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Inhaled hyperosmolar agents for bronchiectasis.

BACKGROUND: Mucus retention in the lungs is a prominent feature of bronchiectasis. The stagnant mucus becomes chronically colonised with bacteria, which elicit a host neutrophilic response. This fails to eliminate the bacteria, and the large concentration of host-derived protease may contribute to the airway damage. The sensation of retained mucus is itself a cause of suffering, and the failure to maintain airway sterility probably contributes to the frequent respiratory infections experienced by many patients. Hypertonic saline inhalation is known to accelerate tracheobronchial clearance in many conditions, probably by inducing a liquid flux into the airway surface, which alters mucus rheology in a way favourable to mucociliary clearance. Inhaled dry powder mannitol has a similar effect. Such agents are an attractive approach to the problem of mucostasis, and deserve further clinical evaluation. OBJECTIVES: To determine whether inhaled hyperosmolar substances are efficacious in the treatment of bronchiectasis SEARCH STRATEGY: MEDLINE and Cochrane databases were searched, and leaders in the field contacted. SELECTION CRITERIA: Any trial using hyperosmolar inhalation in patients with bronchiectasis not caused by cystic fibrosis. DATA COLLECTION AND ANALYSIS: One reference were identified by the searches conducted. MAIN RESULTS: Only one trial was identified, a crossover study of 11 patients with bronchiectasis. The outcome measure was tracheobronchial clearance of a particulate radioaerosol after inhalation of dry mannitol on a single occasion, with appropriate controls. Airway clearance doubled in the central and intermediate regions of the lung, but not in the peripheral region, after mannitol administration. No side effects were observed, but two patients were premedicated with nedocromil to prevent bronchospasm. REVIEWER'S CONCLUSIONS: Dry powder mannitol has been shown to improve tracheobronchial clearance in bronchiectasis, as well as cystic fibrosis, asthmatics, and normal subjects. It is not yet available for clinical use. Hypertonic saline has not been specifically tested in bronchiectasis, but improve clearance in these other conditions and in chronic bronchitis. Longer term randomised controlled studies of mannitol and hypertonic saline with clinical endpoints are now needed.

Bronchiectasis↗

Mucolytics for bronchiectasis.

BACKGROUND: Bronchiectasis is usually characterised by the production of large quantities of sputum that patients frequently have difficulty in expectorating. Mucolytic agents target hyper-secretion or changed physiochemical properties of sputum to make it easier to clear. One drug, recombinant human DNase, breaks down the DNA that is released at the site of infection by neutrophils. OBJECTIVES: The objective of this review was to assess the effects of ingested or inhaled mucolytics in patients with bronchiectasis. SEARCH STRATEGY: We searched the Cochrane Airways Group trials register, reference lists of relevant articles. We also contacted experts in the field and drug companies. SELECTION CRITERIA: Randomised trials of mucolytic treatment in people with bronchiectasis but not cystic fibrosis. DATA COLLECTION AND ANALYSIS: Data extraction was performed independently by two reviewers. Study authors were contacted for confirmation. MAIN RESULTS: Two trials were included. In one study, compared to placebo, high doses of bromhexine combined with antibiotics eased difficulty in expectoration (weighted mean difference -0.53, 95% confidence interval -0.81 to -0.25 at 16 days). There was also a reduction in sputum production with bromhexine (weighted mean difference -21.5%, 95% confidence interval -38.9 to -4.1 at day 16). There was no difference in forced expiratory volume. In a second study, compared to placebo, recombinant human DNase showed no difference in forced expiratory volume or forced vital capacity. Adverse effects, including influenza-like symptoms, were more common in the group receiving recombinant human DNase. REVIEWER'S CONCLUSIONS: There is not enough evidence to evaluate the routine use of mucolytics for bronchiectasis. High doses of bromhexine coupled with antibiotics may help with sputum production and clearance.

Anti-Bacterial Agents↗

Airways obstruction and bronchiectasis: correlation with duration of symptoms and extent of bronchiectasis on computed tomography.

