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Congenital lobar emphysema: a case with bronchial atresia and abnormal bronchial cartilages.

An infant with congenital left upper lobe emphysema presenting with respiratory distress and failure to thrive, which necessitated lobectomy, is described. Careful pathological examination revealed two defects in association with the emphysema: bronchial atresia and defective bronchial cartilage in the affected lobe. Whilst congenital lobar emphysema is the term usually employed to describe this clinical presentation of respiratory distress in infancy, 'panlobular emphysema of infancy' would be a more accurate description. Congenital defects in the airways may account for the development of emphysema, but there may be a more fundamental congenital tissue abnormality at alveolar level.

Bronchi↗

Blood platelets in experimental lung emphysema. Comparative analysis of the number and aggregation abilities of platelets in left and right ventricular blood of the heart.

Quantitative and morphological analyses (in SEM) of blood platelets collected from the left and right ventricles of the rat heart in the course of experimental lung emphysema were done. Platelet aggregation index was estimated, too. Emphysema was induced by a single intratracheal instillation of papain solution in a dose of 20 mg/kg b.w/1 ml PBS. The animals were sacrificed after 2 and 24 hours and 7, 14, 28 days later. Within 24 hours of the experiment a slight decrease was observed in the number of platelets in the blood collected from the left ventricle compared to the right one as well as to control animals. Also a reduction in platelet aggregation coefficient value was noted. However, in the later period of emphysema progression (after 7th day), a statistically significant increase was found in the number of blood platelets in the left ventricle. A relation was noted between quantitative changes of blood platelets and emphysema progression evaluated morphometrically. The ultrastructural examinations in SEM suggest the occurrence of platelet satellitosis in animals intratracheally injected with papain solution. The present results indicate the possibility of a significant contribution of blood platelets to the pathogenesis of experimental lung emphysema.

Animals↗

Total respiratory impedance and early emphysema.

It can be postulated that patients in early stages of pulmonary emphysema have normal values of total respiratory resistance and reactance. The purpose of this study was to investigate whether pulmonary emphysema, detected functionally by a decrease of the single breath diffusing capacity (DLCO) by at least 25% of predicted, and an increase of the static lung compliance (CLst) by at least 50% of predicted, can be accompanied by normal values of respiratory resistance (Rrs) and reactance (Xrs), measured between 2 and 24 Hz by the forced oscillation technique. In a prospective study, we determined CLst in 26 patients, who had been selected on the basis of normal values of Rrs and Xrs, and a DLCO of less than 75% of predicted. In 17 of these patients, CLst was more than 150% of predicted. Since there were only minor abnormalities on routine lung function tests and chest X-ray, it is likely that these patients presented early emphysema. In the nine other patients, CLst was within normal limits: four suffered from interstitial lung disease; the remaining five were probably in a preliminary stage of early emphysema. In conclusion, early emphysema should systematically be considered as the first diagnosis in patients with normal values of Rrs and Xrs, and a decrease of DLCO. Onset of interstitial lung disease is a possible alternative.

Female↗

[Pulmonary emphysema: quantification using computed tomography and correlations with respiratory function tests].

Pulmonary emphysema can be defined in precise anatomical terms. Only histological examination of an entire lung will enable the extension and the severity of the emphysema to be fully assessed. The authors propose a visual score, using computed tomography to quantify emphysema in 61 subjects. 51 had chronic airflow obstruction (BPCO) and were divided into 31 chronic bronchitics (BC) and 20 emphysematous subjects (EP). 10 volunteers who were free of any respiratory pathology were chosen as controls. A visual score for the computed tomography was established for the subjects as a whole. Double reading of the data enabled the reproducibility of the method to be checked in 10 subjects (r = 0.98, p < 0.001). No emphysema was found in the 10 controls, the computed tomography score was appreciably more elevated in the EP subjects than in the BC group at 1.3 and 0.44 respectively (p < 0.001). In the BC, the computed tomographic score was not correlated with the PaO2 (r = 0.54, p < 0.001) and the FEV1 (VEMS) (r = < 0.44, p < 0.05). On the other hand, in the PE group, the score was correlated with the FEV1 (r = 0.52, p < 0.05) and the residual volume (r = 0.06, p < 0.05) and the total lung capacity (r = 0.63, p < 0.05) and the TLCO (r = 0.56, p < 0.05) and the TLCO/VA (r = 0.59, p < 0.05). The adoption of a visual computed tomographic score enabled the authors to find the correlations between pulmonary emphysema and the most specific tests of respiratory function.

