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PubMed1997

Bronchiectasis in bone marrow transplantation.

Two patients are described with clinical and radiographic bronchiectasis which occurred after allogeneic bone marrow transplantation for haematological malignancy. Both had evidence of chronic graft versus host disease in other organs. Increased immunosuppression with corticosteroids resulted in clinical response, although both patients persisted with chronic mucopurulent sputum production and one had progressive airflow obstruction. Bronchiectasis may be an under-recognised manifestation of chronic graft versus host disease of the lung.

Adult↗

PubMed1998

Bronchiectasis in association with coeliac disease.

A 48 year old woman presented with a history of fatigue, regular sputum production, and wheeze. High resolution computed tomographic scanning of the thorax demonstrated widespread bronchiectasis. Coeliac disease was diagnosed on the basis of an iron deficiency anaemia, subtotal villous atrophy on small bowel biopsy, and raised anti-gliadin and antiendomysial antibodies. The temporal relationship of the bronchiectasis and coeliac disease, and the subsequent stabilisation of her clinical symptoms and improvement in pulmonary physiology following treatment with inhaled corticosteroids, suggests a relationship between the two conditions which may be due to immunological mechanisms.

Albuterol↗

PubMed1999

Congenital broncho-oesophageal fistula associated with bronchiectasis in adults. Report of two cases and review of the literature.

Congenital broncho-oesophageal fistula is a rare entity in adult patients. This anomaly may cause various symptoms such as respiratory infections, coughing bouts when eating or drinking and even haemoptysis. Even rarer than its occurrence with the above-mentioned symptoms is its presentation with bronchiectasis. A congenital broncho-oesophageal fistula presenting with bronchiectasis in a 28-year-old male and 36-year-old female are described. In reported cases, symptoms of chronic recurrent pulmonary suppuration were initially attributed to alternative aetiologies. In both cases, with such an unusual presentation, the observation of the fistulous tract was coincidental. Surgical division of the fistula associated with lobectomy resulted in complete resolution of symptoms.

Adult↗

PubMed2001

The effect of erythromycin on mucociliary transportability and rheology of cystic fibrosis and bronchiectasis sputum.

BACKGROUND: Erythromycin has been shown to diminish sputum production in hypersecretory states by a mechanism that is still unclear. OBJECTIVES AND METHODS: We have investigated the effect of erythromycin on the ciliary transportability of cystic fibrosis and non-cystic fibrosis bronchiectasis sputum in vitro using the mucus-depleted bovine trachea. RESULTS: Additional erythromycin in concentrations up to 20 microg/g did not significantly alter the ciliary transportability of sputum from 6 cystic fibrosis and 6 bronchiectasis patients. Sputum viscoelasticity measured with parallel-plate rheology was also little changed. These erythromycin concentrations also had little effect on the beating frequency of bovine tracheal cilia. CONCLUSIONS: These results suggest that the presence of erythromycin in sputum neither alters the physical properties of the gel nor the activity of cilia. The clinical effects of erythromycin on pulmonary hypersecretory states therefore have another explanation.

Anti-Bacterial Agents↗

PubMed1979

Bronchiectasis: a cause of pulmonary symptoms in heroin addicts.

Extensive and severe bronchiectasis was found in 7 heroin-addicted individuals with pulmonary symptoms whose chest roentgenograms were not suggestive of severe airway disease. Abnormalities consisted of varicose and cylindrical alterations. Pulmonary function tests revealed airflow obstruction, decreased lung volumes, and diffusion capacity impairment. Arterial blood gas analysis demonstrated mild hypoxemia in all patients and chronic hypocapnia in 4. Serial pulmonary function tests in 2 patients revealed only modest improvement in the degree of airflow obstruction. The occurence of bronchiectasis appeared to be related to episodes of heroin-induced pulmonary edema and infection.

Adult↗

PubMed1984

Acute phase response in bronchiectasis and bronchus carcinoma.

Measurement of the acute phase response in patients suffering from bronchiectasis, emphysema, bronchus carcinoma and various benign space occupying lesions was undertaken, using sensitive immunoradiometric assays for C-reactive protein and serum amyloid-A protein. In some patients with bronchiectasis, clinically judged to be in remission, the results show a major ongoing acute phase response. Such a response could predispose these patients to the development of reactive secondary amyloidosis. In bronchus carcinoma, the application of these measurements to judge the extent of tumour growth is limited as infection complicating obstruction is a more potent initiator of the acute phase response than the neoplastic process per se.

