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Query: UMLS:C0034067 (emphysema)
 11,506 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We retrospectively reviewed the clinical and radiographic findings in patients with pneumatosis intestinalis (PI), who were identified among 441 patients with cystic fibrosis. Since 1944, the age at onset and the incidence of PI have increased. Pneumomediastinum, pneumothorax, or pulmonary interstitial emphysema was found in 95% of patients with PI compared with 62% of patients without PI. The type, distribution, and severity of PI often changed with time. PI is correlated with the development of obstructive pulmonary disease, which facilitates air dissection into interstitial spaces. Dissection of air is often clinically silent and tends to be self-perpetuating.
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PMID:Pneumatosis intestinalis in cystic fibrosis. 372 32

The lungs of 9 patients with cystic fibrosis were studied by morphometric techniques to determine the amount of bronchiectasis, emphysema, pneumonia, bronchial gland enlargement, and small airways narrowing and density. The severity of these processes, which should account for the clinical picture of air-flow limitation in these patients, varied greatly among patients of different ages. Bronchiectasis was present in all children, but the amount of bronchiectasis did not appear to increase with age. Mild destructive emphysema was seen only in adults, but many younger patients had overinflation without destructive emphysema, as recognized by increased mean linear intercept (interalveolar distance). Despite clinical evidence of mucous hypersecretion, bronchial gland enlargement was present only in some patients. The younger patient showed normal small airways, but children and younger teenagers tended to have dilated small airways. Older teenagers and adult predominantly had stenosis of small airways. Varying patterns of pulmonary pathology are seen in cystic fibrosis and may be related to rapidity of progression of disease.
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PMID:Quantitative aspects of lung pathology in cystic fibrosis. 374 Jun 55

We used macroscopic morphometry and multivariate analysis (Hotelling's T2) to quantitate the extent of chronic lung disease and its regional distribution in formalin-inflated right lungs obtained at autopsy from 17 young adults with cystic fibrosis (CF) and 5 without CF as control subjects. Lungs were point-counted, by lobe, for 6 anatomic variables: parenchyma, nonparenchyma, bronchi, vessels, cysts, and emphysema, and the results were expressed for each as volume proportion (Vv). Considering all 6 variables simultaneously, CF lungs differed significantly from control lungs (p = 0.003). For individual variables, Vv bronchus was significantly elevated and Vv parenchyma was significantly reduced in the CF group. Within the CF lungs, there was a significant difference between upper and lower lobes when all variables were considered together (p = 0.001). For individual variables, Vv parenchyma and Vv vessels were significantly less, and Vv bronchus was significantly greater in the upper compared with the lower lobe. There was no difference in the upper versus lower lobe Vv for any variable in the control group. These results indicate that lung disease and remodeling are irregularly distributed in CF and that the upper lobe is disproportionately severely involved.
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PMID:Regional distribution of macroscopic lung disease in cystic fibrosis. 396 22

Four adult patients with biopsy-proven bronchiolitis were identified and prospectively evaluated. Each patient presented with the rapid onset (weeks to months) of severe respiratory disease that was clinically distinct from asthma, chronic bronchitis, bronchiectasis, cystic fibrosis, and emphysema. Bronchiolitis patients were evaluated by pulmonary function testing and bronchoalveolar lavage (BAL) before and after two months of treatment with 1 mg/kg/day of prednisone. Initial BAL results of bronchiolitis patients were compared to those of cigarette smokers with chronic bronchitis (n = 4), asymptomatic cigarette smokers (n = 5), and normal nonsmoking volunteers (n = 5). Neutrophils comprised 53 +/- 13 percent of the cells recovered by BAL in bronchiolitis patients but only 3 +/- 2 percent of the cells in chronic bronchitis patients, 1.5 +/- 0.6 percent of the cells in asymptomatic smokers, and 0.3 +/- 0.3 percent of the cells in normal volunteers (p less than 0.01, all comparisons). Moreover, prednisone produced a striking decrease in lower respiratory tract neutrophils (53 +/- 13 percent to 8 +/- 3 percent, p less than 0.05) in all bronchiolitis patients while lung function either improved (two patients) or remained unchanged (two patients). These findings suggest a central role for the neutrophil in bronchiolitis and argue that BAL may be clinically useful in the diagnosis and management of these patients.
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PMID:Adult bronchiolitis. Evaluation by bronchoalveolar lavage and response to prednisone therapy. 400 57

