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Evaluation of nebulised hypertonic saline (7%) as an adjunct to physiotherapy in patients with stable bronchiectasis.

Sputum clearance is of prime importance in the management of patients with bronchiectasis. While nebulised normal isotonic saline (0.9%) (IS) has been anecdotally used to treat patients with tenacious sputum, the use of hypertonic saline (7%) (HS) could have potential muco-protective and clearance properties. 24 patients with bronchiectasis were randomised to receive four single treatment schedules in random order: (1) active cycle breathing technique (ACBT) alone, (2) nebulised terbutaline then ACBT, (3) nebulised terbutaline, nebulised IS then ACBT and (4) nebulised terbutaline, nebulised HS then ACBT. Sputum weights were significantly higher after HS than IS (P = 0.002). Ease of expectoration also differed overall (P < 0.0001) and was significantly lower with HS than with IS (P = 0.0005). Sputum viscosity differed between treatment phases, with a significant linear trend to reduced sputum viscosity with HS (P = 0.0002). These changes were associated with small but statistically significant differences in FEV1 (P = 0.043) and FVC (P = 0.011) between treatment phases. Nebulised hypertonic saline can be used safely and effectively as an adjunct to physiotherapy in selected patients. A long-term prospective trial is now indicated to determine its effectiveness on long-term infection rate, quality of life and lung function.

Aged↗

Exophiala dermatitidis infection in non-cystic fibrosis bronchiectasis.

A 54-year-old female presented with an exacerbation of right middle lobe bronchiectasis. A bronchoscopic bronchial washing and repeated trials of sputum culture consistently recovered no other infectious agent except Exophiala dermatitidis. Her illness was improved by administrations of intravenous miconazole and nebulized amphotericin B when sputum cultures yielded no fungi, demonstrating a pathogenic role of the fungi. The present case illustrates E. dermatitidis as a pathogenic agent in non-cystic fibrosis bronchiectasis.

Antifungal Agents↗

Surgical treatment for an unusual cause of localized bronchiectasis.

A 17-year-old girl presented with recurrent episodes of pneumonia related to localized bronchiectasis in the lingula. On computed tomography and magnetic resonance imaging, the cause of this was found to be an inwardly projecting exostosis arising from the left fifth rib. The patient underwent thoracotomy and excision of the exostosis with the affected area of lung. We report here the unusual case of a rib exostosis presenting with localized bronchiectasis.

Adolescent↗

Bilateral pulmonary resection for bronchiectasis: a 40-year experience.

Ninety-nine patients underwent bilateral pulmonary resection for severe multisegmental bilateral bronchiectasis at the Overholt Thoracic Clinic during the period 1937 to 1977. A total of 216 operations were performed, and 20 patients underwent three or more procedures. The operative mortality was 1.4% and the incidence of severe complications, 7%. Follow-up ranged from 1 to 30 years (average 10.2 years). Only 1 patient was lost to follow-up. Improvement in pulmonary symptoms was achieved in 83 patients; there was no improvement in 9 patients; and 4 patients were worse following resection. The results suggest that bilateral bronchiectasis need not be a contraindication to operation. In properly selected patients, lasting symptomatic improvement can be provided by resection.

Adolescent↗

Detection of bronchiectasis by high-resolution computed tomography in the yellow nail syndrome.

High resolution computed tomography of the lungs was performed in three patients with the yellow nail syndrome to detect bronchiectasis. Standard CT was performed in a fourth patient. Evidence of bronchiectasis with bronchial wall thickening and bronchial dilatation was observed in all four patients. High resolution CT is a non-invasive method for detecting and assessing the extent of airway disease in the yellow nail syndrome.

Aged↗

Severe teratospermia in an infertile man with bronchiectasis.

Morphologic examination of sperm samples from an infertile man with bronchiectasis revealed a severe teratospermia characterized by rare abnormalities that were present in most of his spermatozoa. These abnormalities were represented by round-headed spermatozoa, changes in chromatin condensation, acrosomal alterations, multiple tails, and complete subcellular derangement. The similarity between the above-reported abnormalities and those observed in various species of laboratory animals strongly suggests that the observed defects might be the result of a defective function of the manchette during spermiogenesis. The absence of specific defects of the axonemal morphologic features in sperm tails and in respiratory cilia, together with some laboratory data, suggests that association between infertility and bronchiectasis cannot be related, in our patient, to a generalized syndrome.

Adult↗

Association of HLA class I antigen deficiency related to a TAP2 gene mutation with familial bronchiectasis.