Adenocarcinoma↗

PubMed1985

Acute phase response in bronchiectasis and bronchus carcinoma.

Measurement of the acute phase response in patients suffering from bronchiectasis, emphysema, bronchus carcinoma and various benign space-occupying lesions was undertaken, using sensitive immunoradiometric assays for C-reactive protein (CRP) and serum amyloid-A protein (SAA). In some patients with bronchiectasis, clinically judged to be in remission, the results show a major ongoing acute phase response. Such a response could predispose these patients to the development of reactive secondary amyloidosis. In bronchus carcinoma the application of these measurements to judge the extent of tumor growth is limited as infection-complicating obstruction is a more potent initiator of the acute phase response than the neoplastic process per se.

Amyloid↗

PubMed1991

Role of bromhexine in exacerbations of bronchiectasis. Double-blind randomized multicenter study versus placebo.

The effectiveness of bromhexine in the treatment of patients with bronchiectasis, in a stage of clinical exacerbation, was assessed in a double-blind, placebo-controlled trial involving 88 in-patients. Bronchiectasis was diagnosed by bronchography and/or CT scan. Bromhexine or matched placebo was administered as 30-mg capsules three times daily per os. Ceftazidine, 1 g i.m., was given to all patients once a day for the first week only. Bromhexine seemed to improve the clinical picture, with significantly positive trends for expectoration, quantity of sputum and auscultatory findings. It also increased the FEV1 and was well-tolerated. Both patients and investigators judged it efficacious.

Adult↗

PubMed1995

Long-term course of bronchiectasis and bronchiolitis obliterans as late complication of smoke inhalation.

We describe the long-term course of a patient with bronchiectasis and bronchiolitis obliterans, both of which developed as late complications of a smoke inhalation injury. Sequential chest X-rays obtained during the observation period showed gradual progression of bronchiectasis from the saccular to the cystic type. Symptoms, spirometry and blood gas analysis, however, remained stable for 15 years. We believe that symptoms and physiological derangement were due mainly to bronchiolitis obliterans, and that once the pathophysiological condition had been established following the initial injury, it could be maintained by conservative medical management.

Adult↗

PubMedDate not supplied

Comparison of oral ciprofloxacin and amoxycillin in treating infective exacerbations of bronchiectasis in Hong Kong.

A randomised double-blind controlled clinical trial was conducted to compare oral ciprofloxacin (500 mg b.d.) and amoxycillin (1 g t.d.s.) in the treatment of infective exacerbations of bronchiectasis. The commonest organism isolated from sputum was Pseudomonas aeruginosa, which accounted for 34% of all positive sputum cultures. Other Pseudomonas species and Haemophilus influenzae, accounted for 19%, respectively. Ciprofloxacin produced better clinical response, a higher sputum to serum antibiotic level (mean of 0.65 in the ciprofloxacin group vs. 0.18 in amoxycillin group, p = 0.0001), broader spectrum of antibacterial activity and less side-effect. It was also better tolerated by patients. The findings suggest that ciprofloxacin is an effective treatment of infective exacerbations of bronchiectasis.

Administration, Oral↗

PubMed1997

Inhaled gentamicin reduces airway neutrophil activity and mucus secretion in bronchiectasis.

To investigate whether aerosolized gentamicin (GM) prevents myeloperoxidase (MPO)-mediated airway injury and mucus hypersecretion, a short course of aerosol therapy (3 d) with GM 40 mg or 0.45% saline (saline) twice per day was conducted. Twenty-eight patients with bronchiectasis and mucus hypersecretion after adequate chest care and hydration were enrolled in a randomized, double-blind fashion. MPO levels in sputum collected on arising were determined by fluorometric assay at 655 nm before and after treatment. The sputum MPO level significantly decreased in patients receiving aerosolized GM, from 0.22 +/- 0.04 to 0.14 +/- 0.04 U/g (n = 15), but not in patients with saline inhalation (0.23 +/- 0.03 to 0.17 +/- 0.02 U/g; n = 11). The daily sputum amount significantly decreased from 94.6 +/- 21.6 to 58.1 +/- 17.8 ml (n = 13, p < 0.01) in the GM group, whereas it increased from 78.6 +/- 25.4 ml to 120.5 +/- 33.9 ml (n = 11, p < 0.05) in the saline group. The change in the amount of daily sputum was related to that in the sputum MPO level in the GM group (r = 0.61; p < 0.01). Inhalation of GM, but not saline, significantly (p < 0.05) increased the value of peak expiratory flow (PEF) from 186.4 +/- 25.1 to 216.4 +/- 26.4 L/min and decreased the variability of PEF from 24.6 +/- 5.1 to 6.1 +/- 2.3 %. The nocturnal desaturation and the 6-min walking distances were also significantly improved in the GM group (11.2 +/- 3.8 to 0.6 +/- 0.5 min/h; 324.9 +/- 43.1 to 408.1 +/- 25.9 m; p < 0.05; respectively), but not in the saline group. Subjective improvements in the Borg scale and self-sputum assessment were found in the GM group only. In conclusion, aerosolized GM is effective in improving airway hypersecretion and inflammation in patients with bronchiectasis.

