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[Morphological classification of surgical forms of bullous emphysema in adult. Role of electron microscopy (author's transl)].

The definition and the classification of bullae are recalled and documented by some examples. Two types of bullae are opposed : bulla found in normal parenchyma and bulla associated with widespread emphysematous lesions. The biochemical and biological mechanisms of the emphysematous changes are briefly described, with a distinction of two different pathogenesis : on one hand, an inherited defect in connective tissue synthesis as in Marfan syndrome, and on the other hand, an unbalance between proteases and antiproteases. The pathologist's contribution to the classification of the surgical forms of emphysema is documented by an electron microscopic study of lung biopsy in some cases : two cases with bullae (a Marfan syndrome and an alpha 1 AT deficiency of the Pi Z phenotype) with diffuse panlobular emphysema; and 4 cases of bullae without radiological evidence of emphysema. The elastic fibres were modified only in the 2 first cases but were normal in the others. A follow up of such cases is needed.

Humans↗

[Pulmonary emphysema, hepatic lesions, and insulin-dependent diabetes in a patient with alpha-1-antitrypsin (Pi ZZ) deficiency (author's transl)].

A 47-year-old patient with panlobular emphysema and insulin-dependent diabetes had an alpha-1-antitrypsin phenotype Pi ZZ deficiency. Liver function tests were abnormal, and postmortem examination of the liver demonstrated abnormal intrahepatocytic globules of A1AT (a typical finding when the allele Z is present), but also fibrosis with steatosis. The patient's sister, Pi ZZ, had neither diabetes nor bronchopneumopathy, and no anomalies in liver function. Needle puncture biopsy of the liver had not been conducted. The phenotype Pi ZZ is typically associated with panlobular emphysema in adults, and cholestatic hepatitis in children. From reports in the published literature, it appears that isolated hepatic lesions or those associated with emphysema are rare. The fortuitous association of diabetes and hepatic lesions in this typical case of pulmonary affection in an adult is discussed.

Diabetes Complications↗

Pulmonary emphysema associated with the FZ alpha 1-antitrypsin phenotype.

In one family three brothers were found to have a moderate deficiency of alpha 1-antitrypsin associated with the unusual Pi (protease inhibitor) phenotype FZ. The Pi phenotypes of their six living siblings were found to be FM (in three), M (in two) and MZ (in one). The three FZ brothers all had moderate to severe obstructive airways disease, and two had at least moderately severe pulmonary emphysema. Additional risk factors included moderate cigarette smoking in two and prolonged exposure to grain dust in all three. The same risk factors applied to the six non-FZ siblings, but they had only mild symptoms and pulmonary dysfunction or no lung problems at all; one, a female smoker with the MZ phenotype, had probable early emphysema demonstrated radiologically. The three FZ men may have had reduced fertility, as they produced only 1 child among them, as compared with 39 among the other eight siblings. This family study thus suggests that individuals with the FZ phenotype are at risk for pulmonary emphysema and chronic obstructive airways disease, particularly in the presence of other risk factors, such as cigarette smoking and grain dust exposure.

Female↗

Orbital emphysema complicated by acute central retinal artery occlusion: case report and treatment.

Orbital emphysema is a well-known clinical entity that has been considered benign and requiring no treatment. A case of marked orbital emphysema with simultaneous central retinal artery occlusion is described. The prompt relief of this occlusion following aspiration of air from the orbit suggests that orbital emphysema is an unreported cause of central retinal artery occlusion.

Emphysema↗

[Diagnosis and course of congenital lobar emphysema].

Within 20 years 22 children with congenital lobar emphysema were observed. 13 children were infants, 9 were already older than a year, thus, clearly deviating from the age structure reported in literature. 15 children underwent an operation, in 2 children the alterations disappeared completely respectively partially. One child prematurely born died before operation. The upper lobe was concerned most frequently (11 times), followed by the right upper lobe (6 times) and the right middle lobe (4 times). Bronchoscopy was showing in surprising frequency a stenosis of the trachea (2 times) respectively of the main bronchus (7 times). In 13 of 15 cases bronchographically investigated the displacement and compression of the adjacent lobar bronchi were predominant. The regression of the emphysema observed in a x-ray picture series initially with an "opaque lung" and later on with spontaneous disappearance of the lobar emphysema is more fully described. In the opinion of the authors the analysis of their cases is justifying a change in the conception concerning age structure and urgency of surgery. Bronchological examination is considered of importance for differential diagnostics especially to delimit cystic pulmonary alteration.

