Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0034067 (emphysema)
11,506 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The respiratory symptoms of gastro-oesophageal reflux, which sometimes includes massive and fatal inhalation, are well-known in infants. In older children the digestive signs are not clinically evident and the reflux mainly, if not exclusively, can be translated by recurring respiratory symptoms. The series of 36 cases presented in this work concerns children between 3 months and 15 years old, for whom the first signs were respiratory, with often a silent gastro-oesophageal reflux for several months, and even several years in some cases. The physiopathology of the respiratory symptoms concerns principally the repeated alimentary aspiration and/or gastric content during nocturnal decubitus. The pulmonary lesions caused by the reflux can be either localized, with atelectasis, obstructive emphysema or bronchiectasis, or generalized with granulomatous reactions around the food particles. Other respiratory conditions such as asthma or cystic fibrosis can be also associated with gastro-oesophageal reflux. The diagnostic criteria are discussed.
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PMID:[Recurrent bronchopneumopathies caused by gastroesophageal reflux in children. Clinical, x-ray and histologic studies of 36 cases]. 61 80

The kinesitherapeutic and functional re-educational approach is indicated for each type of respiratory insufficiency, special attention being paid to those syndromes and diseases most frequently encountered in clinical practice. In the case of these (asthma, mucoviscidosis, emphysema, bronchorrhoea), the essential phases of kinesitherapy and re-education are outlined in greater detail. Clinically and psychologically, the conclusions sum up the attitude which should be assumed by the attending staff in cases of patients with respiratory insufficiency. Finally, the importance of physiopathological and clinical data and physiokinesitherapeutic experience are recalled.
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PMID:[Functional re-education in various types of respiratory insufficiency]. 85 94

The autopsies of 82 patients with cystic fibrosis were reviewed with respect to pathologic changes in the lungs and their respective prevalence among different age groups. Although bronchitis, mucopurulent plugging, and bronchopneumonia were almost universally present among children of all ages, epithelial metaplasia and bronchiectasis were rarer among infants and progressively more prevalent in older age groups. Emphysema was absent in patients under two years of age and affected 11 per cent of the patients two to six years of age and 40 per cent of the patients older than six years, but was never of a severe degree by the point count method. Pulmonary hemorrhage, although uncommon, was usually associated with prominent arterial vessels in walls of bronchiectatic airways. Quantitative assessment of bronchial glands revealed Reid indices significantly higher in patients with cystic fibrosis when compared to noncystic fibrosis patients, but there was no increase in these indices with the age of the patients. Glandular hypertrophy, predominance of mucous acini within glands, and goblet cell hyperplasia of the bronchial mucosa all suggest an explanation for the copious mucous secretion of patients with cystic fibrosis. However, it was not possible to ascertain whether these findings reflect a general exocrine defect of such patients or whether they were merely a response to chronic airway infection, even though the latter is a more plausible assumption.
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PMID:The lung in cystic fibrosis. A quantitative study including prevalence of pathologic findings among different age groups. 126 16

There were 1,536 lung transplants reported to the St. Louis International Lung Transplant Registry as of September 1, 1992. The number of centers performing lung transplants increased each year. The 1- and 2-year actuarial survival statistics for all transplants were 68% and 60%, respectively. The most common indication for transplantation was chronic obstructive pulmonary disease, followed by idiopathic pulmonary fibrosis, emphysema secondary to alpha-1 antitrypsin deficiency, and cystic fibrosis. Among the total of 492 deaths reported (34%), sepsis was the leading cause of death.
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PMID:Current status of lung transplantation--report of the St. Louis International Lung Transplant Registry. 130 24

Between November 1989 and April 1991, 14 bilateral single lung transplantations (BSLT) were performed at our institution using the technique we have described without omentoplasty and rarely cardiopulmonary bypass. The indications included emphysema (8), cystic fibrosis (3), infected fibrosis (1), alveolar microlithiasis (1) and lymphocytic interstitial pneumonitis (1). Maximum mean pulmonary artery pressure was 53 mmHg and minimal right ventricular ejection fraction was 15%. Two patients experienced bronchial complications: 1 complete left bronchial dehiscence, 1 late partial stenosis which required a temporary insertion of a stent. One patient had a posterior dehiscence which healed spontaneously. Five patients died postoperatively (3 of infection, 1 after a volume mismatch and 1 after a circulating anticoagulant). BSLT is the technique of choice for double lung transplantation in adults and heart lung transplantation has very few indications in infected end-stage pulmonary disease. We hope that modification of our immunosuppressive regimen will decrease postoperative mortality.
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PMID:Bilateral single lung transplantation. Complications and results in 14 patients. 851 59

