Abstract：
BACKGROUND:The natural history and optimal therapeutic management of emphysema associated with alpha-1 antitrypsin deficiency are poorly understood. The purpose of this cohort of patients with emphysema associated with alpha-1 antitrypsin deficiency is to describe their characteristics and to study the factors associated with the evolution of their pathology. MATERIALS AND METHODS:It is a multicentre, prospective, cohort study including all the patients in metropolitan France with alpha-1 antitrypsin deficiency associated with emphysema (diagnosed by CT scanning) and an obstructive ventilatory defect. All respiratory physicians will be approached. Patients meeting the inclusion criteria will be followed up every 6 months for 5 years. Clinical and spirometric data as well as those relating to quality of life will becollected. The principal criterion will be FEV1; the secondary criteria: quality of life, exacerbations requiring hospitalistion and mortality. EXPECTED RESULTS AND VIEWPOINTS:300-500 patients are expected to be included. At the conclusion incorporation of this cohort into AIR (Alpha-antitrypsin International Registry) will increase the statistical power. This study could also provide the base for prospective trials and research projects.
journal_name
Rev Mal Respirjournal_title
Revue des maladies respiratoiresauthors
Thabut Gdoi
10.1019/200530093subject
Has Abstractpub_date
2005-12-01 00:00:00pages
1053-7issue
6 Pt 1eissn
0761-8425issn
1776-2588pii
S0761-8425(05)85738-9journal_volume
22pub_type
杂志文章,多中心研究abstract：:60 patients were studied with severe chronic respiratory failure (IRC) and a permanent tracheostomy treated with domiciliary ventilation (VADT). The study commenced from the time of the tracheostomy and included length of survival (by the actuarial method) quality of life, subjectively and objectively (progress of blo...
journal_title：Revue des maladies respiratoires
pub_type： 杂志文章
doi：
更新日期：1985-01-01 00:00:00
abstract：INTRODUCTION:Since 2002, Belgian law has authorized the practice of euthanasia under certain clear conditions. All cases have to be reported to the Assessment and Control Commission (ACC). To date, more than 9000 cases have been reported. AIM:To make a statement about the Belgian experience requires consideration of s...
journal_title：Revue des maladies respiratoires
pub_type： 历史文章,杂志文章,评审
doi：10.1016/j.rmr.2015.10.006
更新日期：2016-10-01 00:00:00
abstract：INTRODUCTION:Primary ciliary dyskinesia (PCD) is an inherited disease responsible for a disruption of normal ciliary function. Its clinical presentation is usually in early childhood with pulmonary and otorhinolaryngologic symptoms. Early diagnosis is essential to avoid the development of bronchiectasis. The aim of the...
journal_title：Revue des maladies respiratoires
pub_type： 杂志文章,评审
doi：10.1016/j.rmr.2011.02.014
更新日期：2011-09-01 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:This review presents the state of knowledge regarding the acute and chronic toxicity of diesel engine exhaust in humans. STATE OF ART:The health effects of diesel engine exhaust, which is a complex mixture of gas and particulate matter (ultrafine and fine particles), are mainly irritation of the respirato...
journal_title：Revue des maladies respiratoires
pub_type： 杂志文章,评审
doi：10.1016/j.rmr.2014.07.012
更新日期：2015-02-01 00:00:00
abstract：:Respiratory handicap due to neurological diseases is often underestimated. Given clinical signs are either mild or absent, systematic measurement of the vital capacity is the best mean to detect in practice the restrictive syndrome. The onset of home mechanical ventilatory support should be decided at steady state, ap...
journal_title：Revue des maladies respiratoires
pub_type： 杂志文章,评审
doi：
更新日期：1998-09-01 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：INTRODUCTION:Between 5 and 7% of babies are born prematurely. In the paediatric age group, the respiratory morbidity of these patients is well known, particularly in cases of bronchopulmonary dysplasia (BPD). On the other hand, very few data are available concerning their adult respiratory status. BACKGROUND:There are...
journal_title：Revue des maladies respiratoires
pub_type： 杂志文章,评审
doi：10.1016/j.rmr.2011.05.015
更新日期：2011-12-01 00:00:00
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：INTRODUCTION:Although guidelines recommend the prescription of written asthma action plans (WAAP), their use remains limited. METHODS:A prospective survey was performed from 2013 to 2014. We interviewed respiratory physicians, paediatric respiratory physicians and allergologists taking care of asthmatic patients and p...
journal_title：Revue des maladies respiratoires
pub_type： 杂志文章
doi：10.1016/j.rmr.2015.10.001
更新日期：2016-05-01 00:00:00
abstract：INTRODUCTION:Most admissions for asthma are preventable. The objective of this study was to describe readmissions for asthma. METHODS:We used the nationwide hospital database to identify readmissions for asthma in patients aged two to 44 years recorded in metropolitan France between 2002 and 2005. We selected patients...
journal_title：Revue des maladies respiratoires
pub_type： 杂志文章
doi：10.1016/j.rmr.2011.09.023
更新日期：2011-11-01 00:00:00
abstract：:Cardiopulmonary exercise testing (CPET) is the most comprehensive investigation for understanding the mechanisms responsible for dyspnea in patients with chronic respiratory disease. The two observations presented here illustrate how CPET can contribute to the management of patients with interstitial lung diseases. A ...
journal_title：Revue des maladies respiratoires
pub_type： 杂志文章,评审
doi：10.1016/j.rmr.2014.04.101
更新日期：2014-10-01 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:The histology and staging of bronchial carcinoma determines the treatment options for the condition. Endobronchial ultrasound allows the needle aspiration of mediastinal lymph nodes or pulmonary neoplasia where there is tracheo-bronchial contact under visual control. This procedure is aid for diagnosis and...