Adult↗

[Long-term follow-up of pulmonary emphysema].

Definition of lung emphysema is based on morphologic criteria (irreversible destruction of alveolar space). In advanced stages of the disease, emphysema may be suspected clinically, by lung auscultation, lung function tests, and radiology. In early stages, there are characteristic functional findings, such as an irreversible decrease in forced expiratory volumes or flows. These simple tests are easily available. In this article, the natural course of lung emphysema is described, based on long term changes in lung function. The typical discrepancy between normal airway resistance and a decrease in FEV1 allows suspicion of early emphysema. In the further development of emphysema, an increase of airway resistance together with hypercapnia indicates severe functional disturbances and cor pulmonale.

Adrenal Cortex Hormones↗

Increased leukocyte elastase of the tracheal aspirate at birth and neonatal pulmonary emphysema.

OBJECTIVE: The authors have previously shown the association of elevated neonatal serum IgM and chorioamnionitis in infants in whom pulmonary emphysema characteristic of Wilson-Mikity syndrome subsequently developed. This paper extends the observation to the measurement of polymorphonuclear leukocyte elastase-alpha 1-proteinase inhibitor complex (PMN elastase-alpha 1-PI) in tracheal aspirates of infants with chronic lung disease. PATIENTS: Tracheal aspirates were obtained from 90 very low birth weight neonates within 24 hours of birth. Serum also was collected within 72 hours of birth, and placentas were examined for signs of inflammation. RESULTS: The mean PMN elastase-alpha 1-PI was significantly elevated (21.8 micrograms/mg albumin) in infants with a pulmonary emphysema syndrome like that designated by Wilson-Mikity compared either with those with bronchopulmonary dysplasia (1.5 micrograms/mg albumin, P < .01) or those with respiratory distress syndrome in whom bronchopulmonary dysplasia did not develop (2.3 micrograms/mg albumin, P < .01). Infants with pulmonary emphysema had a significantly elevated mean serum IgM and a high incidence of chorioamnionitis. CONCLUSIONS: The level of PMN elastase-alpha 1-PI was increased in the tracheal aspirates of newborns in whom pulmonary emphysema developed. Intrauterine inflammation may increase the level of PMN elastase in the fetal respiratory tract. This increase in PMN elastase-alpha 1-PI in fetal lung tissue may cause lung injury in utero, resulting in postnatal pulmonary emphysema consistent with the Wilson-Mikity syndrome following ventilation.

Bronchopulmonary Dysplasia↗

[Interleukin-8 in sputum from patients with pulmonary emphysema].

We measured concentrations of interleukin-8 (IL-8) in sputum from 38 patients with pulmonary emphysema, from 30 patients with bronchial asthma, and in induced sputum from 10 healthy subjects. Concentrations of IL-8 were significantly higher in sputum from patients with emphysema than in sputum from patients with asthma (mean +/- SE: 22,961 +/- 1,412 pg/ml and 9,135 +/- 1,590 pg/ml, respectively, p<0.0001) and were also higher than in sputum from healthy subjects (3,253+/-687 pg/ml, p<0.0001). Concentrations of IL-8 in sputum from patients with emphysema were unrelated to whether the patient was a current smoker or an ex-smoker, and did not correlate with concentrations of IL-8 in serum. Concentrations of IL-8 in sputum from patients with emphysema correlated significantly with FEV1% (r=-0.78, p<0.0001) and with %RV/TLC (r=0.63, p<0.01). These data suggest that IL-8 is mainly involved in chronic airway inflammation and that concentrations of IL-8 in sputum are closely related to airflow obstruction in patients with pulmonary emphysema.