Aging↗

[Two complications of bullous emphysema. Apropos of a case].

Infection and cancer are two classical complications of bullous emphysema. We report the case of a 47-year-old patient who presented a tuberculous infection then cancer within less than one year interval. The questions concerning diagnosis of infection are discussed. Pyogenic germs are usually involved and tuberculosis much less often. Exceptionally atypical mycobacteria, notably xenopi, are rarely the cause. The diagnosis can be particularly difficult in case of haemorrhage or cancer on bullae. The diagnosis of cancer in bullous emphysema is also studied. The relationships between tuberculosis and cancer in bullous emphysema are discussed.

Adenocarcinoma↗

[Comparison of 99mTc-technegas scintigraphy and high resolution CT in pulmonary emphysema].

This study was undertaken to assess the usefulness of technetium-99m technegas scintigraphy axial images (technegas) compared with high resolution CT (HRCT) in 12 patients with pulmonary emphysema. All patients were male adult and heavy smokers and their mean age was 65. All patients inhaled 505 MBq technegas in several tidal volume breaths in the supine position without breath holding. SPECT imaging was performed by a Picker model Prism 2000. HRCT was performed after technegas. The technegas images showed different degree of changes from areas of heterogeneity to hot spots or defects. In emphysema, HRCT images showed low attenuation areas varying in size and number. In 4 of 12 patients, the degree of abnormal findings on technegas increased according to the degree of abnormal findings on HRCT. In the remaining 8 patients, however, more detailed findings were shown by technegas than by HRCT. There was no patients in which HRCT showed a greater changes than on technegas. HRCT can demonstrate low attenuation areas, even less than 5 mm in diameter. Moreover previous reports showed that aerosol inhalation would also demonstrate the airway disease. Assessment of the degree of involvement revealed by both methods indicated the superiority of technegas over HRCT. We conclude that technegas can represent the ventilation involvement easily more than HRCT in pulmonary emphysema.

Aged↗

Reduced work of breathing after single lung transplantation for emphysema.

BACKGROUND: Pulmonary emphysema, with or without chronic bronchitis, has emerged as the most common indication for successful single lung transplantation. Although gas exchange can be expected to improve after successful transplantation, such changes do not adequately explain the improvement in dyspnea experienced by these patients and resulting in improved quality of life. METHODS: We prospectively studied the respiratory mechanics of 14 single lung transplantation recipients with pulmonary emphysema, of whom 10 have been followed up beyond 1 year. The mean age of the group was 48.8 years (range, 42 to 60 years) for the seven men and seven women. Average donor-predicted total lung capacity was 0.6 L (+0.2 [standard error]) greater than recipient-predicted total lung capacity. Comparison of pulmonary resistance, dynamic lung compliance, and static lung compliance were taken to examine the possible role of reduced airways resistance and of improved elastic recoil in the reduced work of breathing. Results were analyzed by means of a one-tailed paired Student t test and linear regression analysis (both stepwise and multivariate); results are tabulated by mean (+/- 1 [standard error]). RESULTS: Between preoperative measurements and 12 months postoperatively, maximum lung elastic recoil increased from 8.1 (+/- 0.7) to 11.3 (+/- 1.0) cm H2O, p < 0.01; pulmonary resistance fell from 8.3 (+/- 0.8) to 5.4 (+/- 0.7) cm H2O sec/L, p < 0.01. Dynamic lung compliance fell from 0.23 (+/- 0.04) to 0.12 (+/- 0.02) L/cm H2O, p < 0.02, and static lung compliance fell from 0.66 (+/- 0.13) to 0.22 (+/- 0.05) L/cm H2O, p < 0.001. CONCLUSIONS: The decline in lung compliance after single lung transplantation reflects the importance of improvement in elastic recoil and reduced chest wall distention, improving the work of breathing. The 67% decline in static lung compliance (300% increase in elastic recoil) is probably the single most important mechanical factor leading to reduced dyspnea after single lung transplantation for emphysema.

Airway Resistance↗

Alveolar integrity in pulmonary emphysema using technetium-99m-DTPA and technetium-99m-HMPAO radioaerosol inhalation lung scintigraphy.

