Abstract:
:The occurrence of pulmonary fibrosis in numerous individuals from the same family suggests a genetic cause for the disease. During the last 10 years, mutations involving proteins from the telomerase complex and from the surfactant system have been identified in association with pulmonary fibrosis. Mutations of TERT, the coding gene for the telomerase reverse transcriptase, are the most frequently identified mutations and are present in 15% of cases of familial pulmonary fibrosis. Other mutations (TERC, surfactant proteins genes) are only rarely evidenced in adults. Patients with mutations involving the telomerase complex may present with pulmonary fibrosis, hematologic, cutaneous or liver diseases. Other genetic variations associated with pulmonary fibrosis such as a polymorphism in the promoter of MUC5B or a polymorphism in TERT have been recently described, and could be considered to be part of a polygenic transmission. Evidence for mutations associated with the development of pulmonary fibrosis raises numerous clinical questions from establishing a diagnosis, providing counselling to deciding on therapy, and requires specific studies. From a pathophysiological point of view, the function of the genes highlights the central role of alveolar epithelium and aging in fibrogenesis.
journal_name
Rev Mal Respirjournal_title
Revue des maladies respiratoiresauthors
Borie R,Kannengiesser C,Nathan N,Tabèze L,Pradère P,Crestani Bdoi
10.1016/j.rmr.2014.07.017subject
Has Abstractpub_date
2015-04-01 00:00:00pages
413-34issue
4eissn
0761-8425issn
1776-2588pii
S0761-8425(14)01143-7journal_volume
32pub_type
杂志文章,评审abstract:INTRODUCTION:Mechanical ventilation is associated with ventilator-induced diaphragmatic dysfunction (VIDD) in animal models and also in humans. BACKGROUND:The main pathophysiological pathways implicated in VIDD seems to be related to muscle inactivity but may also be the consequence of high tidal volumes. Systemic ins...
journal_title:Revue des maladies respiratoires
pub_type: 杂志文章,评审
doi:10.1016/j.rmr.2014.08.013
更新日期:2015-04-01 00:00:00
abstract::We reviewed the records of 15 Human Immunodeficiency Virus (HIV) infected patients with pulmonary cryptococcosis (PC). PC was the first AIDS-defining manifestation in nine patients. HIV infection was identified simultaneously with the onset of PC in 4 patients. The CD4+ lymphocyte count was low in all cases (median, 2...
journal_title:Revue des maladies respiratoires
pub_type: 杂志文章
doi:
更新日期:1997-11-01 00:00:00
abstract::A 28-year-old girl suffering from lysinuric protein intolerance was referred on account of severe chronic respiratory failure. Since childhood she had failure to thrive with chronic hyperammonaemia and interstitial lung disease but normal respiratory function. At the age of 21, she experienced haemoptysis and worsenin...
journal_title:Revue des maladies respiratoires
pub_type: 杂志文章
doi:10.1016/j.rmr.2010.06.022
更新日期:2010-09-01 00:00:00
abstract:INTRODUCTION:Rates of lung cancer in women have been increasing continually for several years. The basic surgical management of this condition is the same in both sexes but a number of differences are apparent. METHODS:We analysed data entered onto the Epithor database between June 2002 and June 2006 concerning 8535 s...
journal_title:Revue des maladies respiratoires
pub_type: 杂志文章
doi:10.1016/s0761-8425(07)91390-x
更新日期:2007-09-01 00:00:00
abstract::Allergic bronchopulmonary aspergillosis (ABPA) is an immunologic bronchopulmonary inflammation due to an immune response of the lower respiratory tract against Aspergillus fumigatus. The major clinical features of ABPA include asthma, recurrent pulmonary infiltrates, immediate wheal and flare skin reactivity to A. fum...
journal_title:Revue des maladies respiratoires
pub_type: 杂志文章,评审
doi:
更新日期:2000-02-01 00:00:00
abstract::The diaphragm is the muscle most implicated in breathing. Its morphological exploration usually depends on pulmonary radiography, fluoroscopy, CT-scanning and MRI. Its function is evaluated by the classical respiratory functional tests, the measurement of maximum inspiratory and expiratory pressures, the transdiaphrag...
journal_title:Revue des maladies respiratoires
pub_type: 杂志文章,评审
doi:10.1016/j.rmr.2017.02.005
更新日期:2017-06-01 00:00:00
abstract:INTRODUCTION:Many pregnant women are asthmatics and maternal asthma is a source of questions and complications concerning both the progress of the pregnancy itself and the impact on the foetus. In this situation good asthma control is essential as the disease can deteriorate with acute exacerbations, possibly precipita...
journal_title:Revue des maladies respiratoires
pub_type: 杂志文章,评审
doi:10.1016/s0761-8425(09)74042-2
更新日期:2009-04-01 00:00:00
abstract:INTRODUCTION:Despite the development of international guidelines for the management of asthma, epidemiological surveys suggest that control of the disease remains poor. In order to investigate clinical practice a postal questionnaire was used to study the evaluation of asthma control, the assessment of factors associat...