The sensitivity of high-resolution computed tomography (HRCT) in identifying the pulmonary lesions of cystic fibrosis (CF) was evaluated. Thirty-nine patients (16 males, 23 females; mean age 19.1 years) were examined by chest HRCT. According to Shwachman and Kulczycki criteria, the clinical score of the patients ranged from 40 to 95, thus covering most possible variations of lung disease severity. All the patients presented diffuse thickening of bronchial walls, expression of the characteristic CF bronchial inflammation. Bronchiectases were the second most common lung lesions: discrete dilatation of bronchi was observed in 87% of cases; the localization, pattern and extent of bronchiectasis were accurately detected by HRCT. Pleural thickening and hilar adenopathy were frequently identified (in 64% and 82% of the patients, respectively). Bronchoceles were seen in 64% of the patients; atelectasis (33%) and subpleural bullous dystrophic emphysema (28%) were observed less frequently. On HRCT, the localization of the disease processes within the secondary pulmonary lobule was possible in all patients. In agreement with international literature, the identification of these lesions confirms HRCT as the more sensitive technique for early visualization and location of the manifestations of CF bronchopathy. A larger range of experience coming from a systematic use of HRCT in chronic inflammatory lung diseases would increase our knowledge of pathogenetic processes and allow improvement of therapeutic perspectives.
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PMID:High-resolution computed tomography in cystic fibrosis. 142 47

Significant progress has been made since the first successful human heart-lung transplantation (HLT) for pulmonary vascular disease performed in 1981. The refinement of surgical techniques, use of cyclosporin as the main immunosuppressant, technique of distant organ procurement to expand the donor organ pool, and improved diagnosis and management of pulmonary infection and rejection have all contributed to this accomplishment. This has inevitably coincided with the extension of this procedure to other groups of patients with end stage heart and lung disease. Initially, HLT was offered to patients with cardiac disease associated with pulmonary hypertension. Because of the success, consideration was given to transplantation for parenchymal pulmonary diseases, initially pulmonary fibrosis and emphysema, and then suppurative lung disease such as in cystic fibrosis (CF). However, the application of HLT to patients with CF lagged behind because of concern related to the risk of sepsis, the systemic nature of the disease, malnourishment, and fear of recurrence of the epithelial CF defect in the transplanted lungs.
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PMID:Lung transplantation for cystic fibrosis. 145 9

Forty-four patients affected with cystic fibrosis (CF) were examined by means of high-resolution Computed Tomography (HRCT) of the chest; the sensitivity of this technique was evaluated in identifying small pulmonary structure alterations, and its role outlined. In all cases, HRCT scans allowed early lobular shape anomalies to be detected and secondary pulmonary lobular lesions to be correlated with disease evolution. A classification for lobular lesions was proposed: Stage I - Thickening of the bronchovascular bundle and/or intralobular septa and/or middle interstitial compartment. Unessential changes in lobular ventilation. Preservation of lobular shape. Stage II - Intralobular emphysema. Acinar filling with normal ventilation (A) or diffuse hypoventilation of the lobule (B). Preservation of lobular shape. Stage III - Lobular consolidations. Preservation of lobular shape. Stage IV - Patch distribution of lesions characterized by derangement of lobular structures. Changes in normal lobular shape. The identification of these lesions confirms HRCT as the most sensitive technique for the early visualization and localization of signs of CF bronchopneumonopathy. A broader experience coming from the systematic use of HRCT in chronic inflammatory lung diseases would increase our knowledge of pathogenetic processes and allow therapeutic perspectives to be improved.
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PMID:[High-resolution computerized tomography. Staging of lobular lesions in cystic fibrosis]. 149 77

The types of lung transplants are single lung (performed on either side), double lung en bloc (which requires total cardiopulmonary bypass and myocardial protection), and bilateral-sequential lung transplant (which uses separate single lung transplants with separate main stem bronchial anastomoses). Single lung transplants are used for restrictive lung disorders, such as pulmonary fibrosis; double lung procedures are used for patients with obstructive lung disease, such as emphysema, and bilateral-sequential lung transplantation is used in patients diagnosed with generalized bronchiectasis, including cystic fibrosis, or any other chronically infected lung disease. The criteria for differentiating single versus bilateral transplantation is constantly being evaluated and redefined. The future of lung transplantation depends on continued research, improved methods of preservation, improved care to potential donors, and an increase in the availability of donors.
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PMID:Pulmonary transplant. Transplantation for end-stage lung disease: a nurse's perspective. 153 47

The protease-antiprotease imbalance is thought to be involved in a variety of destructive lung diseases: pulmonary emphysema, chronic bronchitis, cystic fibrosis and adult respiratory distress syndrome. Bronchoalveolar lavage allowed the investigators to assess the protease-antiprotease shift in such conditions but sometimes gave conflicting results. The role of bronchoalveolar lavage as a research and diagnostic tool in diseases characterised by protease-antiprotease imbalance is reviewed, as well as its potential usefulness in the near future.
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PMID:Protease-antiprotease imbalance: local evaluation with bronchoalveolar lavage. 157 28


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