journal_title：Revue des maladies respiratoires
pub_type： 杂志文章
doi：10.1016/s0761-8425(08)74350-x
更新日期：2008-09-01 00:00:00
abstract：INTRODUCTION:Exhaled carbon monoxide (ECO) concentration provides an easy, fast and non invasive means of assessing smoking status. However, some difficulties have to be considered when interpreting ECO levels. BACKGROUND:CO is a non-specific biomarker of tobacco exposure. Its half-life is short (2-6 hours). CO reflec...
journal_title：Revue des maladies respiratoires
pub_type： 杂志文章,评审
doi：10.1016/j.rmr.2009.09.004
更新日期：2010-04-01 00:00:00
abstract：INTRODUCTION:Patients suffering from chronic obstructive pulmonary disease (COPD) are often admitted to hospital with an exacerbation of their disease that manifests itself with an increase in cough and/or sputum, increased dyspnoea and sometimes chest pain. These episodes are most often attributed to an acute exacerba...
journal_title：Revue des maladies respiratoires
pub_type： 杂志文章,多中心研究
doi：10.1016/s0761-8425(04)71420-5
更新日期：2004-09-01 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:This questionnaire-based epidemiological study was aimed at identifying possible sleep disturbance in a sample of active French males. METHODS:Eight hundred and fifty male subjects, aged 22 to 66 years, completed a structured sleep questionnaire supplemented by information about their life habits and medi...
journal_title：Revue des maladies respiratoires
pub_type： 杂志文章
doi：10.1016/j.rmr.2010.07.003
更新日期：2010-09-01 00:00:00
abstract：:An experimental model of lung obstruction has been achieved in the cat reared in our laboratory. 30 young adults (J) and one adult subject (A) had tracheal stenosis from 8 to 15 weeks and were the object of a study of pulmonary airways and were compared with four young controls and seven older controls. The presence o...
journal_title：Revue des maladies respiratoires
pub_type： 杂志文章
doi：
更新日期：1989-01-01 00:00:00
abstract：:We report a case of a man aged 36, in whom a diagnosis of mucoviscidosis was diagnosed based on the association of diffuse bronchiectasis and obstructive azoospermia. We discussed some clinical and biological aspects leading to the diagnosis of mucoviscidosis in the adult. ...
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：:Pleurisies due to the syndrome of ovarian hyperstimulation typically accompany ascites and in their most severe form are associated with haemoconcentration, hypovolaemia and thromboembolic phenomena. It is not unusual in this context for pleural effusions to be isolated. They may occur during treatment for sterility b...
journal_title：Revue des maladies respiratoires
pub_type： 杂志文章
doi：
更新日期：1996-01-01 00:00:00
abstract：INTRODUCTION:The correct use of medication and the ability to assess the severity of the disease and to react appropriately in the case of exacerbation are essential objectives in the management of asthma patients. This study, conducted in a school of asthma in Seine-Saint-Denis, aims to measure the influence of socio-...
journal_title：Revue des maladies respiratoires
pub_type： 杂志文章
doi：10.1016/j.rmr.2014.02.008
更新日期：2015-03-01 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：INTRODUCTION:This study analyzes the relationship between the occurrence of spontaneous pneumothorax and atmospheric pressure. PATIENTS AND METHODS:We conducted a retrospective study on 74 patients hospitalized for spontaneous pneumothorax in the department of respiratory diseases from the Ibn Roshd university hospita...
journal_title：Revue des maladies respiratoires
pub_type： 杂志文章
doi：10.1016/j.rmr.2018.04.008
更新日期：2019-01-01 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：BACKGROUND:Exercise-induced desaturation is a well-described phenomenon in COPD patients during exercise assessments such as the six minute walk test (6MWT). Some of the pathophysiological mechanisms involved in this O2 desaturation could be modified by individualized exercise training as part of a pulmonary rehabilita...
journal_title：Revue des maladies respiratoires
pub_type： 杂志文章
doi：10.1016/s0761-8425(07)91126-2
更新日期：2007-05-01 00:00:00
abstract：:Toxocariasis is a frequent disease in children, but the severe clinical manifestations are rare in the literature (diffuse interstitial pneumonia with hypoxaemia and acute severe asthma). The diagnosis is made thanks to the reliability of serological techniques (the ELISA test and using antigen excretion-secretion tes...
journal_title：Revue des maladies respiratoires
pub_type： 杂志文章
doi：
更新日期：1994-01-01 00:00:00
abstract：:Clinical data support the hypothesis that aggressivity is inhibited amongst asthmatics. The relationship of violence, as a marker of aggressiveness, to asthma was examined in a population-based sample of 12.466 students of secondary schools in France (Enquête sur la Santé de l'adolescent 1993/1994) using an epidemiolo...
journal_title：Revue des maladies respiratoires
pub_type： 杂志文章
doi：
更新日期：2001-06-01 00:00:00
abstract：:The authors report the observation of multiple epitheliomas of the back in a patient submitted to numerous radiological examination forty years earlier. They recall the likely risk of the appearance of these cutaneous tumours after this type of exposure. ...
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:Aspergillosis colonisation in cystic fibrosis (CF), usually due to Aspergillus fumigatus (AF), classically presents as allergic bronchopulmonary aspergillosis. However, aspergillus infection can produce a range of manifestations: from minor colonization to an invasive infection. CASE REPORT:A 14-year-old ...
journal_title：Revue des maladies respiratoires
pub_type： 杂志文章
doi：10.1016/s0761-8425(06)71600-x
更新日期：2006-09-01 00:00:00
