Abstract：
:Respiratory disease is the major cause of morbidity and mortality in cystic fibrosis. Cystic fibrosis was long treated in the pediatric setting, but improved survival has led to the implication of adult pneumology in therapeutic management. Since the gene causing cystic fibrosis has been clone, our knowledge of the pathophysiology of the disease has literally exploded, particularly concerning the deleterious consequences of defective expression and distribution of CFTR (cystic fibrosis transmembrane conductance regulator) protein in chronic lung inflammation and infection. This knowledge has led to an optimization of existing therapeutic strategies and to the formulation of hypotheses for the development of new pharmaceutical reagents, allowing an assessment of disease outcome not only in terms of survival but also in terms of quality of life. Early in vivo clinical trials have been encouraging although the efficacy of gene transfer and expression remain modest and the optimal vector remains to be determined. Different potential pharmacological approaches are being studied in order to correct for defective CFTR function at different levels of gene mutation, and to modulate the disorder in transepithelial ionic transfer. One could expect in the near future to see combinations of complementary genotype-specific drugs used for the treatment of cystic fibrosis after patient genotyping to categorize the type of mutation.
journal_name
Rev Mal Respirjournal_title
Revue des maladies respiratoiresauthors
Reynaud-Gaubert Msubject
Has Abstractpub_date
1999-09-01 00:00:00pages
495-509issue
4eissn
0761-8425issn
1776-2588pii
MDOI-RMR-08-1999-16-4-0761-8425-101019-ART64journal_volume
16pub_type
杂志文章,评审abstract：INTRODUCTION:Lung hyperinflation (LH) has become a major concern in the management of chronic obstructive pulmonary disease (COPD). MAIN AIM:To evaluate the role of lung volumes in the positive diagnosis of COPD and in the assessment of airway obstruction reversibility. POPULATION AND METHODS:Three hundred and sixty-...
journal_title：Revue des maladies respiratoires
pub_type： 杂志文章
doi：10.1016/j.rmr.2013.05.009
更新日期：2014-01-01 00:00:00
abstract：:Inhaled medications are widely used in patients suffering from bronchial diseases. Beside their pharmacological properties, nebulised solutions have physico-chemical characteristics that can alter bronchial reactivity. Non-isotonic solutions can induce a bronchial hyperresponsiveness and/or a severe bronchonconstricti...
journal_title：Revue des maladies respiratoires
pub_type： 杂志文章
doi：
更新日期：1998-04-01 00:00:00
abstract：:Pleuropulmonary blastoma is an uncommon malignant lung tumor observed in children. Outcome is often unfavorable. Two boys and two girls, mean age 4.5 years, were admitted for nonspecific respiratory signs. Oriented by radiology findings, the diagnosis of pleuropulmonary blastoma was confirmed at pathology examination ...
journal_title：Revue des maladies respiratoires
pub_type： 杂志文章
doi：
更新日期：1999-06-01 00:00:00
abstract：AIM:To our knowledge, the proportion of active tuberculosis diagnosed after 12-18 months during a screening tuberculosis process in a specialized centre (centre de lutte antituberculeuse [CLAT]) has not been described in France. The majority of other countries do not have any recommendation to screen at this time. MET...
journal_title：Revue des maladies respiratoires
pub_type： 杂志文章
doi：10.1016/j.rmr.2014.06.031
更新日期：2015-09-01 00:00:00
abstract：BACKGROUND:There is no standard second-line treatment for small cell lung cancer (SCLC). The prognosis of these patients is poor and special attention should be paid to both quality of life and economic factors. METHODS:The aim of this phase II randomised trial (GFPC0501) is to compare, in patients with progressive SC...
journal_title：Revue des maladies respiratoires
pub_type： 杂志文章,多中心研究,随机对照试验
doi：10.1016/s0761-8425(07)91136-5
更新日期：2007-05-01 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：OBJECTIVE:The management of COPD aims to improve integrated indices such as health-related quality of life (HRQoL). Experts recommend repeated and methodical assessment of HRQoL, particularly by the use of questionnaires. Though these tools give pertinent information for groups of patients, they have limitations in des...
journal_title：Revue des maladies respiratoires
pub_type： 杂志文章
doi：10.1016/j.rmr.2010.03.020
更新日期：2010-05-01 00:00:00
abstract：:Primary malignant melanoma of the lower airways is very rare. 24 cases have been collected over the past century. Our case was a patient of 52 years of age who had been discovered to have a tracheal tumour which was found to be a malignant melanoma and was treated with a Laser. The major problem is to establish that t...