Two siblings with pansinusitis, nasal polyps, and bronchiectasis were found to have histocompatibility lymphocyte antigen (HLA) class I antigen deficiency ("bare lymphocyte syndrome") and dysfunction of natural killer cells. Reduced class I cell surface expression resulted from a single mutation in the TAP2 gene, which is located in the class II region of the major histocompatibility complex and encodes subunit 2 of the class I peptide transporter. The defect was transmitted in an autosomal recessive manner. This deficiency did not lead to severe viral infections but was apparently associated with susceptibility to bacterial infections of the respiratory mucosae. We suggest that class I HLA typing should be systematically performed in children with unexplained bronchiectasis.

Adolescent↗

Bronchiectasis in childhood.

Although bronchiectasis is relatively uncommon, it should be considered in a child with any of the following findings that persist: infiltrate on x-ray cough, purulent sputum, crackles or harsh breath sounds on auscultation of the chest, or hemoptysis. It is very likely that a pediatrician will encounter one or more children with this condition. This article deals with the pathogenesis, diagnosis, and treatment of bronchiectasis in childhood.

Age Factors↗

[Pulmonary nocardiosis in an immunocompetent patient with bronchiectasis].

We present a case of pulmonary nocardiosis in an immunocompetent patient with bronchiectasis in the middle lobe and lingula. The radiologic findings were pulmonary nodules, areas of consolidation, and multiple bronchiolar impactions. The patient died and the definitive diagnosis was confirmed at autopsy. Nocardiosis should be taken into account in patients with exacerbation of bronchiectasis.

Aged↗

CT of airways disease and bronchiectasis.

High-resolution CT is accepted as an accurate noninvasive means of diagnosing bronchiectasis. A wide spectrum of abnormalities may be identified at HRCT in patients with airway disease, including various distinctive patterns of bronchiectasis in specific clinical settings, such as ABPA, MAC infection, AIDS, and CF. Characteristic CT findings occasionally suggest a specific diagnosis that may not have been under clinical consideration. HRCT also provides significant clinical use in assessing the degree and extent of airway disease, and allows noninvasive monitoring of disease progression, regression, or response to therapy.

AIDS-Related Opportunistic Infections↗

Self-administered home intravenous antibiotic therapy in bronchiectasis and adult cystic fibrosis.

10 patients (mean age 23.1, range 17.1-40.5 years), 8 with cystic fibrosis (CF), and 2 with advanced bronchiectasis without CF, were taught, while being treated in hospital for exacerbations of pseudomonas infection, how to continue to give themselves intravenous antibiotics at home. They were discharged after satisfactory antibiotic levels had been achieved, and 22 courses were given at home over a total of 116 patient-days. In 14 of these, the greater part of the course was given at home (mean duration 6.6, range 5-10 days); 2 of these were given without admission to hospital. In the 8 patients with two or more infective exacerbations within a 12-month period there was no difference between home and hospital treatments in clinical improvement or in relapse time, defined as the interval between completion of treatment and subsequent antibiotic therapy. Self-administration of antibiotics intravenously at home for selected adults with cystic fibrosis and severe bronchiectasis reduces hospital stay and does not seem to be associated with an increased rate of recurrent infection.

Adolescent↗

[Bronchiectasis and rheumatoid arthritis. Incidence and etiopathogenic aspects. Review of the literature].

Many pleural and pulmonary manifestations are described in rheumatoid arthritis, due to the disease itself or drug-induced. A literature review revealed 289 reports of bronchiectasis since 1928, the respiratory symptoms preceding the articular features in 90%. Although the frequency of bronchiectasis in the general population is difficult to assess, and thus maybe underestimated, the prevalence in rheumatoid arthritis seems higher. Thus the recent studies, using modern processes such as high resolution computed tomography of the lungs, assess this association to about 25%. Nevertheless, the methodology of these studies is open to criticism, so the high frequency must be qualified. The pathogenic and aetiological factors remain unknown, and miscellaneous hypotheses, especially immunological, have been suggested. The relationship between both affections can not be asserted, but the association is unlikely to be fortuitous.

Arthritis, Rheumatoid↗

[PiSZ-phenotype alpha 1-antitrypsin deficiency: a rare cause of bronchiectasis].

Panacinar emphysema is the most characteristic pulmonary disease in patients with alpha1-antitrypsin deficiency (AAT). Bronchiectasis can also arise with AAT deficiency, although the association is much less common and no clear cause-effect relationship has yet been established. We report the case of a woman who presented with bronchiectasis and PiSZ-phenotype ATT deficiency, without emphysema. A review of the literature showed that the case is exceptional.