UNLABELLED: The alveolar integrity (AI) in 17 male patients with pulmonary emphysema (EMPH) diagnosed by chest x-ray was measured by 99mTc-DTPA and 99mTc-HMPAO radioaerosol inhalation lung scintigraphy. METHODS: The patients were divided into two groups: (A) nine patients with pulmonary emphysema and normal carbon monoxide diffusion capacity (DLCO) and (B) eight patients with pulmonary emphysema and abnormal DLCO. The degree of AI damage in EMPH was presented as the slope of the time-activity curves from the dynamic left lung imagings in DTPA and HMPAO. The AI of EMPH patients were compared with the AI of 16 normal controls. RESULTS: The results show that: (1) the slope of DTPA is larger than that of HMPAO in each of the portions of the left lung for any of the study groups; (2) statistical differences were found between the normal controls and EMPH patients in HMPAO but not in DTPA; and (3) the correlation was not good between DLCO and DTPA/HMPAO in EMPH patients. CONCLUSION: Our results suggest that: (1) at least two different mechanisms in the lungs were at work; (2) the AI damage in EMPH developed mainly in the lipophilic part of the alveoli; and (3) the AI damage presented as slopes of DTPA/HMPAO in our study was different from the traditional pulmonary function such as DLCO.

Administration, Inhalation↗

[The etiology of peptic ulceration in patients with chronic pulmonary emphysema].

The purpose of this study was to determine the etiology of peptic ulceration in patients with pulmonary emphysema. We performed endoscopy in 50 patients with chronic pulmonary emphysema, these were patients with and without peptic ulcer. There was no significant differences between the patients with and without ulcer, with respect to past history of pneumonia, degree of dyspnea, current drugs, nutritional status, the extent of gastric mucosal atrophy, gastric mucosal blood flow, gastric mucosal hexosamine content and serum pepsinogen levels. But patients with peptic ulcer were significantly younger than patients without peptic ulcer. Cigarettes per day in patients with peptic ulcer 29.3 +/- 11.6 cigarettes/day were significantly higher than those without peptic ulcer 22.5 +/- 10.0 cigarettes/day. We concluded that increased incidence of peptic ulceration in patients with chronic pulmonary emphysema is related to the quantity of cigarette smoking.

Aged↗

[Changes of lung function in experimentally induced emphysema before and after unilateral homotransplantation of the canine lung].

Pulmonary function studies after single-lung homotransplantation in dogs with experimentally induced emphysema. Pulmonary emphysema was induced in 8 bastard dogs by intratracheal instillation of papain (dose 2 mg/kg 3 to 5 times in 6 to 10 weeks). Development of emphysema was documented by weekly pulmonary function studies. The emphysematous animals underwent single-lung homotransplantation. Postoperative studies of lung function were performed immediately after transplantation and 1 week postoperatively. Bronchospirometric measurement revealed that ventilatory function and oxygen consumption of the graft was at least equal to the animal's own emphysematous lung. Up to 1 week after transplantation there is no evidence of serious ventilation-perfusion imbalance.

Animals↗

[Lobar emphysema in children].

Lobar emphysema is a very rare congenital anomaly of the lungs. The clinical presentation varies from dyspnea which is usually seen during the first six months of life to mild course later during the life. Surgery is the most common way of management. In our two cases the first symptoms appeared at the age of three months and three years, respectively. In the first case emphysema was located in the right upper lobus and was surgically removed. In the second case emphysema was located in the left upper lobus. The parents refused surgery and following hospital treatment the child was without dyspneic symptoms.

Child, Preschool↗

[Evaluation of desmosine excretion in urine and activity of elastase in neutrophils of patients with pulmonary emphysema].

In 78 patients with pulmonary emphysema (49 smokers, 29 nonsmokers) urinary desmosine excretion (UDE) and neutrophil elastase activity (NE) were evaluated. UDE and NE were similar in smokers and nonsmokers and significantly higher than in age, sex-matched control groups. UDE correlated better with NE in nonsmokers than in smokers. Cigarette smoking did not effect UDE in patients with pulmonary emphysema. Obtained results indicate, that UDE measurement could be used in diagnosis and monitoring of lung elastolysis in pulmonary emphysema and suggest the key role of neutrophil elastase in pathogenesis of this disorder.

Adult↗

[Severe subcutaneous emphysema and hypercapnia during laparoscopic cholecystectomy].

