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PubMed1992

High resolution computed tomography (HRCT) in emphysema associated with alpha-1-antitrypsin deficiency.

Alpha-1-antitrypsin (AAT) deficiency is an inherited disorder associated with an increased prevalence of pulmonary emphysema. To date there has been no description of the appearances on CT of the emphysema associated with AAT deficiency. We have reviewed the CT scans of 17 patients with proven AAT deficiency. Eleven out of 17 were examined with HRCT. The CT features were correlated with those on chest radiography. Areas of low density corresponding to parenchymal destruction and reduced perfusion, and attenuation of the pulmonary vasculature were present in all cases. Frank bulla formation occurred in 7/17 patients and was not a major feature. A striking CT finding was bronchial wall thickening and/or dilatation in 7/17 patients with gross cystic bronchiectasis in one patient. In contrast to appearances on chest radiography the upper zones were affected in all cases (17/17) and fine section scanning was particularly valuable in demonstrating changes in these less severely affected regions. Estimation of the extent of disease correlated well with abnormal lung function tests.

Adult↗
PubMed1990

Infantile lobar emphysema in association with congenital heart disease.

Three cases of congenital heart disease (CHD) are presented; all infants developed the unusual complication of lobar emphysema (LE). In all cases delay in reaching the correct diagnosis resulted in two of the infants undergoing a second operation to resect the emphysematous lobe. The chest radiograph in all cases was dominated by the heart disease and the radiological signs of LE were initially missed. The radionuclide ventilation/perfusion lung scan was used in two of these infants to provide conclusive proof and to emphasise the clinical significance of the condition. Lobar emphysema in association with CHD should be recognised early and lobectomy performed at the same time as surgical correction of the heart defect.

Female↗
PubMed1982

Computed tomography in pulmonary emphysema.

Fifty-three patients with chronic obstructive airways disease and 19 age-matched controls were studied using computed tomography (CT). The study shows that CT can detect the presence and distribution of pulmonary emphysema. Pulmonary vascular changes detectable on chest radiography correlate well with lung density as measured by CT. Patients with marked CT changes of emphysema had significantly greater impairment of diffusion capacity and FEV1.0/VC than the patients with less severe changes.

Adult↗
PubMed1984

Computed tomography in pre-operative assessment of bullous emphysema.

Nine patients with bullous emphysema for which surgery was being considered have been investigated with computed tomography (CT). In six out of nine patients (67%) CT showed more extensive disease or bilateral disease where only unilateral was suspected from postero-anterior and lateral chest radiographs. Only one patient proceeded to surgery and post-operative CT showed satisfactory re-expansion of the compressed lung. Surgery was abandoned in one patient as a result of the CT findings. Computed tomography is of value in patients with bullous emphysema in assessing the extent of disease and may assist the surgeon in operative planning.

Adult↗
PubMed1990

Basic mechanisms leading to focal emphysema in coal workers' pneumoconiosis.

Coal miners develop focal emphysema characterized by dilatation of second- and third-order respiratory bronchioles with coal mine dust-laden macrophages infiltrating the wall. A reticulin network with small amounts of collagen and atrophy of smooth muscle occurs. To evaluate the mechanisms of lung injury associated with this lesion, 17 long-term non- or ex-smoking West Virginia underground coal miners underwent bronchoalveolar lavage (BAL) and were compared to healthy nonsmoker and smoker controls. The coal miners had evidence of an alveolar macrophage-neutrophil alveolitis with a significant increase in neutrophils/microliter of epithelial lining fluid and an increased gallium lung scan index (206 +/- 26 units). Alveolar macrophages lavaged from coal miners spontaneously released exaggerated amounts of superoxide anion and hydrogen peroxide in vitro compared to nonsmoking controls. Coal workers had significantly elevated levels of neutrophil elastase in BAL fluid complexed with alpha 1-antitrypsin (P less than 0.01) and normal levels of alpha 1-antitrypsin. An accumulation of activated, dust-laden inflammatory cells with increased release of oxidants and elastase may contribute to the development of focal emphysema identified at postmortem in miners with coal workers' pneumoconiosis.

Adult↗
PubMed1987

An emphysema model in rats treated intratracheally with elastase.

Pulmonary functions, morphology, and morphometry were examined in rats at 3, 7, and 10 weeks after a single intratracheal administration of 6.5 units of porcine pancreatic elastase in order to obtain a model of pulmonary emphysema which would be suitable for studying the responses of emphysematous lungs to atmospheric pollutants. Functional residual capacity and residual volume of the elastase-treated rats increased at all the times studied, but their total lung capacity increased only at 7 and 10 weeks compared with those of the saline-treated control rats. The increase in static lung compliance and the decrease in peak flow and maximum flow at 50% of total lung capacity during forced expiration were also observed in all except the 3-week elastase animals. The elastase-treated lungs showed morphological changes characteristic of emphysematous lesions. The increase in mean linear intercept length and the decrease in total alveolar surface area were demonstrated by these elastase-treated lungs. Based on these results, we conclude that an adequate and suitable model of pulmonary emphysema could be obtained in rats 7-10 weeks after treatment with the present dose of elastase.

