Presse Medicale最新文献_第6页

The European reference network on rare lung diseases (ERN-LUNG) ambition to offer better lung health for all 欧洲罕见肺部疾病参考网络（ERN-LUG）致力于为所有人提供更好的肺部健康。

IF 2.7 3区医学

Presse Medicale Pub Date : 2023-09-01 DOI: 10.1016/j.lpm.2023.104172

Marc Humbert , Thomas Wagner

引用次数: 0

Bronchiectasis 支气管扩张。

IF 2.7 3区医学

Presse Medicale Pub Date : 2023-09-01 DOI: 10.1016/j.lpm.2023.104174

Miguel Barbosa, James D. Chalmers

{"title":"Bronchiectasis","authors":"Miguel Barbosa, James D. Chalmers","doi":"10.1016/j.lpm.2023.104174","DOIUrl":"10.1016/j.lpm.2023.104174","url":null,"abstract":"<div>Bronchiectasis is a final common pathway of a wide variety of underlying conditions including infectious, autoimmune, allergic, genetic and inflammatory conditions. Patients experience a chronic disease with variable clinical symptoms and course, but most experience cough, sputum production and recurrent exacerbations. Symptoms of bronchiectasis lead to poor quality of life and exacerbations are the major driver of morbidity and mortality. Patients are often chronically infected with bacteria with the most common being Pseudomonas aeruginosa and Haemophilus influenzae. Treatment of bronchiectasis includes standardised testing to identify the underlying cause with targeted treatment if immune deficiency, allergic bronchopulmonary aspergillosis or non-tuberculous mycobacterial infection, for example, are identified. Airway clearance is the mainstay of therapy for patients with symptoms of cough and sputum production. Frequently exacerbating patients may benefit from long term antibiotic or mucoactive therapies. Bronchiectasis is a heterogeneous disease and increasingly precision medicine approaches are advocated to target treatments most appropriately and to limit the emergence of antimicrobial resistance.</div>","PeriodicalId":20530,"journal":{"name":"Presse Medicale","volume":"52 3","pages":"Article 104174"},"PeriodicalIF":2.7,"publicationDate":"2023-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41148274","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Lymphangioleiomyomatosis 淋巴管平滑肌瘤病。

IF 2.7 3区医学

Presse Medicale Pub Date : 2023-09-01 DOI: 10.1016/j.lpm.2023.104173

Davide Elia , Roberto Cassandro , Antonella Caminati , Francesca Luisi , Sergio Harari

引用次数: 0

Cystic fibrosis 囊性纤维化

IF 2.7 3区医学

Presse Medicale Pub Date : 2023-09-01 DOI: 10.1016/j.lpm.2023.104169

Isabelle Fajac , Pierre-Régis Burgel

{"title":"Cystic fibrosis","authors":"Isabelle Fajac , Pierre-Régis Burgel","doi":"10.1016/j.lpm.2023.104169","DOIUrl":"10.1016/j.lpm.2023.104169","url":null,"abstract":"<div>Cystic fibrosis (CF) is an autosomal recessive genetic disease caused by variants in the gene encoding for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. CFTR dysfunction results in abnormal chloride and bicarbonate transport in epithelial cells, leading to a multiorgan disease dominated by respiratory and digestive manifestations. The respiratory disease, which is characterized by airway mucus plugging, chronic bacterial infection and progressive development of bronchiectasis, may lead to chronic respiratory failure, which is the main cause of premature death in people with CF. Over the past 50 years, major progress has been obtained by implementing multidisciplinary care, including nutritional support, airway clearance techniques and antibiotics in specialized CF centers. The past 10 years have further seen the progressive development of oral medications, called CFTR modulators, that partially restore ion transport and lead to a major improvement in clinical manifestations and lung function, presumably resulting in longer survival. Although an increasing proportion of people with CF are being treated with CFTR modulators, challenges remain regarding access to CFTR modulators due to their high cost, and their lack of marketing approval and/or effectiveness in people with rare CFTR variants. The anticipated increase in the number of adults with CF and their aging also challenge the current organization of CF care. The purpose of this review article is to describe current status and future perspective of CF disease and care.</div>","PeriodicalId":20530,"journal":{"name":"Presse Medicale","volume":"52 3","pages":"Article 104169"},"PeriodicalIF":2.7,"publicationDate":"2023-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10142490","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Pulmonary arterial hypertension 肺动脉高压