journal_title：Revue des maladies respiratoires
pub_type： 杂志文章
doi：
更新日期：1990-01-01 00:00:00
abstract：UNLABELLED:Severe pneumonia due to cytomegalovirus in chronic obstructive pulmonary disease. INTRODUCTION:We describe two cases of immunocompetent patients with chronic obstructive pulmonary disease (COPD) who developed severe cytomegalovirus (CMV) pneumonia. The clinical and radiological context and CMV replication i...
journal_title：Revue des maladies respiratoires
pub_type： 杂志文章
doi：10.1016/j.rmr.2013.09.013
更新日期：2014-05-01 00:00:00
abstract：INTRODUCTION:Whereas numerous case reports have described statin-induced lung injuries, statin-induced pleural effusions are uncommon. CASE REPORT:An 84-year-old man presented with bilateral pleural effusions two years after starting treatment with atorvastatin. No other cause of pleural effusion was found and all sym...
journal_title：Revue des maladies respiratoires
pub_type： 杂志文章
doi：10.1016/j.rmr.2015.12.010
更新日期：2016-09-01 00:00:00
abstract：:Acquired hemophilia A (AHA) is a rare disease, defined by the production of anti-factor VIII antibodies causing disordered hemostasis. It is idiopathic in 50% of cases, but sometimes associated with solid tumors. We report a case where AHA led to the diagnosis of lung cancer. CASE REPORT:An 82-year-old man with spont...
journal_title：Revue des maladies respiratoires
pub_type： 杂志文章
doi：10.1016/j.rmr.2017.08.005
更新日期：2018-09-01 00:00:00
abstract：:We report a study of 8 patients with acute Mycoplasma pneumoniae infection of the respiratory tract admitted to the Army Hospital Desgenettes over a 10 months period. Our clinical observations are compared with a review of the literature. We observed a seasonal outbreak in spring and autumn. This infection was encount...
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：BACKGROUND:A written action plan (WAP) reduces emergency visits for asthma exacerbations. However, a WAP is underused and often focused on asthma control. The innovation is an AppWeb that includes an expert software aimed at diagnosing the level of severity of asthma exacerbations and delivering a personalized digital ...
journal_title：Revue des maladies respiratoires
pub_type： 杂志文章,多中心研究,随机对照试验
doi：10.1016/j.rmr.2016.10.881
更新日期：2017-11-01 00:00:00
abstract：:Broncho-alveolar lavage is a simple investigation to carry out and enables the gathering of biological information such as inflammatory and immuno-competent cells, tumour cells, microorganisms as well as mineral particles which are found in the biological milieux present in the distal air spaces. The performance of LB...
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：METHODS:A questionnaire was sent to 50 000 general practicioners (GP) and specialists. RESULTS:4,898 physicians (9.4%) responded, including 59.1% GP, 16.9% pneumologists, 13% pediatricians and about 10% other specialists, ENT, allergologists, and intensivists. The main reason for pneumologists to prescribe nebulizatio...
journal_title：Revue des maladies respiratoires
pub_type： 杂志文章
doi：10.1016/s0761-8425(08)70465-0
更新日期：2008-01-01 00:00:00
abstract：INTRODUCTION:Pulmonary arterial hypertension (PAH) is related to hypertrophic remodelling of the pulmonary vessels. Proliferation of pulmonary vascular smooth muscle cells (PA-SMCs) plays a key role in these changes. BACKGROUND:The PA-SMCs of patients suffering from idiopathic or secondary PAH are characterized by abn...
journal_title：Revue des maladies respiratoires
pub_type： 杂志文章,评审
doi：
更新日期：2006-04-01 00:00:00
abstract：:AIDS was the cause of the majority of deaths from HIV infection before 1996 but since the introduction of antiretroviral therapies the causes of mortality have changed considerably. In 2010, 75 % of deaths were due to diseases other than AIDS, the majority being cancers. Lung cancer is the most common in terms of both...
journal_title：Revue des maladies respiratoires
pub_type： 杂志文章,评审
doi：10.1016/j.rmr.2013.07.009
更新日期：2014-02-01 00:00:00
abstract：:Surgery is the ultimate curative treatment for resectable non-small cell lung cancer (NSCLC). However, the prognosis for operated patients remains disappointing. Multiple randomized studies have shown that administering perioperative chemotherapy improves the prognosis and increases the cure rate by around 3-5%. The p...
journal_title：Revue des maladies respiratoires
pub_type： 杂志文章,评审
doi：10.1016/j.rmr.2020.12.002
更新日期：2021-01-01 00:00:00
abstract：:Several filters specific for respiratory function tests have been on the market for several years. Recommended by the manufacturers to avoid contaminating the equipment and thus improve patient safety, these filters require a considerable financial investment. We studied the passage of diluted blood into artificial sa...