Animals↗
PubMed2004

Bone marrow-derived cells contribute to lung regeneration after elastase-induced pulmonary emphysema.

All-trans retinoic acid (ATRA) is known to reverse the anatomic and physiologic signs of pulmonary emphysema. However, the origin of the progenitor cells involved in this lung regeneration remains unclear. Recently, it was shown that bone marrow could be the source of progenitor cells for several cell types. Mice with elastase-induced emphysema were treated with ATRA, granulocyte colony-stimulating factor (G-CSF), or a combination of both. ATRA or G-CSF promoted lung regeneration and increased bone marrow-derived cell (BMC) numbers in alveoli. Combined treatment of both had an additive effect, which indicated that BMC mobilization might be important in lung regeneration.

Animals↗
PubMed1980

Polyalveolar lobe: anatomic and physiologic parameters and their relationship to congenital lobar emphysema.

Polyalveolar lobe is one of the recently described pathological entities that can give rise to congenital lobar emphysema (CLE). In polyalveolar lobe, the total alveolar number as determined by microscopic point-counting of randomly taken lung sections is increased three to fivefold. The airways and arteries are normal for age in number, size and structure. Follow-up over 10-20 yr of patients operated upon for congenital lobar emphysema, including those of polyalveolar lobe etiology shows that these patients do well clinically. All patients have some pulmonary function abnormalities, specifically a decrease in airway conductance, and forced expiratory volume. It is not possible to distinguish patients with polyalveolar lobes from other CLE patients on the basis of pulmonary function data. All patients had normal lung volumes and vital capacities, despite losing 8%-20% of their pulmonary parenchyma. Based on compensatory lung growth, it may be preferable to operate earlier, rather than later in these cases.

Child, Preschool↗
PubMed1984

Surgery of acquired lobar emphysema in the neonate.

Surgical treatment for pulmonary interstitial emphysema (PIE) has been reported with increasing frequency. We have done pulmonary lobectomies in 14 neonates with the localized form of PIE. This type of treatment was successful in 11 patients. Two patients expired in the hospital and one showed no improvement. Another six very compromised patients with diffuse PIE were treated during the same period with multiple visceral pleurotomies. Only one survived. Infants with diffuse PIE are usually poor surgical candidates, while those with acquired lobar emphysema (ALE) have been treated successfully with lobectomies.

Female↗
PubMed1988

Successful resolution of unilateral pulmonary interstitial emphysema in a premature infant by selective bronchial balloon catheterization.

Pulmonary interstitial emphysema (PIE) is a common complication in premature infants with respiratory distress syndrome. The development of pulmonary interstitial emphysema leads to marked respiratory embarrassment in an already compromised infant. Although usually bilateral, PIE may be unilateral. Various forms of treatment for unilateral PIE in the premature infant have been described, including selective bronchial intubation, unilateral pneumonectomy, visceral pleurotomy, pure oxygen administration, and high-frequency low-pressure ventilation. A recently reported conservative regimen consisting of downward lateral positioning of the affected lung for several days may not be successful. We describe a premature infant with progressive worsening of unilateral PIE, which was successfully treated by selective bronchial balloon catheterization after failure of conservative management. In this manner, we avoided selective bronchial intubation or other aggressive forms of treatment.

Bronchi↗
PubMed1984

Association of hemolytic anemia and early-onset pulmonary emphysema in three siblings.

Three of four siblings born to nonconsanguineous parents of Italian origin were affected with severe congenital hemolytic anemia of unknown cause, and early-onset pulmonary emphysema. Two of the three affected siblings died of septic shock after splenectomy, at the ages of 7 and 3 1/2 years, respectively. The remaining affected sibling was shown to have cutis laxa and severe pulmonary emphysema at 15 years of age. Assay of serum components indicated that alpha 1-antitrypsin and alpha 2-macroglobulin levels were normal or slightly elevated. However, there was markedly elevated activity of an elastase-like serum enzyme. The relation of the hemolytic anemia to the pulmonary findings in this family is not clear; pedigree analysis suggests a recessively inherited defect.

Adolescent↗
PubMed2003

Feasibility and safety of the airway bypass procedure for patients with emphysema.