journal_title:Revue des maladies respiratoires
pub_type: 杂志文章
doi:10.1016/s0761-8425(05)85475-0
更新日期:2005-04-01 00:00:00
abstract:OBJECTIVES:To estimate the prevalence of treated chronic obstructive pulmonary disease (COPD) and its associated costs by stage of severity. METHODS:The study was conducted on the 2011 data of the french general beneficiary sample database (EGB). EGB is a 1/97th sample of the whole population of the beneficiaries of t...
journal_title:Revue des maladies respiratoires
pub_type: 杂志文章
doi:10.1016/j.rmr.2014.10.731
更新日期:2015-09-01 00:00:00
abstract::We report a case of eosinophilic pneumonia secondary to taking Tetracycline whose severity required the use of mechanical ventilation and steroid therapy. On the basis of this case, we review the characteristics of drug-induced pneumonia as well as the differential diagnosis which are evoked. ...
journal_title:Revue des maladies respiratoires
pub_type: 杂志文章
doi:
更新日期:1996-10-01 00:00:00
abstract:BACKGROUND:To update full time educated youth data on smoking prevalence related to gender and to assess perception and behaviour related to smoking, we have led a self-administrated question-study from January to June 2002 in Abidjan with students from 8 to 22 years old. METHODS:Our population was divided in 3 groups...
journal_title:Revue des maladies respiratoires
pub_type: 杂志文章
doi:10.1016/s0761-8425(04)71410-2
更新日期:2004-09-01 00:00:00
abstract:BACKGROUND:Optimal duration of first line chemotherapy for non surgical non small cell lung cancer (NSCLC) is still debated. It concerns the length of initial chemotherapy and the use of maintenance treatments. METHODS:Literature review was performed on maintenance and duration of chemotherapy. RESULTS:The use of mai...
journal_title:Revue des maladies respiratoires
pub_type: 杂志文章,评审
doi:
更新日期:2006-11-01 00:00:00
abstract::The measurement of non-allergic bronchial hyperreactivity brings limited information in the diagnosis of asthma, whether for epidemiological purposes or for an individual patient. Its sensitivity and specificity in the general population amount to 46 and 89% respectively. It follows that the positive and negative pred...
journal_title:Revue des maladies respiratoires
pub_type: 杂志文章,评审
doi:
更新日期:1994-01-01 00:00:00
abstract::Passive smoking, measured in practice by using specific biomarkers, is a well known factor of morbidity and mortality. The main victims are children, often starting from conception, but adults are not spared. Many respiratory diseases are caused and/or worsened by passive smoking and environmental tobacco smoke (ETS) ...
journal_title:Revue des maladies respiratoires
pub_type: 杂志文章
doi:10.1016/s0761-8425(09)74696-0
更新日期:2009-06-01 00:00:00
abstract::In order to achieve mediastinal lymph node staging in bronchial cancer, axial mediastinoscopy (combined with left anterior mediastinoscopy for cancers of the left upper lobe) is by far the most efficient and the most reliable technique. Since mediastinoscopy has been part of the investigations that can be made before ...
journal_title:Revue des maladies respiratoires
pub_type: 杂志文章,评审
doi:
更新日期:1992-01-01 00:00:00
abstract:INTRODUCTION:Epidemiological studies of chronic obstructive pulmonary disease (COPD) are rare in the developing countries, particularly in Viet Nam where the consumption of tobacco continues to increase. The aim of this study was to evaluate the feasibility of early screening of smokers for bronchial obstruction using ...
journal_title:Revue des maladies respiratoires
pub_type: 杂志文章
doi:10.1016/s0761-8425(09)72583-5
更新日期:2009-03-01 00:00:00
abstract::The authors report an "analytic epidemiological" study, showing the risk of relapse in tuberculous patients in the Bas-Rhin in 1967 to 1977. This was possible thanks to statutory notification of tuberculous cases, with an index updated by annual returns. The study relates to French subjects notified as new cases of al...
journal_title:Revue des maladies respiratoires
pub_type: 杂志文章
doi:
更新日期:1984-01-01 00:00:00
abstract::An acute haemodynamic study was carried out prospectively in 6 patients fulfilling the diagnostic criteria of systemic sclerosis with pulmonary arterial hypertension. This study consisted of 7 baseline measurements and the effect of 6 drugs administered intravenously and known for their effects as vasodilators in the ...
journal_title:Revue des maladies respiratoires
pub_type: 杂志文章
doi:
更新日期:1990-01-01 00:00:00
abstract:INTRODUCTION:Kerosene pneumonitis is rare. It most commonly occurs in children who have ingested a hydrocarbon by accident. In adults, pneumonitis caused by aspiration of incompletely vaporized kerosene may be seen in fire-eaters can cause pneumonitis. CASE REPORTS:We report six cases of fire-eater's pneumonitis, from...