Female↗

[Comparison between high-resolution computed tomography and 99mTc-technegas SPECT pulmonary emphysema].

Scintigraphy with 99mTc-technegas was recently introduced for clinical imaging of lung ventilation. This method has been found to be useful in emergencies, to be more suitable for single photon emission computed tomography (SPECT) than other agents used in ventilation scintigraphy, and could reveal abnormalities in ventilation more easily than high resolusion computed tomography (HRCT) in pulmonary emphysema. We compared 99mTc-technegas SPECT with HRCT in six regions: the right upper, middle, and lower lobes, the left upper lobe, the lingula, and the left lower lobe, in 15 patients with pulmonary emphysema. Patients with centrilobular emphysema tended to show stronger changes in upper lobes than in lower lobes on both 99mTc-technegas SPECT and HRCT. Some regions showed no change on HRCT but various changes on 99mTc-SPECT. Patients with panlobular emphysema showed severe changes on 99mTc-SPECT in lower lung fields in which well-demarcated areas of low attenuation were not seen on HRCT. We conclude that 99mTc-SPECT is useful for detecting early changes and panlobular changes in pulmonary emphysema.

Adult↗

A recurrent case of spontaneous mediastinal emphysema.

The first case of spontaneous mediastinal emphysema was reported by Hamman et al. in 1939. It is a rare condition that primarily occurs in young adults. In many instances, spontaneous mediastinal emphysema is caused by yelling, shouting or active exercise, but the cause is often unknown. It is rare for cases of spontaneous mediastinal emphysema to recur. Recently, we experienced a recurrent case of spontaneous mediastinal emphysema in a patient with a duodenal ulcer. Vomiting appeared to be the cause of the recurrence in our patient. Of the 58 patients with spontaneous mediastinal emphysema who have been reported in Japan so far, recurrences were documented only in our patient and two others.

Adult↗

[Effects of oxitropium bromide on exercise and lung function in patients with pulmonary emphysema].

Anticholinergic drugs have been reported to be effective in pulmonary emphysema. We studied the effects of the anticholinergic drug oxitropium bromide on exercise capacity and lung function in patients with this disease. We studied 11 men and 1 women, aged 69.3 +/- 4.5 yrs, in whom chest radiography showed pulmonary emphysema. Before and after the subjects inhaled two puffs of oxitropium bromide, they walked in a corridor for 12 minutes, and the distance they walked and spirometric data were recorded. FVC and FEV1 were significantly higher after inhalation of the drug. FEV1% did not change, and the distance walked increased slightly. We also studied symptoms and peak expiratory flow rate in 37 patients with pulmonary emphysema before and after the start of therapy with inhaled oxitropium bromide. Symptom scores of wheezing and sleep improved, and peak expiratory flows increased significantly. We conclude that inhaled oxitropium bromide can improve lung function and slightly increase exercise capacity in patients with pulmonary emphysema. These effects may be caused by bronchodilation-induced increases in FEV1 and FVC. Monitoring of peak expiratory flow in patients with pulmonary emphysema may be useful for evaluating the clinical effectiveness of oxitropium bromide.

Aged↗

[Associations between breathing pattern during submaximal exercise and exercise capacity in patients with pulmonary emphysema].

We sought to clarify the factors associated with exercise capacity in patients with pulmonary emphysema. Exercise capacities of 20 men with pulmonary emphysema were evaluated by bicycle ergometery, and the results were used to divide the subjects into two groups: high exercise capacity (n = 10) and low exercise capacity (n = 10). Pulmonary-function tests were done, emphysema scores were computed from CT scans, breathing pattern was recorded during submaximal exercise (up to 20 watts), and index of rapid shallow breathing was computed. Neither FEV1 nor airway resistance differed between the two groups, and patients with lower exercise capacity tended to have lower tidal volumes and higher values of the index of rapid shallow breathing during submaximal exercise. Functional residual capacity measured by body plethysmography and emphysema scores were inversely associated with exercise capacity. We speculate that among patients with pulmonary emphysema and a given degree of airway obstruction, a high functional residual capacity causes breathing during submaxinal exercise to be rapid and shallow, and that this rapid and shallow breathing makes ventilation inefficient, increases the work of breathing, and limits exercise capacity.