A 41-year old, 50 kg female was scheduled for laparoscopic cholecystectomy. Anesthesia was induced with thiopental 250 mg IV and endotracheal intubation was performed using succinylcholine 60 mg IV. Anesthesia was maintained with N2O (67%)-oxygen-sevoflurane (1.5-2%) and pancuronium was used for muscle relaxation. The lungs were mechanically ventilated with TV 500 ml and RR 12.min-1. Immediately after the start of incision, PECO2 was 30 mmHg. But about thirty minutes after introducing carbon dioxide pneumoperitoneum, subcutaneous emphysema and high PECO2 (60 mmHg) were noted and arterial blood gas analysis showed PaCO2 63.2 mmHg, PaO2 135.4 mmHg and pH 7.32. Generally in laparoscopic cholecystectomy, subcutaneous emphysema is more common than in gynecologic laparoscopy and especially with severe subcutaneous emphysema, there is a risk of hypercapnia. This is because carbon dioxide in subcutaneous tissue is more absorbable than that in peritoneal cavity. As carbon dioxide in subcutaneous tissue is absorbed continuously after the operation, the patient should be carefully observed postoperatively.

Adult↗

[The congenital lobar emphysema (author's transl)].

The congenital lobar emphysema has no uniform etiology. We can however suppose that malformations especially of the cartilage are of importance. The whole lobe is mostly involved, with predilection of the left upper-lobe. The symptomatology of the congenital lobar emphysema is a manifold one. Striking are respiratory embarrassement with dyspnea and cyanosis. For the diagnosis an X-ray is necessary. Remarkable is a transparent lung in which you can still see an unimportant pattern. For the differential diagnosis et first congenital cysts come in question. By intercurrent infections a sudden severe deterioration can result, also if the cases show a favourable course. The method of choice is a timely operation, necessary is the resection of the affected lobe. This is a report of 17 own observations. In 11 cases we found a congenital lobar emphysema, in 6 cases congenital lung-cysts. We could examine 10 patients till to eleven years from the beginning of the disease. All the children had a normal development, except of insignificant residues.

Cysts↗

CT measurement of mediastinal anterior junction line in emphysema patients.

The anterior junction line (AJL), the sterno-aortic distance and the sagittal thoracic diameter were measured in CT sections at the carinal level in 22 patients with emphysema and 22 controls. The measured distances were all significantly larger in the emphysema patients. An AJL of 3 cm or more and a sterno-aortic distance of 4 cm or more was found in respectively 10 and 16 of the 22 emphysema patients but in none of the control patients.

Adult↗

Emphysema: concepts under change--a pathologist's perspective.

The diagnosis of pulmonary emphysema is often neither as obvious nor as easy as is generally assumed. In this review, the methodologies are discussed by which emphysema can be accurately diagnosed. The classification of emphysema is outlined, with the salient gross and microscopic features listed.

Emphysema↗

Age and experimental obstructive emphysema. A morphometrical study on the rat.

Age, as a risk factor in the development of experimental obstructive emphysema, is proposed as the hypothesis of this study. Ninety-two Wistar rats were organized into two age groups: adult (16 weeks) and middle-aged (56 weeks). Each age group was subdivided into three groups: a control group, consisting of unmanipulated animals; a "cannula" group consisting of animals into whose trachea a cannula was implanted; and a "valve" group, consisting of animals into whose trachea a valve had been implanted. The survival was one month. A histomorphometric study was performed on the lungs and the results were compared statistically. Throughout the experiment the amount of food consumed by each animal and the variations in weight were monitored. After sacrifice, the lungs were processed for light microscopy. Thirteen histomorphometric variables were quantified and subsequently systematized into three groups: those which quantified the size of the distal airspace ("area of the alveolar section", "alveolar chord" and "mean linear intercept"): those which quantified the tissue ("wall thickness", "tissue density", "internal perimeter of each alveolar section", "internal alveolar perimeter per field" and "alveolar section/section perimeter"); and those which quantified the elastic fibre ("elastic fibre area", "elastic fibre perimeter", "elastic fibre area/elastic fibre perimeter", "elastic fibre density" and "elastic fibre density per tissue density"). The results were compared statistically and the sensitivity, specificity and misclassification indices were calculated, as well as the attributable and relative risk. From the results, it was observed that, in general, the animals of the valve and cannula groups in both age groups displayed a decrease in food intake and a body weight loss. The middle-aged animals were the only group which displayed significant differences in all the morphometric variables except wall thickness, when the cannula and valve groups were compared with the control group. In both the cannula and valve groups, the values of the variables which quantified the distal airspace increased, while the values of the variables which quantified the lung tissue and the elastic fibre decreased. In the manipulated middle-aged group, the attributable risk of developing emphysema was 56.66% and the relative risk 5.55; in the group of manipulated adult animals, the attributable risk was 23.55% and the relative risk 1.66. The results of this study lead us to propose that the middle-aged rats with experimental airflow obstruction displayed a greater risk of developing emphysema than the adult rats which were subjected to the same procedure.

Aging↗