OBJECTIVE: We have proposed that direct passages created between pulmonary parenchyma and large airways (airway bypass) could take advantage of the extensive collateral ventilation present in emphysematous lungs to provide improvement in expiratory flow and respiratory mechanics. A critical step in the safe performance of these procedures is to create passages through the airway wall into lung parenchyma while avoiding injury to adjacent blood vessels. METHODS: The procedure consists of selection of a target site bronchoscopically, use of a Doppler catheter to detect and avoid peribronchial blood vessels, and creation of a passage through the airway wall with a cautery probe. To evaluate the safety of airway bypass, 10 patients were treated during prescheduled lobectomies for neoplasm. The procedure was done after thoracotomy and immediately before resection and was confined to airways in the lung identified for removal. Airway bypass was subsequently performed in 5 patients undergoing lung transplantation for emphysema just before lung excision to evaluate the procedure in emphysematous patients. RESULTS: Twenty-nine passages (1-5 per subject) were created in the patients undergoing lobectomy. Eighteen passages were created (3-4 per subject) in the patients undergoing transplantation. There were 2 instances of mild bleeding in the patients undergoing lobectomy and no bleeding in the patients undergoing transplantation. Both instances were treated with suction and topical application of epinephrine and resolved without incident. CONCLUSION: The results of this study confirm that passages can be made safely through the airways of human subjects. These clinical results support further investigation of the efficacy of the airway bypass procedure in patients with emphysema.

Adult↗
PubMed1996

Should lung volume reduction for emphysema be unilateral or bilateral?

Both unilateral and bilateral lung volume reduction procedures are being advocated for treatment of severe, generalized emphysema. We analyzed the results of 166 consecutive patients who underwent unilateral (n = 87) or bilateral (n = 79) thoracoscopic stapled lung volume reductions to help define the role for these procedures. There was no statistically significant difference in the operative mortality (3.5% vs 2.5%), mean length of stay (11.4 +/- 1 vs 10.9 +/- 1 days), or morbidity for the unilateral and bilateral groups, respectively (p not significant for all variables). Oxygen dependence was eliminated in 18 (36%) of 50 patients who had unilateral procedures and 30 (68%) of 44 of those who had bilateral procedures (p < 0.01). Prednisone was eliminated for 38 (54%) of 51 unilateral-procedure patients, compared with 30 (85%) of 35 bilateral-procedure patients (p = 0.02). Overall, bilateral procedures produced a mean improvement in the forced expiratory volume in 1 second (FEV1) of 57%, compared with 31% for unilateral reduction procedures (p < 0.01). Our bilateral staple procedure produced a 72.8% mean increase in the FEV1 for patients who had upper lobe emphysema. Especially compromised patients (age > or = 75, with preoperative room air Po2 < or = 50 mm Hg or FEV1 < or = 500 ml) had the same morbidity and operative mortality with unilateral or bilateral procedures, but they had a higher 1-year mortality (17% vs 5%), primarily because of respiratory failure after the unilateral operation (p < .001). Although unilateral staple lung volume reduction may produce an excellent result in a given patient, the bilateral procedure appears to be the procedure of choice, because it provides better overall results at no increased morbidity or mortality compared with the unilateral procedure. The results of bilateral staple lung volume reduction by thoracoscopy appear to be comparable to those of median sternotomy.

Aged↗
PubMed1996

Unilateral thoracoscopic surgical approach for diffuse emphysema.

We evaluated the use of a lateral thoracoscopic approach for lung reduction surgery in patients with diffuse emphysema. Sixty-seven patients with a mean age of 61.9 years underwent operation. Operative side was determined by preoperative imaging. The procedures were laser ablation in 10 patients and stapler resection in 57 patients. Ten patients, including six of the 10 patients in the laser-only group had poor outcome (death or hospitalization longer than 30 days), leading us to abandon the laser technique. Of the remaining 57 patients undergoing primary stapled resection, duration of chest tube placement averaged 13 days (range 3 to 53 days) with a mean hospital stay of 17 days (range 6 to 99 days). Seven patients required ventilation for longer than 72 hours, six patients underwent conversion of the procedure to open thoracotomy, four patients acquired arrhythmias, and three patients were treated for empyema. There was one early death (1.7%), from cardiopulmonary failure. Forty patients returned for 3-month evaluation. Significant (p < 0.0001) improvements were seen in forced vital capacity (2.69 L after vs 2.26 L before) and forced expiration volume in 1 second (1.04 L after vs 0.82 L before), with 25 of 40 patients (63%) showing an improvement of more than 20%. Lung volume measures, in particular residual volume, fell significantly. Arterial blood gas analysis revealed that carbon dioxide tension fell significantly in patients with preoperative hypercapnia (carbon dioxide tension > 45 mm Hg, p = 0.018). Six-minute walk test results improved (894 feet after vs 784 feet before, p = 0.002), and symptomatic benefit was confirmed by significant improvement in the dyspnea index. The combination of both hypercapnia and reduced single-breath diffusing capacity for carbon monoxide was significantly more frequent (p = 0.0026) and was 86% specific (5 of 6 patients) in predicting serious postoperative risk. We conclude that the lateral thoracoscopic surgical approach to diffuse emphysema offers significant improvement in pulmonary mechanics and functional impairment. Patients with a combination of hypercapnia and reduced single-breath diffusing capacity for carbon monoxide should not be considered for this procedure because of significant perioperative risk.