Administration, Inhalation↗

PubMed1997

Validation of the St. George's Respiratory Questionnaire in bronchiectasis.

There is no disease-specific instrument available for health status assessment in bronchiectasis. We examined the stability, validity and responsiveness of a measure designed for asthma and COPD, the St. George's Respiratory Questionnaire (SGRQ), in this condition. One hundred and eleven patients were studied on 2 separate d 6 mo apart. On both days each patient completed the SGRQ and measures of general and disease-specific health, mood, and fatigue. They also performed a shuttle walking test and comprehensive lung function tests. Repeatability was tested over 2 wk in 23 patients. The intraclass correlation (ri) for the SGRQ Total score was 0.97. The SGRQ component scores correlated well with relevant markers of disease activity. Examples include: SGRQ Symptoms score versus MRC Wheeze score, r = 0.634, p < 0.0001; Activity score versus shuttle walking test, r = -0.659, p < 0.0001; and impacts score versus physical fatigue, r = 0.610, p < 0.0001. Changes in the SGRQ Total score from entry to follow-up also correlated with changes in other measures of the patients' health. There were significant differences in the SGRQ total score between patients who improved and those who deteriorated over the 6 mo in respect to wheeze (F = 5.6, p < 0.01) and breathlessness (F = 6.05, p < 0.01). We conclude that the SGRQ reflects impaired health in bronchiectasis patients.

Adult↗

PubMed1999

Dry powder versus intravenous and nebulized gentamicin in cystic fibrosis and bronchiectasis. A pilot study.

Aminoglycosides are a mainstay of therapy for patients with cystic fibrosis (CF) or non-CF bronchiectasis who are infected with Pseudomonas aeruginosa (Psa). Traditionally, aerosolized antibiotics are delivered by liquid nebulization. The objective of this study was to determine whether a gentamicin dry powder inhaler (DPI) is as microbiologically active and potentially safe as gentamicin inhaled via a small-volume nebulizer (SVN) or given intravenously. The study was done according to a randomized, single-dose, and triple crossover protocol. Ten patients with CF or non-CF bronchiectasis and chronically infected with Psa were recruited. Patients received a single dose of either gentamicin 160 mg via DPI or SVN, or gentamicin at 5 mg/kg by intravenous infusion. In seven of the 10 patients, the minimum inhibitory concentration (MIC) was achieved in sputum after DPI and SVN, with mean (95% confidence interval) gentamicin concentrations at 2 h after administration of 13.1 microgram/g sputum (range: 2.2 to 23.9 microgram/g) and 97.2 microgram/g sputum (range: 0.3 to 194.2 microgram/g), respectively, whereas gentamicin levels in the sputum after intravenous administration failed to reach the MIC. Gentamicin given by DPI and SVN significantly decreased the sputum Psa density (p < 0.05), by almost one order of magnitude. No significant decline in bacterial counts was observed after intravenous gentamicin. When gentamicin was inhaled, blood concentrations were minimal, and were below concentrations known to cause systemic toxicity. For treatment of Psa infections susceptible to gentamicin, gentamicin administration by DPI appeared to be as efficient as by SVN, despite the delivery of a 7-fold lower dose to the airways.

Aerosols↗

PubMed2000

Severe bronchiectasis in patients with "cystlike" structures within the ciliary shafts.

Cilia are minute hairlike structures which sweep the respiratory tract clean by continuous beating. Ciliary dysfunction can occur which leads to development of bronchiectasis. We describe the clinical and investigation profiles of four patients who had early-onset severe and otherwise idiopathic bronchiectasis associated with a previously undescribed ciliary defect in which numerous cystlike structures are present within the ciliary shafts. The patients had otherwise normal ciliary microtubular ultrastructure and central microtubular orientation. Our newly described ciliary abnormality appears to be a primary defect and more systematic studies should be performed to evaluate this further.