Bronchiectasis↗

[Renal AA amyloidosis secondary to bronchiectasis: a report of two cases (including one with Mounier-Kuhn syndrome)].

INTRODUCTION: We describe 2 patients with AA renal amyloidosis secondary to bronchiectasis (one patient had tracheobronchomegaly or Mounier-Kuhn syndrome). CASE REPORTS: The time period between the diagnosis of bronchiectasis and the development of renal amyloidosis was 40 years and 30 years respectively. Both patients evolved to end-stage renal failure requiring dialysis. CONCLUSION: Although the incidence of renal amyloidosis secondary to bronchectasis has declined, it remains a dreadful complication.

Aged↗

Radiological evidence of progression of bronchiectasis.

Radiological evidence of progression of bronchiectasis was sought in a group of 84 consecutive adult patients admitted to a tertiary unit with a particular interest in the disease. Methodical comparison for each patient of the earliest and most recent chest X-rays (n = 84), bronchograms (n = 1) and thoracic computed tomography (CT) scans (n = 32) was performed. Fifteen patients (18%) were considered to show radiological evidence of progression of bronchiectasis, 14 on chest X-ray, two of whom also showed progression on CT scans, and in one patient on bronchography alone. The likelihood of finding evidence of radiological progression increased the longer the interval between examinations. Serial radiology allows identification of patients with progression of disease and indicates the need to review management strategy.

Adolescent↗

Humidification as an adjunct to chest physiotherapy in aiding tracheo-bronchial clearance in patients with bronchiectasis.

Humidification of inspired air or oxygen is frequently utilized by respiratory physiotherapists to relieve sputum retention. Cold water, jet nebulizing humidifiers are in widespread use but there has been no previous attempt to investigate the efficacy of this treatment. We have performed a single-blind, cross-over study to quantify the effect of humidification as an adjunct to chest physiotherapy. Seven patients with bronchiectasis completed the trial, with a mean (range) age of 51 years (41-64 years) and mean (range) percent of predicted FEV1 of 46% (29-76%). On 2 days, separated by 1 week, subjects were randomly allocated to humidification or no humidification as a precursor to an optimal chest physiotherapy regimen. A radio-aerosol of human serum albumin millimicrospheres labelled with 99mTechnetium was used to measure sputum clearance by serial gamma camera images. Serial measurements of sputum weight and FEV1 were also recorded. Humidification (30 min) was followed by postural drainage (20 min) with the subject using the forced expiration technique to assist clearance. When humidification was combined with physiotherapy there was a significant increase in total wet weight of sputum (P less than 0.05) with a median (range) increase of 6 g (-9-15.5 g); and a significant increase in total radiolabel clearance (P less than 0.05) with a median (range) increase of 8.7% (1-13%), compared to physiotherapy alone. This study demonstrates that the use of cold water, jet nebulizing humidifiers significantly increases tracheo-bronchial clearance above that of an optimal physiotherapy regimen alone in bronchiectasis.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

Inhaled steroids in patients with bronchiectasis.

The effect of inhaled beclomethasone diproprionate (1500 micrograms day-1) on symptoms, pulmonary function and sputum production was examined in a double-blind, placebo-controlled, cross-over study in 20 patients with bronchiectasis. An 18% reduction in daily sputum production (P less than 0.003) was observed on treatment with inhaled steroid compared to placebo. A small, significant, improvement in morning peak expiratory flow rate (P less than 0.03) and forced expiratory volume in 1 s (P less than 0.03) was seen but the absolute changes are unlikely to be of clinical importance. Symptom scores for cough improved significantly (P less than 0.02). Inhaled steroids may have a role in the management of bronchiectasis by reducing cough and sputum production.

Administration, Inhalation↗

The use of probenecid to increase the serum amoxycillin levels in patients with bronchiectasis.

Amoxycillin 3 g twice daily is effective in treating patients with bronchiectasis who have daily purulent sputum. However, it is a relatively expensive treatment if used for prolonged periods. This pilot study in six patients with bronchiectasis showed that the concurrent administration of probenecid with a smaller dose of amoxycillin produced similar clinical responses and pharmacokinetic profiles to those found with high dose amoxycillin alone. The amount of amoxycillin used was reduced by two thirds, producing a potential saving of approximately pound 1000 per patient per annum in those taking long term treatment.

Adult↗