Aged↗

[Pulmonary emphysema: indications for surgical treatment].

Pulmonary emphysema is defined as abnormal irreversible enlargement of airspaces distal to the terminal bronchioli, accompanied by destruction of their walls and without obvious fibrosis. It occurs usually as a consequence of smoking. Advanced stages of emphysema lead to dyspnea and are associated with airflow obstruction, hyperinflation and reduced diffusing capacity. Measures that have been shown to alter the course of the disease are smoking cessation and long-term oxygen administration to correct arterial hypoxemia. Antibiotics, bronchodilators, corticosteroids and rehabilitation may provide symptomatic relief. Surgery is another treatment option for selected cases. Recurrent pneumothoraces resulting from rupture of subpleural bullae may be prevented by thoracoscopic pleurodesis. Large bullae that compress adjacent lung and mediastinal structures may be resected. In selected cases of diffuse advanced emphysema associated with significant hyperinflation, volume reduction by resection of the most destroyed areas of the lungs may improve dyspnea, pulmonary function and exercise tolerance. In severely symptomatic patients with reduced life expectancy due to endstage emphysema, lung transplantation may provide with dramatic symptomatic and functional improvement. If the indications for operation are carefully considered and the intervention is performed by an experienced team, surgery is a valuable treatment modality in certain patients with pulmonary emphysema.

Humans↗

EMPHYSEMA IN LUNG MACROSECTIONS CORRELATED WITH SMOKING HABITS.

The relationship between smoking and pulmonary emphysema was explored in 71 autopsies, by correlating Gough-Wentworth lung macrosections with smoking histories. An increasing percentage of smokers was found with each increment of emphysema. Particularly close relationship was observed between centrilobular emphysema and smoking. No definite association was found between smoking and panlobular emphysema.

Emphysema↗

Orbital emphysema after endoscopic third ventriculostomy and posterior fossa surgery in the sitting position.

INTRODUCTION: Orbital emphysema can be associated with optic nerve and vascular compromise, with subsequent potentially irreversible visual loss. MATERIALS AND METHODS: We report a 4-year-old girl who presented right orbital emphysema after posterior fossa surgery for pilocytic astrocytoma resection in the sitting position. Endoscopic third ventriculostomy (ETV) had been previously carried out for the treatment of symptomatic obstructive hydrocephalus. Our hypothesis is that periorbital emphysema developed due to air passing from the intracranial to the extracranial compartment through the ETV burr hole. RESULT AND DISCUSSION: The emphysema resolved spontaneously without any sequelae. To our knowledge, this is the first description of orbital emphysema after posterior fossa surgery in the sitting position.

Astrocytoma↗

Association between polymorphism in gene for microsomal epoxide hydrolase and susceptibility to emphysema.

BACKGROUND: The first-pass metabolism of foreign compounds in the lung is an important protective mechanism against oxidative stress. We investigated whether polymorphisms in the gene for microsomal epoxide hydrolase (mEPHX), an enzyme involved in this protective process, had any bearing on individual susceptibility to the development of chronic obstructive pulmonary disease (COPD) and emphysema. METHODS: We designed PCR-based genotyping assays to detect variant forms of mEPHX that confer slow and fast activity. We used these assays to screen 203 blood-donor controls and groups of patients with asthma (n = 57), lung cancer (n = 50), COPD (n = 68), and emphysema (n = 94), who were attending specialised clinics in Edinburgh, UK. FINDINGS: The proportion of individuals with innate slow mEPHX activity (homozygotes) was significantly higher in both the COPD group and the emphysema group than in the control group (COPD 13 [19%] vs control 13 [6%]; emphysema 21 [22%] vs 13 [6%]). The odds ratios for homozygous slow activity versus all other phenotypes were 4.1 (95% CI 1.8-9.7) for COPD and 5.0 (2.3-10.9) for emphysema. INTERPRETATION: Genetic polymorphisms in xenobiotic enzymes may have a role in individual susceptibility to oxidant-related lung disease. Epoxide derivatives of cigarette-smoke components may be the cause of some of the lung damage characteristic of these diseases.