journal_title:Revue des maladies respiratoires
pub_type: 杂志文章
doi:
更新日期:2003-12-01 00:00:00
abstract::We report the case of a 73 year old man presenting with severe hypoxaemia due to an anatomical right to left shunt from a persistent left superior vena cava (VCSG) anastomosing with the left auricle. This was fortuitously discovered while placing a left sub clavicular catheter; the diagnosis of the VCSG draining into ...
journal_title:Revue des maladies respiratoires
pub_type: 杂志文章
doi:
更新日期:1986-01-01 00:00:00
abstract::Based on immunoassay techniques used to identify and quantify major indoor allergens, epidemiological studies have demonstrated that level of exposure depends on the geographical situation (climate, urbanization) and that there is a relationship between exposure and sensitization of predisposed patients. Most of the m...
journal_title:Revue des maladies respiratoires
pub_type: 杂志文章
doi:
更新日期:2000-02-01 00:00:00
abstract::The imaging techniques used to investigate patients with asthma and to assess the effects of asthma treatments include computed tomography (CT), helium magnetic resonance imaging (MRI), single-photon emission computed tomography (SPECT), and positron-emission tomography (PET). Only MRI does not involve radiation expos...
journal_title:Revue des maladies respiratoires
pub_type: 杂志文章,评审
doi:10.1016/j.rmr.2011.05.001
更新日期:2011-06-01 00:00:00
abstract:INTRODUCTION:The epidemiology of chronic beryllium disease (CBD) in France is poorly understood. The aim of this study was to determine the number of prevalent cases of CBD in France between 2010 and 2014. METHODS:We conducted a national survey using a specific questionnaire distributed by the professional pathology s...
journal_title:Revue des maladies respiratoires
pub_type: 杂志文章
doi:10.1016/j.rmr.2020.03.003
更新日期:2020-05-01 00:00:00
abstract::Chest radiograph and computed tomography are the most appropriate imaging tools for detecting asbestos-related pleural and parenchymal disease due to their availability and performances. The cost and irradiation delivery of conventional chest X-rays are limited. Technical parameters and reading should be standardized....
journal_title:Revue des maladies respiratoires
pub_type: 杂志文章,评审
doi:
更新日期:1999-12-01 00:00:00
abstract:INTRODUCTION:The anti-Hu associated syndrome is a rare paraneoplastic neurological syndrome, which is principally associated with small-cell lung cancer. OBSERVATION:We report the case of a patient with the following clinical features: dysautonomia with severe gastroparesis, sensory neuropathy and a rhombencephalitis....
journal_title:Revue des maladies respiratoires
pub_type: 杂志文章
doi:10.1016/j.rmr.2010.02.006
更新日期:2010-03-01 00:00:00
abstract:INTRODUCTION:To prevent and control chronic respiratory diseases, the WHO has outlined a programme to be applied in all the countries. In Sub-Saharan countries like Senegal, its application faces many barriers. METHODS:This work is a detailed analysis of the main areas of the programme based on the socio-economic, cul...
journal_title:Revue des maladies respiratoires
pub_type: 杂志文章,评审
doi:10.1016/s0761-8425(04)71352-2
更新日期:2004-06-01 00:00:00
abstract::Cypress belongs to the Cupressaceae family, which includes 140 species with non-deciduous foliage. The most important genera in allergic diseases are Cupressus sempervirens or Green cypress, Cupressus arizonica or Blue cypress, Juniperus oxycedrus, Juniperus communis and Thuya. Because J. oxycedrus pollinates in Octob...
journal_title:Revue des maladies respiratoires
pub_type: 杂志文章,评审
doi:10.1016/j.rmr.2013.09.014
更新日期:2013-12-01 00:00:00
abstract::The objective of this study was to determine the prevalence of skin sensitization to certain pollens in some regions of Morocco. This multicentric study involving 10 centres included a consecutive series of 640 patients consulting for the first time between february and april 1998 for asthma and/or rhinitis and/or con...
journal_title:Revue des maladies respiratoires
pub_type: 杂志文章,多中心研究,评审
doi:
更新日期:2001-10-01 00:00:00
abstract:INTRODUCTION:Chest physiotherapy is regularly prescribed for children, particularly in cystic fibrosis. Gastro-oesophageal reflux is common in this disease and is associated with certain chest physiotherapy manoeuvres. AIM OF THE STUDY:To evaluate the influence of two chest physiotherapy techniques on gastro-oesophage...
journal_title:Revue des maladies respiratoires
pub_type: 杂志文章
doi:10.1016/j.rmr.2015.02.004
更新日期:2015-05-01 00:00:00
abstract::A 74-year-old woman was hospitalised for pleuro-pneumonia of the right base. The chest x-ray showed the presence of a right paratracheal opacity which persisted during the course of the infectious episode. A computed tomographic scan of the thorax showed a voluminous anterior mediastinal mass which compressed the trac...
journal_title:Revue des maladies respiratoires
pub_type: 杂志文章
doi:
更新日期:1997-12-01 00:00:00