Adult↗
PubMed1996

A randomized, prospective trial of stapled lung reduction versus laser bullectomy for diffuse emphysema.

Two procedures (laser bullectomy and lung reduction surgery with staples) are currently available for the surgical treatment of patients with diffuse emphysema. We compared the efficacy of these two surgical approaches in 72 patients, aged 67 +/- 7 years (mean +/- standard deviation), who had diffuse emphysema scored as severe on computed tomography and severe fixed expiratory airflow obstruction. The patients were prospectively randomized to undergo either neodymium:yttrium aluminum garnet contact laser surgery (n = 33) or stapled lung reduction surgery (n = 39) by unilateral thoracoscopy. The operative mortalities were 0% and 2.5%, respectively. No significant differences were noted between the groups (p < 0.05) with respect to operating time, hospital days, or air leakage for more than 7 days. However, a delayed pneumothorax developed in six patients (18%) who had laser treatment (p = 0.005). The operations eliminated dependency on supplemental oxygen in 52% of the laser group and 87.5% of the stapled lung reduction group (p = 0.02). The mean postoperative improvement in the forced expiratory volume in 1 second at 6 months was significantly greater for the patients undergoing the staple technique (32.9% vs 13.4%, p = 0.01) than for the laser treatment group.

Aged↗
PubMed1998

Preoperative and postoperative imaging in the surgical management of pulmonary emphysema.

For patients with emphysema, imaging studies have been useful for diagnostic purposes and for preoperative patient selection for surgical intervention, such as bullectomy, lung transplantation, and LVRS. Chest radiography is useful in evaluating hyperinflation. Inspiratory and expiratory films are used to estimate diaphragmatic excursion and air-trapping. CT scan is used to evaluate the anatomy and distribution of emphysema throughout the lungs, providing information clinically unobtainable by other means. Both imaging techniques are useful for detecting other disease processes. Radionuclide lung scanning also provides an estimate of target areas, volume occupying but nonfunctioning lung. Cohort studies utilizing these imaging techniques have demonstrated associations between preoperative characteristics and postoperative outcome. The imaging studies, especially the chest radiograph, have also played an important role in postoperative management. Many other imaging options are available, such as HRCT scan, quantitative CT scan, and single photon emission CT scan. Other techniques, such as MR imaging, may play a future role as well.

Cohort Studies↗
PubMed2000

Pulmonary artery remodeling modifies pulmonary hypertension during exercise in severe emphysema.

To clarify the relation between the vessel remodeling and the physiology of pulmonary circulation in chronic obstructive pulmonary disease (COPD), we studied the pulmonary hemodynamics at rest and during exercise (25W) and the morphology of pulmonary arteries with external diameters of 100-200 microm in ten patients with severe emphysema. The wall thickness (WT) was defined as the intima plus media. The percent WT of the external diameter (% WT) in emphysema (36.0 +/- 4.3%) was significantly increased compared with that (22.6 +/- 3.3%) in five control lungs. The % WT was not related to pulmonary arterial pressure (Ppa) at rest, but was highly correlated with exercise Ppa (r = 0.721, P = 0.02) and with deltaPpa (Ppa during exercise-Ppa at rest) (r = 0.899, P = 0.0004). These findings suggest that pulmonary artery remodeling leads to reduced recruitability and distensibility of pulmonary vessels and is closely related to exercise pulmonary hypertension.

Aged↗
PubMed1988

Cadmium fume inhalation and emphysema.

Lung function and chest radiographs of 101 men who had worked for 1 or more years manufacturing copper-cadmium alloy were compared with those of a referent group matched for age, sex, and employment status. Cigarette consumption was similar in the two groups. The cadmium workers had an excess of abnormalities of lung function and of radiographic changes consistent with emphysema. Classification of the cadmium workers by exposure categories based on either estimated cumulative cadmium exposure or liver cadmium measured by neutron activation analysis showed that abnormalities of lung function were greatest in those with the highest cumulative cadmium exposure or liver cadmium. The difference in the transfer coefficient (KCO) between cadmium workers and referents increased linearly with increasing cumulative exposure without evidence for a threshold. The estimated mean decrement in KCO for a cadmium worker employed 5 or more years with a cumulative exposure of 2000 yr.microgram.m-3 (exposure to the current UK control limit of 50 micrograms.m-3 for a working lifetime of 40 yr) lies between 0.05 and 0.3 mmol.min-1.kPa-1.l-1 (95% confidence interval). This decrement is consistent with the functional and radiological changes of emphysema observed in this group of workers.

Aged↗