Many patients with bronchiectasis have diffuse airways obstruction and this may be the dominant symptom. This study was performed to assess whether the severity of airway obstruction in these patients is related to duration of bronchiectatic symptoms or to the extent of bronchiectasis demonstrated by computed tomography (CT). Twenty-six patients were studied. The severity of airflow obstruction was measured by standard physiological testing and the extent of bronchiectasis demonstrated by CT was assessed using a scoring system. Correlation was measured by Kendall's rank correlation coefficient. A highly significant correlation between FEV1 and CT score was demonstrated and a significant correlation was also observed between FEV1 and duration of symptoms. A weak correlation was present between CT score and symptom duration.

Adult↗

The surgical management of childhood bronchiectasis. A review of 96 consecutive pulmonary resections in children with nontuberculous bronchiectasis.

In 195 children with nontuberculous bronchiectasis, periodic bronchography and clinical examinations were conducted over a period of 16 years (average 9.4 years). This was provided a critical assessment of surgical accomplishments in 96 consecutive resections and a parallel observation of 111 cases not submitted to resection. The final clinical assessment of the surgical cases shows 75% to be well or much improved, 22% to be improved, and 4% unchanged, while patients not submitted to resection have remained largely unchanged (69%) or have become worse (23%). The isolated superior segment can be preserved in children with good results, provided there is clear bronchographic evidence that the segment is entirely free of disease. When partially diseased segments are retained and required to fill a large volume, there is a tendency for even slightly altered bronchi to deteriorate postoperatively. Serial bronchography has proved helpful in determining when the disease has reached a mature, stable state and in planning the extent of resection.

Adolescent↗

Bronchiectasis-related amyloidosis as a cause of chronic renal failure.

Bronchiectasis is defined as acquired and permanent abnormal dilation and destruction of the bronchial walls. Secondary amyloidosis is a disorder characterized by the deposition of amyloid A (AA) in multiple organs and tissues in the body. End-stage renal disease (ESRD) secondary to bronchiectasis-related amyloidosis has only been mentioned in case reports. Little is known about the complications of bronchiectasis-related amyloidosis and the outcomes in patients who develop ESRD due to amyloid deposition in the kidneys. The aim of this study was to identify the clinical characteristics of this patient group, and to report the outcomes of these cases relative to bronchiechtasis type. We assessed the records of 40 patients with AA-type amyloid nephropathy and ESRD who were on hemodialysis (HD) at Baskent University Hospital between 1997 and 2000. The diagnosis of amyloidosis was based on histopathological findings in kidney, rectum, bone marrow, lymph node, thyroid, bladder, liver, and stomach biopsies. Bronchiectasis was diagnosed on the basis of history and findings on physical examination, chest X-ray, and thoracic high-resolution computerized tomography (HRCT). The patients' records were retrospectively evaluated for cause of secondary amyloidosis, and cases with causes other than bronchiectasis were excluded. Secondary amyloidosis due to bronchiectasis and recurrent pulmonary infection was identified in 40% (16 patients) of the 40 patients. For each of these 16 cases, we recorded patient age, duration of bronchiectasis, duration of HD, amount of lung involvement, and biopsy site(s). The means for age, duration of bronchiectasis, and duration of HD in the 16 patients were 50.6 +/- 13.5 years, 22.18 +/- 12.02 years (range, 6-42 years), and 30.81 +/- 36.94 months (range, 4-144 months), respectively. The most common biopsy site was the rectum (n = 8). Thoracic HRCT revealed cystic bronchiectasis in 8 cases (50%). Four of these 8 patients (25% of the group of 16) died from sepsis within 3-year follow-up period. Two of the four patients who died had bilateral cystic bronchiectasis, and the other two had unilateral cystic bronchiectasis. In the other eight patients in the group, thoracic HRCT showed chronic fibrotic changes in the pulmonary parenchyma and minimal traction bronchiectasis. Four of these patients exhibited apical fibrosis and bronchiectasis (25% of the group of 16), and these radiological findings were considered sequelae of previous tuberculosis infections. In conclusion, chronic respiratory infections and associated bronchiectasis remain a serious problem in Turkey due to insufficient prevention, diagnosis, and treatment. It is important that patients with progressive cystic and diffuse bronchiectasis be followed carefully, as they may develop amyloidosis and ESRD in time. Also, the clinical course in patients with cystic bronchiectasis may be worse than that in other types of bronchiectasis due to complicating pulmonary infections.

Adult↗

CT findings in bronchiectasis: limited value in distinguishing between idiopathic and specific types.