Adult↗

Histologically proven extrinsic allergic alveolitis with severe obstructive pulmonary emphysema

Anamnesis: 61-year old man with progressive shortness of breath on exercise. Cough and expectoration during the last 6 years. - Exposure: Driver of cereals, massive exposure to mouldy and pest contaminated grains. Gave up his profession in 1979 due to dyspnea with short (2-3 h) latency after exposure. Since 1980 intermittent exposure during occasional jobs; renewed symptomatology. Aspergillus fumigatus detected on agar plates inoculated with material from wet areas in bathroom and kitchen. - Clinical symptoms: Barely audible vesicular breathing, barrel-shaped thorax, inspiratory-intercostal retraction. - Bodyplethysmography: Obstructive pulmonary emphysema with FEV1 0.8 l, TLC 7.8 l, RV/TLC relation 67%. - Precipitin-detection: Significantly increased IgG against Fusarium. Other moulds including Aspergillus: negative; thermophilic actinomycetes: negative; pigeon and chicken: negative; Ouchterlony with native material from patients flat: negative. - CT including HR-CT: Bilateral-substantial emphysema, no bullae, no ground glass-opacity, no signs for interstitial lung diseases, no mediastinal enlargement of lymph nodes. - Alpha-1-Antitrypsin: 1.67 to 2.3 g/l (normal range), phenotype M1. - Histology: In resected material from right-side lung-volume-resection detection of pulmonary emphysema as well as lymphocyte infiltration and numerous epitheloid cell granulomas with Langhans'giant cells without caseation assessed as residues of an exogenous allergic alveolitis. - Conclusion: In a patient with lung volume reduction surgery due to severe emphysema histologically a persistent exogenous allergic alveolitis was detected, which might have caused the emphysema.

Journal Article↗

Iatrogenic retropharyngeal emphysema with impending airway obstruction.

The causes, diagnosis, sequelae, and treatment of interstitial emphysema of the face, neck, and thorax have been previously described in the medical and dental literature and are reviewed here. We present a unique case of a patient who, following dental amalgam restoration, developed severe retropharyngeal emphysema with impending airway obstruction, cervicofacial subcutaneous emphysema, and pneumomediastinum. Management consisted of urgent tracheotomy, broad-spectrum prophylactic antibiotic coverage, close observation, and reassurance. The likely mechanisms of precipitation and extension of the emphysema in this patient are discussed.

Adult↗

Diffuse soft tissue emphysema in anorexia nervosa: a case report.

OBJECTIVE: Anorexia nervosa (AN) is an eating disorder that occurs mainly among young females and its prevalence has increased in recent decades. METHODS: We reported a patient with AN with spontaneous diffuse soft tissue emphysema and bicytopenia, both of which are very rare complications of AN. Our patient, a 20-year-old woman, had lost 41.6% of her previous body weight within 9 months. Her body mass index was 10.8 and amenorrhea had persisted for 5 months. RESULTS: After admission, she was diagnosed with AN-restrictive subtype. She was noted to have crunching sounds in the neck and upper chest wall during hospitalization. A chest X-ray and chest computed tomography scan both revealed diffuse soft tissue emphysema including subcutaneous emphysema, pneumomediastinum, and pneumoretroperitoneum. DISCUSSION: Diffuse soft tissue emphysema improved gradually and completely resolved on the 21st day of hospitalization. This condition seems to be benign and only a short period of hospitalization is required.

Adult↗