In recent years associations have been reported between male infertility and chronic pulmonary infections. These disorders include immotile-cilia syndrome, cystic fibrosis and Young's syndrome. For each disorder, case reports or small groups of patients have been described. However, it remains to be determined if these congenital disorders play a significant role in primary bronchiectasis (P.B). To do this, we compared the fertility rate of 35 patients (24 males, 11 females) with clinical and radiological evidence of P.B. and of 71 patients (54 males, 17 females) with secondary bronchiectasis (S.B) due to a defined chest disease (mainly tuberculosis and chronic bronchitis with emphysema). We abstracted the clinical records of this group of 106 patients, hospitalized between 1966 and 1983 for: age at the time of hospitalization, sex, diagnosis (P.B. vs. S.B.), association with sinusitis and situs inversus, socio-economic status, ethnic group, marital status and fertility (graded as 0 or 1+). The crude association between P.B. and infertility is strong (p greater than 0.10). However age may represent a confounding factor as patients with P.B. are younger (p less than 0.01) than patients with S.B., and more often single (p less than 0.01). Initially we restricted the analysis to married patients of both groups, and still got a significant association. Then we compared mean age in childless married people of both groups and observed a non-significant difference. Moreover, when we compared fertility in older patients, for example in people over 30 years old, the association still persists (p less than 0.03). In conclusion, the congenital disorders associating bronchiectasia and infertility must be looked for in the check up of P.B., all the more so as some can be cured of their infertility.
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PMID:[Epidemiological approach to the relationship between bronchiectasis and sterility]. 408 Dec 81

Twentyfour adults, aged 19-47 years, with congenitally nonfunctioning cilia were investigated by clinical examination, radiography, spirometry, and in most cases by tracheobronchial clearance measurements and ultrastructural examination of respiratory tract cilia and/or sperm tails. They all suffered from chronic bronchitis, rhinitis and sinusitis, and most of them had bronchiectasis and a history of otitis. Most had spirometrically overt obstructive lung disease and four radiological signs of emphysema. Clinical profile, lung function and mucociliary clearance data were compared to corresponding data from samples of patients with other obstructive lung diseases. These comparisons indicate that a long-standing impairment of mucociliary clearance is of pathogenetic importance in common chronic obstructive lung disease associated with chronic bronchitis, but not in emphysema associated with severe alpha 1-antitrypsin deficiency without chronic bronchitis, and not in asthma. In cystic fibrosis and in hypogammaglobulinemia an impaired clearance is not of primary pathogenetic significance but may contribute to the development of obstructive lung disease.
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PMID:The immotile-cilia syndrome compared to other obstructive lung diseases: a clue to their pathogenesis. 660 45

It has been previously shown that conventional methods of measuring the single-breath diffusing capacity of the lung for carbon monoxide (DLCOsb) overestimate diffusion in normal subjects when the expiratory flow rate is reduced, when the collection of the exhaled alveolar gas sample is delayed, and/or when too large an alveolar gas sample is collected. Conventional methods use a single breathholding equation to analyze the entire single-breath maneuver, which consists of inhalation, breathholding, and exhalation. We previously developed a method of calculating DLCOsb using 3 separate equations, one for each phase of the single-breath maneuver. Using this method, DLCOsb measurements are unaffected by changes in expiratory flow rate or by the size and timing of the alveolar gas sample, as these parameters are analytically included in the analysis. We hypothesized that in patients with air-flow obstruction, conventional methods of measuring DLCOsb would overestimate diffusion. We examined the effects of measuring DLCOsb using rapid inhalation and exhalation maneuvers in a group of normal subjects and in 3 groups of patients with emphysema, asthma, and cystic fibrosis, respectively. First, we found that, whereas conventional methods specify 750 ml or 1 L of gas to be used for dead space washout, the washout volumes, as measured from a continuous monitor of the helium concentration in the exhaled gas in the single-breath maneuver, exceeded 1 L in 26% of the study group.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Overestimation of the single-breath carbon monoxide diffusing capacity in patients with air-flow obstruction. 670 98

