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Query: UMLS:C0034069 (pulmonary fibrosis)
 7,050 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 38-year-old patient with effort dyspnea, somnolence, cianosis and cor pulmonale is presented. Chest roentgenograms and lung function studies suggested the diagnosis of pulmonary fibrosis. The patient showed also severe hypercapnia with normal resting ventilation and ventilatory response to exercise lower than usual for this condition. Autopsy confirmed the clinical diagnosis. This subject may belong to the growing group of patients where CO2 retention is not explained by their pulmonary pathology.
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PMID:Idiopathic interstitial pulmonary fibrosis with hypercapnia. 117 39

We have studied the mode of ventilation and chemosentivity in 10 patients suffering from pulmonary fibrosis. The total lung capacity was on average 63.5 +/- 8% of the predicted. Their static compliance was 0.078 +/- 0.05 l.cm of water. The patients were studied in the prone position breathing ambient air then on hyperoxia. The response to CO2 was assessed according to the rebreathing method of Read. The results of these patients were compared with those of 11 normal subjects. The ventilation at rest was normal, with a shortened respiratory time and a Ti/Ttot ratio which was lowered. The occlusion pressure (P0.1) was very much higher than that in normal subjects. This rise was correlated with an increase in pulmonary elastance and a reduction in vital capacity. The correction of hypoxia was without effect on the respiratory parameters. In relation to normal subjects the ventilatory response to carbon dioxide in fibrotics was decreased whilst the response of the P0.1 was increased expressing central hyperactivity. In conclusion, fibrotic patients have normal ventilation in spite of an increase in inspiratory work. This normal ventilation results from hyperactivity of the respiratory centre, as in the hyperventilation induced by carbon dioxide when at rest.
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PMID:[The control of respiration in pulmonary fibrosis. The effect of O2 and CO2]. 190 51

Chronic obstructive pulmonary disease (COPD) is equated with chronic bronchitis and emphysema as one disease entity. In COPD airflow limitation is relatively persistent--unlike asthma. Tests for "small-airways disease" form no part of routine practice, for their accuracy in detecting pathological change is debatable. The proteolytic theory of the pathogenesis of emphysema highlights the role of neutrophil elastase, antielastases, oxidants, antioxidants, and thus of potential new treatments. Clinical features of COPD include breathlessness, cough, and sputum, with airflow obstruction and lung hyperinflation. The differential diagnosis includes bronchiectasis, cystic fibrosis, and pulmonary hypertension, but pulmonary fibrosis, etc., is distinguished by radiological infiltrates. Plain chest radiography cannot reliably diagnose emphysema in life, but a new method measuring lung density from the computed tomographic (CT) scan allows location, quantitation, and diagnosis of emphysema (defined by enlargement of distal air spaces) in humans in life. "Pink puffers" with breathlessness, hyperinflation, mild hypoxemia, and a low PCO2 are contrasted with "blue bloaters" with hypoxemia, secondary polycythemia, CO2 retention, and pulmonary hypertension and cor pulmonale. Antismoking measures are a major aim in management. A bronchodilator regimen combining a slow-release oral theophylline with an inhaled beta 2-agonist, ipratropium, and high-dose inhaled steroids is proposed because even modest improvement in obstruction can help these patients. In acute exacerbations with purulent sputum, antimicrobials against Streptococcus pneumoniae and Hemophilus influenzae are used with controlled oxygen therapy aiming to keep the arterial PO2 over 50 mm Hg without the pH falling below 7.25. Influenza prophylaxis is recommended, but pneumococcal vaccination remains debatable. Chronic under-nutrition in "emphysema" implies controlled trials of feeding regimens--but these remain to be assessed. Long-term oxygen therapy is the only treatment known to prolong life in blue bloaters, and oxygen concentrators and transtracheal oxygen delivery are discussed. Pulmonary vasodilators (e.g., beta 2-agonists, hydralazine, nifedipine, angiotensin-converting enzyme [ACE] inhibitors, etc.) have not yet been proved to provide long-term reduction in pulmonary arterial pressure. Blue bloaters have severe nocturnal hypoxemia in rapid eye movement (REM) sleep that is corrected by oxygen or the investigational drug almitrine.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Chronic obstructive pulmonary disease. 304 40

In four normal subjects and 51 patients with chronic bronchitis-emphysema, bronchial asthma and pulmonary fibrosis, the VD/VT ratio was measured by a helium washout technique (wVD/VT) and by the conventional Bohr's method using the arterial CO2 tension (VD/VT). In 16 patients the wVD/VT ratio was compared with that calculated from Bohr's equation with the alveolar CO2 tension measured by the rebreathing technique (AVD/VT). It was found that the values for the VD/VT ratio obtained by the helium washout technique were very close to those obtained by the conventional method using the PaCO2 or PACO2 values.
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PMID:Calculation of the VD/VT ratio by the helium washout technique. 308 19