Adult↗

PubMed2000

Tobramycin solution for inhalation reduces sputum Pseudomonas aeruginosa density in bronchiectasis.

We conducted a placebo-controlled, double-blind, randomized study to evaluate the microbiological efficacy and safety of inhaled tobramycin for treatment of patients with bronchiectasis and Pseudomonas aeruginosa. Patients were randomly assigned to receive either tobramycin solution for inhalation (TSI) (n = 37) or placebo (n = 37), which was self-administered twice daily for 4 wk and followed by 2-wk off-drug. At Week 4, the TSI group had a mean decrease in P. aeruginosa density of 4.54 log(10) colony-forming units (cfu)/g sputum compared with no change in the placebo group (p < 0.01). At Week 6, P. aeruginosa was eradicated in 35% of TSI patients but was detected in all placebo patients. Investigators indicated that 62% of TSI patients showed an improved medical condition compared with 38% of placebo patients (odds ratio = 2.7, 95% confidence interval [CI] 1.1 to 6.9). Tobramycin-resistant P. aeruginosa strains developed in 11% of TSI patients and 3% of placebo patients (p = 0.36). The mean percent change in FEV(1) percent predicted from Week 0 to Week 4 was similar for the TSI and placebo groups (p = 0.41). More TSI-treated patients than placebo patients reported increased cough, dyspnea, wheezing, and noncardiac chest pain, but the symptoms did not limit therapy. Additional study is warranted to further evaluate TSI in bronchiectasis patients.

Administration, Inhalation↗

PubMed1992

Increased adherence of monocytes to fibronectin in bronchiectasis. Regulatory effects of bacterial lipopolysaccharide and role of CD11/CD18 integrins.

Regulated adherence of monocytes to extracellular matrix is a prerequisite for accumulation of mononuclear phagocytes during pulmonary infection and inflammation. We have obtained monocytes from patients with an inflammatory lung disease (bronchiectasis) and from control subjects and have compared their adherence to fibronectin. Spontaneous adherence of monocytes from the control subjects was 20 +/- 2%, whereas that of patients' cells was markedly higher and correlated with the severity of airway inflammation: 65 +/- 5% and 40 +/- 8% in patients with purulent and mucoid sputum, respectively. Endotoxin and cytokines from areas of airway disease are likely to be responsible for the observed monocyte activation, since: (1) endotoxin was detectable in all of the patients but in none of the control subjects; (2) LPS produced a dose-related increase in adherence of normal monocytes in vitro (maximal 65 +/- 2% adherence at 1 microgram/ml of LPS); (3) recombinant cytokines and LPS produced additive effects on monocyte adherence in vitro. The adherence of the patients' monocytes to fibronectin was substantially mediated by CD11/CD18 integrins, via both RGD-dependent and RGD-independent mechanisms. These data indicate that signals arising from foci of infection and inflammation can influence the adherence of monocytes, and they are likely to be determinants of the accumulation of mononuclear phagocytes in the lungs of patients with bronchiectasis.

Adult↗

PubMed1976

Probable familial congenital bronchiectasis due to cartilage deficiency (Williams-Campbell syndrome).

Two siblings in whom respiratory symptoms developed immediately after birth subsequently were found to have bronchiectasis with strikingly similar distribution of lesions (mainly lower lobes). Inspiratory and expiratory bronchograms performed on one of the siblings demonstrated marked ballooning and collapse of proximal bronchi during tidal breathing. The clinical courses and roentgenographic findings, in the absence of other underlying abnormalities, suggest that the basic disease process was the absence of bronchial cartilage (Williams-Campbell syndrome). This would be the first reported familial occurrence of this syndrome. The familial pattern and the neonatal onset of symptoms support the theory of a congenital basis for this variety of bronchiectasis.

Adolescent↗

PubMed1980

Abnormal cilia in Polynesians with bronchiectasis.

Bronchial or nasal ciliated epithelium from 13 patients with bronchiectasis (12 Polynesians and 1 European) was examined by electron microscopy. All cilia examined lacked dynein arams. In 2 patients, this was the only abnormality observed, but in the others, various ciliary abnormalities were present, including cilia with missing tubules, extra tubules, and misplaced tubules, as well as compound cilia, intracytoplasmic cilia, vesiculated cilia, and cilia with long winglike folds. In all of these patients pulmonary mucociliary transport rates were either absent or markedly reduced. It is suggested that these abnormalities were due to a mutation or group of mutations, which was the underlying cause of the bronchiectasis.

Adult↗