Animal models are of two types those that occur spontaneously and those that the scientist produces by artefact. One value of spontaneously occurring models is that if pathogenetic mechanisms are identified, they give new leads for the study of human disease. There is a need for spontaneously occurring examples of so-called primary or idiopathic pulmonary fibrosis, pulmonary hypertension (arterial or venous), and emphysema. Acquired or artefactual models of each of these conditions are available and have led to better understanding of the pathological changes, but they have not led to identification of the basic or primary abnormality. A naturally occurring model of cystic fibrosis could be a major event in our control of this disease. A spontaneously occurring form of asthma is needed as a bridge between experiment and patient. Artefactual models that are needed are of bronchopulmonary dysplasia and shock lung. There is probably enough agreement--but only just--on the nature of bronchopulmonary dysplasia for specific needs to be identified. Here the questions concern the choice of an appropriate species--or several--in which to study the premature lung and its adaptation to air breathing and supportive therapy. Knowledge of comparative anatomy and physiology must influence choice of species for certain models. For adult respiratory failure, or shock lung, a model is needed that progresses to pulmonary hypertension. Spontaneous models of interstitial pneumonia and of infection, both viral and bacterial, are needed. An animal model of a disease is only as useful as the questions we ask of it.
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PMID:Needs for animal models of human diseases of the respiratory system. 696 87

Emphysema, chronic bronchitis, asthma, and cystic fibrosis are often cited as examples of chronic airways obstruction, while sarcoidosis is typically restrictive. Approximately 15 percent of sarcoidosis patients, however, have airways obstruction clinically characterized by wheezing with granulomatous involvement of airways. Since the majority have Stage IV disease by chest radiograph, their lungs usually have honey-combining with pulmonary fibrosis, adhesions, cavities, and mediastinal distortion.Patients had a mixed ventilatory defect, but obstruction of large airways was present as shown by decreased specific airways conductances. Small airways obstruction was also present as shown by low instantaneous flows at the terminal portion of the maximum expiratory flow-volume curve and diminished helium response of this curve. The closing volume, however, was not very sensitive. Radioactive xenon washout from ventilation lung scans and N2 washout from the lungs were prolonged in patients with worse disease.The authors conclude that the obstructive type of physiologic pattern is more frequent than recognized in sarcoidosis, which like that of cystic fibrosis has some restrictive element and is characterized by poor reversibility to bronchodilators. A trial period of beclomethasone dipropionate aerosol was not helpful in two patients. Relief of this distressing airways obstruction continues to pose a challenging problem in management.
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PMID:Chronic airways obstruction in pulmonary sarcoidosis: its poor response to bronchodilators. 742 Apr 39

Mucociliary experiments conducted in vitro using avian tracheal rings show that serum from both normal and cystic fibrosis patients will cause ciliostasis in airways. The evidence suggests that endogenous mucus is not inhibitory to cilia and that inhibition observed with serum is due to blood clotting factors (probably fibrin), since a variety of anticoagulants and fibrin-destroying substances will either prevent or reverse the inhibitory effects of serum. Because fibrin and serum are normally found in the lung, we propose that an early event in the pathogenesis of many lung diseases (e.g., asthma, some forms of emphysema, cystic fibrosis), is an increase in the level of clotting factors in the respiratory tract. Based on these results, we provide a rationale for investigating the usefulness of a number of pharmacologic agents in the treatment of diseases related to mucostasis.
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PMID:Role of clotting factor(s) in the etiology of lung disease related to ciliostasis. 742 80


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