Amiodarone, an antiarrhythmic drug, causes pulmonary fibrosis in some patients during chronic treatment but the mechanism is unknown. We studied the effects of amiodarone on pulmonary biochemistry, morphology and function at doses of 25 and 50 mg/kg/12 hr given to rats by gavage for four weeks. Plasma and pulmonary phospholipids were significantly augmented, 13% and 88% respectively, in the group given amiodarone 50 mg/kg/12 hr compared to pair-fed controls. Typical phospholipidosis-like light and electron microscopic alterations were seen in the lung, their severity related to the extent of biochemical changes induced by amiodarone. Pulmonary function tests revealed mild but not significant changes in O2 and CO2 alveolar exchange efficiency and lung compliance (P-V curve) of treated animals in comparison to pair fed controls. Plasma average concentrations of amiodarone and its main metabolite, desethylamiodarone, after four weeks were 2.46 +/- 0.18 and 0.73 +/- 0.13 micrograms/ml, respectively, in the 50 mg/kg/12 hr group. In the same group amiodarone and desethylamiodarone concentrations in lung were 163 +/- 26 and 569 +/- 153 times higher than those in plasma. A highly significant correlation was found between amiodarone concentrations in plasma and lung and phospholipid content in the lung. A subgroup of animals received amiodarone 50 mg/kg/12 hr for 8 weeks. The pulmonary phospholipidosis-like lesions were similar to those observed after one month of treatment, no fibrosis was evident on light microscopic examination.
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PMID:Amiodarone induced phospholipidosis. Biochemical, morphological and functional changes in the lungs of rats chronically treated with amiodarone. 366 36

Paraquat (PQ) is a herbicide known to generate O2 radicals and to injure lung epithelial cells, leading eventually to pulmonary fibrosis. To test for the possible existence of a direct cytotoxic action of PQ on endothelial cells, we have studied, for up to 5 days, the action of 10(-6) to 10(-4) M PQ on primary cultures of pig aortic endothelial cells and compared these effects to those obtained with exposure to 95% O2-5% CO2. The decrease in DNA and protein content of Petri dishes and the increase in lactate dehydrogenase release were found to depend on PQ concentration and the duration of exposure to PQ. The toxic effects of hyperoxia were intermediate, ranging between those obtained with 10(-5) and 10(-4) M PQ. Hyperoxia and 10(-4) M PQ produced a similar marked inhibition of DNA synthesis after a 1-day period of exposure. Combined exposure to both PQ and hyperoxia resulted in changes comparable to those obtained with hyperoxia alone (decrease in protein and DNA content) or PQ alone (lactate dehydrogenase release). Additive effects were seen only for the inhibition of DNA synthesis. The selenomethionine-related increase in glutathione peroxidase activity had a protective effect against hyperoxia-induced lactate dehydrogenase release but not against PQ induced cytolysis. Finally, shorter exposures to O2 and PQ revealed the existence of a trend toward recovery only for cells exposed to hyperoxia. The prolonged toxic action of PQ could not be related to PQ accumulation and storage by endothelial cells. These studies indicate that PQ can exert a direct, dose-dependent, and prolonged cytotoxic effect on cultured endothelial cells.
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PMID:Direct toxic effects of paraquat and oxygen on cultured endothelial cells. 396 1

During muscular exercise, the alveolo-capillary gas exchange reaches its optimal capacity, but taking blood for arterial blood gases is associated with certain risks and the classical criteria of CO transfer in the steady state are difficult to interpret as they are influenced by age, sex, ventilatory regime. The "specific" CO uptake (VCO Sp) does not correspond to these criteria (6). When related to the ERCO2, it allows the DuCO to be determined (4). It has the same value at the 3rd minute and at the 10th minute of constant exercise (5). Like TCO, it is correlated with the PaO2 during effort (5). In this study, a triangular exercise was performed by men aged between 45 add 55 years, smokers and former smokers, classified into 4 A: 12 healthy subjects; B: 56 cases of chronic respiratory disease; C: 9 cases of chronic obstructive airways disease (COAD); D: 12 cases of diffuse pulmonary fibrosis (DPF). Groups C and D were derived from group B. The VCO SP, TCO and DuCO were measured at each plateau of the triangular exercise. In the patients, the PaO2 was measured at the same time as the FF (CO, CO2). In the last phase of exercise (Ex. Max.): VCO Sp was more frequently altered at rest and more strongly correlated to TCO; TCO/V had the same significance as DuCO. The results of TCO, VCO Sp and DuCO were compared between groups A, C and D.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Steady-state carbon monoxide transfer during progressive muscular exercise in patients. Relation to PaO2. Value of specific VCO]. 402 33