OBJECTIVE: The purpose of this study was to determine whether the pattern and distribution of bronchiectasis shown on CT scans can be used to discriminate between idiopathic cases and those with an identifiable cause. MATERIALS AND METHODS: The CT scans of 168 patients with chronic purulent sputum production and who were suspected of having bronchiectasis were analyzed (117 patients with idiopathic bronchiectasis, 15 with allergic bronchopulmonary aspergillosis, 15 with hypogammaglobulinemia, 15 with impaired mucociliary clearance, and seven with cystic fibrosis diagnosed in adult life). The scans were analyzed in random order by two observers. The extent, site, type, and lobar distribution of bronchiectasis and the severity of bronchial dilatation and bronchial wall thickening were scored. The frequency of these features in the known-cause groups was compared with that in the idiopathic group to identify any significant differences. RESULTS: Compared with idiopathic bronchiectasis, no significant lobar predominance was seen in any of the known-cause groups, apart from a higher frequency of lower lobe involvement in the patients with syndromes of impaired mucociliary clearance (p < .02). The bronchiectasis of allergic bronchopulmonary aspergillosis and adult cystic fibrosis was more often widespread (five or six lobes involved (p < .001 and p < .01, respectively) than idiopathic bronchiectasis. Central bronchiectasis was more common in allergic bronchopulmonary aspergillosis (p < .005), although the sensitivity when this was used as a diagnostic feature was only 37%. In all groups, cylindrical bronchiectasis was the most common type, with varicose and cystic bronchiectasis occurring more frequently in allergic bronchopulmonary aspergillosis (p < .01). On multiple regression analysis, allergic bronchopulmonary aspergillosis and adult cystic fibrosis showed more extensive disease than idiopathic bronchiectasis (p < .0005 and p < .001, respectively), independent of other CT features. In hypogammaglobulinemia, dilatation of the bronchial lumen was less than in idiopathic bronchiectasis (p < .02) independent of disease extent and bronchial wall thickness. CONCLUSION: Although differences in distribution and morphology of bronchiectasis may be seen on CT scans in groups of patients with bronchiectasis of different causes, CT findings applied to individual patients are of limited value in discriminating between idiopathic bronchiectasis and bronchiectasis of various known causes.

Adolescent↗

Diagnosis of bronchiectasis with multislice spiral CT: accuracy of 3-mm-thick structured sections.

The aim of this study was to evaluate the accuracy of 3-mm-thick reconstructed sections in the diagnosis of bronchiectasis with multislice CT (MSCT). Forty consecutive patients suspected of bronchiectasis (23 females, 17 males; mean age 51 years) underwent MSCT of the entire thorax with a 4x1-mm collimation (120 kV, 0.5 s/rotation, 80 mAs/slice) and a pitch of 1.75. From each data set (mean z-axis coverage: 257 mm; mean duration: 21 s), two series of images were systematically generated: 1-mm (group 1) and 3-mm (group 2)-thick reconstructed scans. Both series of images were obtained at 10-mm intervals and reconstructed with a high-spatial-frequency algorithm. Two observers independently analyzed the presence of bronchiectasis and associated abnormalities in group-1 and group-2 lung images. No significant difference between group 1 and group 2 was found in: (a) the detection of bronchiectasis, identified in 24 patients (60%) in group 1 and in 23 patients (57.5%) in group 2 ( p=0.08); (b) the evaluation of the extent of bronchiectasis, identifying focal bronchiectasis in 10 patients (25%) in group 1 and 7 patients (17.5%) in group 2 ( p=0.39) and multifocal bronchiectasis in 16 patients (40%) in both groups; (c) the characterisation of bronchiectasis (cylindral bronchiectasis: group 1, n=24, 60%; group 2, n=21, 53%, p=0.08); varicose bronchiectasis: group 1, n=5, 12.5%; group 2, n=6, 15%, p=0.56); and cystic bronchiectasis: group 1, n=2, 5%; group 2, n=2, 5%). Apart from the identification of abnormal bronchial wall thickening (group 2, n=35, 87.5%, vs group 1, n=31, 77.5%, p<0.05), recognition of associated bronchopulmonary anomalies did not differ between the two groups. This study demonstrates a comparable accuracy of the 3- and 1-mm-thick reconstructed scans in the detection and characterization of bronchiectasis. These results suggest the potential usefulness of 3-mm-thick scans generated from 4x2.5-mm acquisitions in the screening of bronchiectasis, which would allow a 20% radiation dose reduction compared with the present investigation.