journal_title：Revue des maladies respiratoires
pub_type： 杂志文章
doi：
更新日期：1998-12-01 00:00:00
abstract：BACKGROUND:Patients with chronic hypercapnic respiratory failure due to chronic obstructive pulmonary disease (COPD) are very likely to develop acute exacerbations. Non-invasive ventilation is often used to treat acute respiratory failure but little information is available about the benefits of domiciliary non-invasiv...
journal_title：Revue des maladies respiratoires
pub_type： 杂志文章,多中心研究,随机对照试验
doi：10.1016/j.rmr.2012.09.006
更新日期：2012-11-01 00:00:00
abstract：INTRODUCTION:Pulmonary arterial hypertension is a pulmonary vascular disease with a poor prognosis. Continuous intravenous treatment with prostacyclin analogues requires the placement of a tunnelled catheter. The occurrence of catheter-related infections in this context is unusual due to the alkaline pH of the prostacy...
journal_title：Revue des maladies respiratoires
pub_type： 杂志文章
doi：10.1016/j.rmr.2017.03.037
更新日期：2018-03-01 00:00:00
abstract：:Numerous types of cells have a capacity for movement in physiological or pathological situations. For example, this is the case for inflammatory cells in the lung, during acute lobar pneumonia, sarcoidosis and idiopathic pulmonary fibrosis. Cellular migration is a general process which rests on the interaction between...
journal_title：Revue des maladies respiratoires
pub_type： 杂志文章,评审
doi：
更新日期：1990-01-01 00:00:00
abstract：:Some data suggest that survival in non small cell lung cancer (NSCLC) has improved in the last ten years. A comparison between large prospective therapeutic trials from 1979 to 2002 does show some progress but there are many biases, especially the Will Rogers effect, where stage migration due to new methods of diagnos...
journal_title：Revue des maladies respiratoires
pub_type： 杂志文章,评审
doi：
更新日期：2005-12-01 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：:A search for an allergic cause in asthma is fundamental to the diagnosis. Typically it is based on: A clinical history. Specific cutaneous and inhaled provocation tests which can produce a possible reaction (early and delayed) which are of great pathophysiological and therapeutic interest. In vitro tests (which enable...
journal_title：Revue des maladies respiratoires
pub_type： 杂志文章
doi：
更新日期：1986-01-01 00:00:00
abstract：INTRODUCTION:The degree of dyspnoea and muscular dysfunction during an exacerbation of COPD are so severe in some patients that they are unable to follow a traditional exercise training programme based on whole body activity (walking, cycling). Some alternative strategies like electrostimulation may be considered to in...
journal_title：Revue des maladies respiratoires
pub_type： 杂志文章,评审
doi：10.1016/j.rmr.2009.08.004
更新日期：2010-02-01 00:00:00
abstract：:This study was carried out between 1973 and 1982 on the proportional mortality by lung cancer of a group of workers living either in Brussells or in Wallonia and receiving compensation for silicosis or anthrasilicosis. The majority of patients suffered from anthrasilicosis (more than 96%) and had been exposed to the r...
journal_title：Revue des maladies respiratoires
pub_type： 杂志文章
doi：
更新日期：1984-01-01 00:00:00
abstract：INTRODUCTION:The aim of our study was to compare the features at diagnosis in patients with ornithosis to patients with avian acute hypersensitivity pneumonitis (HP). Clinical, biological and radiological differences could potentially help clinicians to distinguish these diseases. METHODS:We conducted a retrospective ...
journal_title：Revue des maladies respiratoires
pub_type： 杂志文章
doi：10.1016/j.rmr.2017.11.013
更新日期：2019-03-01 00:00:00
abstract：:The authors report a case of a plasma cell granuloma in a young woman of 19 with a past history of congenital rubella, presenting with repeated small haemoptyses related to a pedunculated endo-bronchial tumour. The diagnosis was only made per-operatively, permitting an effective excision. ...
journal_title：Revue des maladies respiratoires
pub_type： 杂志文章
doi：
更新日期：1991-01-01 00:00:00
abstract：INTRODUCTION:A chylothorax can occur following any intrathoracic procedure. It is generally straightforward to make the diagnosis but optimal management can be problematic. METHODS:Between 1995 and 2002, three women and one man aged from 13 to 58 years were treated for chylothorax after thoracic surgery. Their initial...
journal_title：Revue des maladies respiratoires
pub_type： 杂志文章
doi：10.1016/s0761-8425(06)71479-6
更新日期：2006-04-01 00:00:00
