectasis. We accordingly evaluated 13 patients with stable bronchiectasis to determine the effects of chest physical therapy on pulmonary function, arterial oxygenation, and sputum production and to assess whether this therapy was associated with any significant side-effects. We found that chest physical therapy was safe and well tolerated and assisted the patients in mobilization of their sputum. However, such therapy had no immediate delayed effects on pulmonary function or oxygen saturation.

Adult↗

PubMed2001

Unilateral bronchiectasis and esophageal dysmotility in congenital adult tracheoesophageal fistula.

Tracheoesophageal fistulas (TEF) in adults are most commonly neoplastic, and very rarely congenital in nature. We report a 45-year-old Hispanic male with TEF and initial presentation of minimal hemoptysis. The patient had radiographic evidence of unilateral upper lobe (RUL) bronchiectasis, massive esophageal dilatation, and dysmotility. However, there was no evidence of esophageal malignancy, achalasia, or Chagas' disease. Bronchoscopy revealed a large TEF in the posterior wall of trachea, which was not visualized on esophagram or esophagoscopy. Bronchoalveolar lavage (BAL) cultures grew Mycobacterium avium complex (MAC). Our report illustrates that idiopathic, or congenital, TEF can be associated with esophageal dysmotility, adulthood bronchiectasis, and atypical mycobacterial superinfection.

Bronchiectasis↗

PubMed1993

Bronchiectasis: CT evaluation.

CT is the imaging method of choice after standard chest radiography for examining patients with suspected bronchiectasis. In most institutions throughout the world, CT has largely eliminated the need for bronchography in the diagnosis of bronchiectasis. Nonetheless, controversy persists concerning the overall accuracy of CT. In an effort to improve overall diagnostic accuracy, we review the wide range of CT appearances of this protean disorder, and emphasize potential problems and technical pitfalls that may arise in routine clinical imaging.

Bronchiectasis↗

PubMed2003

Multidetector CT of bronchiectasis: effect of radiation dose on image quality.

OBJECTIVE: The purpose of our prospective study was to assess the image quality with respect to the radiation dose incurred by multidetector CT (MDCT) in patients with suspected bronchiectasis. SUBJECTS AND METHODS. Image clusters, composed of nine images, using MDCT (120 kVp, a 2.5-mm collimation, a pitch of 6, and 2.5-mm reconstruction intervals) were obtained at each of two levels-the azygous arch and the right inferior pulmonary vein-at 170, 100, 70, 40, 20, and 10 mA. Independently, two chest radiologists assessed and compared the quality of the images obtained at the six milliamperage exposures. Image quality was graded using a 5-point scale with lung and mediastinal window settings. Radiation doses were measured at each of the six milliamperage settings while scanning the whole lung of a thoracic phantom using MDCT. RESULTS: The mean image quality scores at exposures of 170, 100, 70, 40, 20, 10 mA were as follows: 3.9, 3.7, 3.8, 3.2, 2.5, 1.6 at lung window settings and 4.1, 4.3, 4.0, 3.4, 2.3, 1.3 at mediastinal window settings, respectively. Images obtained at 70 mA were rated significantly better than those obtained at 40 mA or less (p < 0.01). The mean radiation dose at 170, 100, 70, 40, 20, 10 mA was 23.72, 14.39, 10.54, 5.41, 2.74, and 1.50 mGy, respectively. CONCLUSION: With a tube current setting as low as 70 mA, MDCT provides images of acceptable quality and volumetric data sets for the evaluation of bronchiectasis. The trade-off of using MDCT rather than conventional high-resolution CT is that the radiation dose is five times higher with MDCT (10.54 mGy) than with conventional high-resolution CT (2.17 mGy with parameters of 120 kVp, 170 mA, 1-mm collimation, and 10-mm intervals).

Adult↗

PubMed1982

Results of pulmonary resection in cases of unilateral bronchiectasis.

Follow-up studies of 22 patients treated surgically for unilateral bronchiectasis showed that all patients were improved postoperatively following an average observation time of 9 years, 50% being completely free of respiratory symptoms. In all patients the number of days of recurrent illness had declined significantly after surgery. As mortality and morbidity rates in this kind of surgery are not negligible, a most careful pre-operative selection of patients suffering from bronchiectasis is indicated.

Adolescent↗

PubMed1998

[A case of bronchiectasis with abundant expectoration of Mycobacterium gordonae].

A 68 year old woman consulted our hospital because of 6 month history of dry cough. Her chest X-ray revealed bronchiectasis in the left lung. Three sputum specimens were culture positive for acid fast bacilli (AFB) 200 colonies. The bacilli were scotochromogenic, niacin test was negative and hydrolysis of tween 80 was positive, and they were identified as M. gordonae. M. gordonae is rarely implicated as a pathogen but a few reports suggest it may be pathogenic. Though our patient had clinical symptom and expected abundant M. gordonae, her chest X-ray revealed no progression. Thus, it was hardly possible to consider this case as a case caused by the infection with M. gordonae. In previous reports of M. gordonae pulmonary disease, chest X-ray findings showed cavity, infiltration and consolidation, however, no case with bronchiectasis was reported and chest X-ray findings of this case are interesting.

Aged↗

PubMed1998

Chest physiotherapy does not exacerbate gastroesophageal reflux in patients with chronic bronchitis and bronchiectasis.

BACKGROUND: Chest physiotherapy is beneficial to patients with mucus hypersecretion. However, it is not risk-free. Chest physiotherapy may affect gastrointestinal motility and the competence of the gastroesophageal sphincter during the procedure. Our study was aimed to investigate whether gastroesophageal reflux is exacerbated or induced by chest physiotherapy. MATERIALS AND METHODS: Thirty-two adult patients with chronic bronchitis or bronchiectasis received chest physiotherapy. An esophageal pH meter was placed 5 cm above the gastroesophageal junction and monitored the reflux episodes from 8 AM on the first day to 12 noon on the second day. Physiotherapy was performed with the patient in three different positions on the second day for one hour. RESULTS: Thirteen patients were found to have gastroesophageal reflux disease. For the study group as a whole, neither the frequency nor the duration of having a pH < 4 during the physiotherapy was significantly different from the mean hourly frequency and mean hourly duration recorded on the first day or from those values recorded at a corresponding time on the first day. As for the patients with gastroesophageal reflux disease, the frequency and total duration of gastroesophageal reflux during physiotherapy were not significantly different from the mean hourly frequency and the mean hourly duration or from the values recorded at the corresponding time on the first day. There was no significant difference in the gastroesophageal reflux frequency or the duration in different body positions. CONCLUSION: Chest physiotherapy including postural drainage, percussion and forced expiration techniques in different positions did not induce or increase the incidence of gastroesophageal reflux in patients with chronic bronchitis and bronchiectasis.

Adult↗

PubMed1999

[Bronchiectasis].

Obstructive impairment of pulmonary function in bronchiectasis depends on the number of segments involved in the bronchiectatic process and severity of the morphologic abnormalities in the region of the dilated bronchi. There is no obstructive change in bronchiectasis with single segmental lesion without fatal cases with multiple dilatation of bronchi. Immotile cilia syndrome, Kartagener syndrome, diffuse panbronchiolitis and cystic fibrosis can result from a multitude of heredity were observed in the obstructive impairment. A case of panbronchiolitis obliterans with diffuse cystic dilatation of bronchi due to mycoplasma pneumoniae infection was presented in severe obstructive disturbance.

Bronchi↗