Adult↗

Value of medium-thickness CT in the diagnosis of bronchiectasis.

The usefulness of CT with 4-mm-thickness cuts at 5-mm intervals in the diagnosis of bronchiectasis was studied by comparing the results of CT with those of bronchography in 323 segmental bronchi in 20 patients. No bronchiectasis was found on either study in 222 segmental bronchi. Of the 101 segmental bronchi showing bronchiectasis on bronchography, CT correctly identified bronchiectasis in 98 segments (97%). Bronchography showed cystic bronchiectasis in 35 and varicose bronchiectasis in 14 segmental bronchi, all of which were correctly identified on CT. Of the 52 segmental bronchi showing cylindrical bronchiectasis on bronchography, CT correctly identified these changes in 49 segments but failed to detect it in three. The presence of interstitial lung disease with emphysema and very localized mild bronchiectasis in one case each made the identification of cylindrical bronchiectasis difficult and caused the false-negative results. When bronchography is used as the criterion, CT with medium-thickness cuts and medium slice intervals appears to be 100% specific for all types of bronchiectasis, 100% sensitive for cystic and varicose bronchiectasis, and 94% sensitive for cylindrical bronchiectasis.

Adolescent↗

Correlation of CT findings with clinical evaluations in 261 patients with symptomatic bronchiectasis.

OBJECTIVE: In a multicenter study, we evaluated the relationships between the extent and severity of bronchiectasis on CT and clinical symptoms, spirometric abnormality, and sputum characteristics. SUBJECTS AND METHODS: The study population included 261 patients with symptomatic, physiologically significant bronchiectasis, who were enrolled in another study evaluating the clinical efficacy of deoxyribonudease in treatment of bronchiectasis. Patients with cystic fibrosis, allergic bronchopulmonary aspergillosis, and fungal or mycobacterial infection were excluded. In addition to high-resolution CT scanning, all patients underwent clinical evaluation, spirometry, and sputum culture. CT features scored by consensus of two observers included the extent of bronchiectasis, type of bronchiectasis (cylindric, varicose, or cystic), extent of mucoid impaction, and degree of bronchial wall thickening. RESULTS: Scores for the severity and extent of bronchiectasis correlated with the forced expiratory volume in 1 sec (FEV1) (r = -.362, p < .0001) and with the forced vital capacity (FVC) (r = -.362, p < .0001). Scores for bronchial wall thickening correlated with the FEV1 (r = -.367, p < .0001) and FVC (r = -.239, p < .001). Patients with cystic bronchiectasis were significantly more likely to grow Pseudomonas from their sputa and to have purulent sputa than were patients with cylindric or varicose bronchiectasis. Patients with cystic bronchiectasis had significantly lower FEV1 and FVC values than did patients with cylindric or varicose bronchiectasis. CONCLUSION: In this patient population, we found weak but significant correlations between the degree of morphologic abnormality on CT and the extent of physiologic impairment. Cystic bronchiectasis was associated with sputum purulence and with the growth of Pseudomonas. CT classification of the type of bronchiectasis may be useful as an index of severity of disease.

Bronchi↗

Increases in CD4+ T lymphocytes, macrophages, neutrophils and interleukin 8 positive cells in the airways of patients with bronchiectasis.