Non-invasive measurement of pulmonary blood flow by acetylene rebreathing was performed in patients with pulmonary diseases. Cardiac output measurement by thermodilution method was also used simultaneously. A fast computation system was coupled with the measurement. Fourteen emphysema patients and 7 pulmonary fibrosis patients were the subjects. Ten patients with normal lung function were normal controls. After the measurement of cardiac output, the subjects rebreathed mixed gas containing acetylene and argon from the end tidal level. The inspiratory and expiratory gas was measured by mass-spectrometer. A lung model made of two compartments connected in series, a compartment of the rebreathing bag and a compartment of the lung were used for the analysis, while taking account of the volume reduction due to O2 and CO2 exchange and the fluctuation of end tidal gas concentration until equilibrium between the bag and the lung was reached. Values in 9 emphysema cases and 6 fibrosis cases were within or near the 90% confidence limits of control. Five emphysema cases were obviously out of the range, and their pulmonary blood flow was less than the cardiac output and their mean arterial PCO2 was higher than that of the other emphysema cases. These cases were considered to have blood flow to the sites with low alveolar ventilation per unit volume.
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PMID:[Determination of pulmonary blood flow by acetylene rebreathing method in patients with chronic respiratory disease]. 834 4

In 16 patients with pulmonary fibrosis, an artificial pneumothorax was introduced using the Veress cannula and the Saugman water manometer. Atmospheric air was introduced by fractionated insufflation to a total volume of 800 ml (median). The interpleural space was found on the first attempt, and in all cases, fractionated insufflation of atmospheric air was conducted while the intrapleural pressure was controlled with the water manometer. In one case, the procedure was stopped because of a rise in the pleural pressure after insufflation of only 50 ml air. This was undoubtedly caused by pleural adhesions not visible on chest X-ray. The main concern with air insufflation is air embolism but this was not observed clinically in any of the present cases. The patients in the present study all suffered from pulmonary fibrosis judged by clinical examination, chest X-ray and pulmonary function tests. Despite a diffusion capacity (DCO/VA) with a median value of 48% expected, the procedure was well tolerated. It has previously been shown that artificial pneumothorax preceding thoracoscopy is well tolerated due to hyperventilation, with an increase in respiratory frequency and a fall in arterial CO2 concentration (PaCO2), while pH and arterial O2 concentration (PO2) remain constant. This probably also explains the tolerance of the patients in this material. Insufflation of air as described here should be restricted to senior pulmonologists because it is an infrequent procedure. The present authors found the procedure to be uncomplicated and easy to perform with little discomfort to the patients.
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PMID:Artificial pneumothorax by the Veress cannula: efficacy and safety. 932 40

It was hypothesized that neutrophil elastase released from activated neutrophils may play an important role in the pathogenesis of pulmonary fibrosis. In the present study, we measured the neutrophil elastase:alpha-1-proteinase inhibitor complex (E-PI) in serum and bronchoalveolar lavage fluid (BALF) in 26 patients with pulmonary fibrosis and evaluated the correlation between E-PI levels and several parameters. E-PI levels in serum of patients with pulmonary fibrosis (635.8+/-112.0 ng.mL(-1)) were significantly elevated compared to normal nonsmokers (122.0+/-4.0 ng.mL(-1)) as well as normal smokers (132.8+/-8.4 ng.mL(-1)) (p<0.01). E-PI levels in serum significantly correlated with hepatocyte growth factor (HGF) levels in serum, C-reactive protein (CRP), and negatively correlated with arterial oxygen tension (Pa,O2), and arterial carbon dioxide tension (Pa,CO2). E-PI/albumin levels in BALF significantly correlated with HGF/albumin levels in BALF, lactate dehydrogenase (LDH)/albumin in BALF, total number of inflammatory cells (alveolar macrophages and neutrophils) in BALF, and several markers derived from epithelial cells in BALF. Our data demonstrated: 1) neutrophil elastase:alpha-1-proteinase inhibitor complex in serum increased in patients with pulmonary fibrosis; and 2) neutrophil elastase:alpha-1-proteinase inhibitor complex in serum and bronchoalveolar lavage fluid correlated with clinical parameters in pulmonary fibrosis. These results suggest that neutrophil elastase may play a significant role in the process of lung injury in pulmonary fibrosis.
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PMID:Neutrophil elastase: alpha-1-proteinase inhibitor complex in serum and bronchoalveolar lavage fluid in patients with pulmonary fibrosis. 954 80


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