BACKGROUND: Bronchiectasis is a chronic suppurative lung disease characterised by irreversible dilation of the bronchi and persistent purulent sputum. The immunopathology of the disease was studied using a quantitative immunostaining technique with particular reference to T lymphocytes, macrophages, and granulocytes. METHODS: Bronchial mucosal biopsy specimens were obtained by fibreoptic bronchoscopy from 12 patients with bronchiectasis (six receiving inhaled steroids) and 11 normal healthy controls. Immunostaining (APAAP method) was performed on frozen cryostat sections with a panel of monoclonal antibodies to total leucocytes (CD45), T lymphocyte phenotypic markers (CD3, CD4, CD8), macrophages (CD68), eosinophils (EG2), and neutrophils (elastase). RESULTS: There was a mononuclear cell infiltrate in both patients with bronchiectasis and normal controls, but an overall increase in total leucocyte cell numbers (CD45+ cells) was identified in those with bronchiectasis (median values 422 cells/mm2 versus 113 cells/mm2 in control tissue, p < 0.001). Intense infiltration of CD3+ T lymphocytes was observed compared with healthy controls (292 cells/mm2 and 40 cells/mm2, respectively, p < 0.001). This comprised predominantly CD4+ T cells (118 cells/mm2) rather than CD8+ T cells (47 cells/mm2). CD3+ cells counts were reduced in those subjects on inhaled steroids compared with those not receiving inhaled steroids (197 cells/mm2 versus 369 cells/mm2, p < 0.05), as were CD4+ cell counts (82 cells/mm2 versus 190 cells/mm2, p < 0.05). Neutrophil and macrophage cell numbers were also increased in patients with bronchiectasis (114 cells/mm2 and 213 cells/mm2, respectively) compared with controls (41 neutrophils/mm2 and 40 macrophages/mm2). EG2+ (activated) eosinophil numbers were much lower than T cells, macrophages, and neutrophils in patients with bronchiectasis but were increased compared with controls (36 cells/mm2 versus 0 cells/mm2, p < 0.001). In view of the markedly increased neutrophil counts in patients with bronchiectasis, biopsy specimens were immunostained for interleukin 8 (IL-8) which was highly significantly increased compared with controls (47 cells/mm2 versus 15 cells/mm2, p < 0.01). IL-8+ cells were less prominent in steroid treated patients than in patients not receiving treatment (30 cells/mm2 versus 60 cells/mm2, p < 0.05). A further characteristic of bronchiectasis was mucous gland hypertrophy. Gland area comprised up to 40% of the tissue in some bronchiectasis sections while no hypertrophy was noted in control biopsy specimens (p < 0.05). CONCLUSION: Airway inflammation in bronchiectasis is characterised by tissue neutrophilia, a mononuclear cell infiltrate composed mainly of CD4+ T cells and CD68+ macrophages, and increased IL-8 expression. Inhaled corticosteroid treatment in patients with bronchiectasis is associated with a less marked infiltration by T cells and IL-8+ cells within the bronchial mucosa, although this finding requires confirmation in a prospective placebo controlled trial.

Adult↗

Bronchiectasis in pediatric AIDS.

OBJECTIVES: There are several reports of the pulmonary findings in children with HIV disease; however, the occurrence of bronchiectasis rarely has been noted. We evaluated occurrence of bronchiectasis in a large group of children referred to us with AIDS pneumopathy. METHODS: From January 1984 to April 1996, 203 children with AIDS and respiratory problems were referred to the pediatric pulmonary division at Children's Medical Center of Brooklyn. Medical records for 164 of these children were available and retrospectively reviewed. RESULTS: Uncomplicated pneumonia was present in 75, 24 had recurrent pneumonia, and 18 had unresolved pneumonia; lymphocytic interstitial pneumonitis (LIP) was diagnosed in 47 patients, worsening with time in all patients. Bronchiectasis was observed in 26 patients (26/164, 15.8%), diagnosed by chest radiograph in 26 (26/26, 100%), confirmed by CT scan of chest in 10 (10/26, 38.4%), and by histology in three (3/26, 11.5%). Median age at time of diagnosis of bronchiectasis was 7.5 years (range, 1 to 16 years). Sixteen children with LIP developed bronchiectasis (16/47, 34.0%). Three patients with recurrent pneumonia (3/24, 12.5%) developed bronchiectasis. Five patients with unresolved pneumonia (5/18, 27.7%) developed bronchiectasis. One infant developed bronchiectasis after Pneumocystis carinii pneumonia; another child developed bronchiectasis after P. carinii and Mycobacterium tuberculosis pneumonia. The CD4+ T-cell counts measured within 6 months of diagnosis of bronchiectasis were available in 23/26 patients and, all were < 100 cells per cubic millimeter. CONCLUSION: We conclude, from our experience, that there is a significant occurrence of bronchiectasis in children with AIDS and pulmonary disease, especially in children developing LIP, recurrent pneumonia and unresolved pneumonia, and CD4+ T-cell counts < 100 cells per cubic millimeter.

AIDS-Related Opportunistic Infections↗