IF 2.7 3区医学

Presse Medicale Pub Date : 2023-09-01 DOI: 10.1016/j.lpm.2023.104168

Athénaïs Boucly , Christian Gerges , Laurent Savale , Xavier Jaïs , Mitja Jevnikar , David Montani , Olivier Sitbon , Marc Humbert

{"title":"Pulmonary arterial hypertension","authors":"Athénaïs Boucly , Christian Gerges , Laurent Savale , Xavier Jaïs , Mitja Jevnikar , David Montani , Olivier Sitbon , Marc Humbert","doi":"10.1016/j.lpm.2023.104168","DOIUrl":"10.1016/j.lpm.2023.104168","url":null,"abstract":"<div>Pulmonary arterial hypertension (PAH) is a rare and progressive disease characterised by remodelling of the pulmonary arteries and progressive narrowing of the pulmonary vasculature. This leads to a progressive increase in pulmonary vascular resistance and pulmonary arterial pressure and, if left untreated, to right ventricular failure and death. A correct diagnosis requires a complete work-up including right heart catheterisation performed in a specialised centre.Although our knowledge of the epidemiology, pathology and pathophysiology of the disease, as well as the development of innovative therapies, has progressed in recent decades, PAH remains a serious clinical condition. Current treatments for the disease target the three specific pathways of endothelial dysfunction that characterise PAH: the endothelin, nitric oxide and prostacyclin pathways.The current treatment algorithm is based on the assessment of severity using a multiparametric risk stratification approach at the time of diagnosis (baseline) and at regular follow-up visits. It recommends the initiation of combination therapy in PAH patients without cardiopulmonary comorbidities. The choice of therapy (dual or triple) depends on the initial severity of the condition. The main treatment goal is to achieve low-risk status. Further escalation of treatment is required if low-risk status is not achieved at subsequent follow-up assessments. In the most severe patients, who are already on maximal medical therapy, lung transplantation may be indicated.Recent advances in understanding the pathophysiology of the disease have led to the development of promising emerging therapies targeting dysfunctional pathways beyond endothelial dysfunction, including the TGF-β and PDGF pathways.</div>","PeriodicalId":20530,"journal":{"name":"Presse Medicale","volume":"52 3","pages":"Article 104168"},"PeriodicalIF":2.7,"publicationDate":"2023-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10137362","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

International variability in the diagnosis and management of disorders of consciousness 意识障碍的诊断和管理的国际差异

IF 2.7 3区医学

Presse Medicale Pub Date : 2023-06-01 DOI: 10.1016/j.lpm.2022.104162

Ariane Lewis

{"title":"International variability in the diagnosis and management of disorders of consciousness","authors":"Ariane Lewis","doi":"10.1016/j.lpm.2022.104162","DOIUrl":"10.1016/j.lpm.2022.104162","url":null,"abstract":"<div>This manuscript explores the international variability in the diagnosis and management of disorders of consciousness (DoC). The identification, evaluation, intervention, exploration, prognostication and limitation of therapy for patients with DoC is reviewed through an international lens. The myriad factors that impact the diagnosis and management of DoC including 1) financial, 2) legal and regulatory, 3) cultural, 4) religious and 5) psychosocial considerations are discussed. As data comparing patients with DoC internationally are limited, findings from the general critical care or neurocritical care literature are described when information specific to patients with DoC is unavailable.There is a need for improvements in clinical care, education, advocacy and research related to patients with DoC worldwide. It is imperative to standardize methodology to evaluate consciousness and prognosticate outcome. Further, education is needed to 1) generate awareness of the impact of the aforementioned considerations on patients with DoC and 2) develop techniques to optimize communication about DoC with families. It is necessary to promote equity in access to expertise and resources for patients with DoC to enhance the care of patients with DoC worldwide. Improving understanding and management of patients with DoC requires harmonization of existing datasets, development of registries where none exist and establishment of international clinical trial networks that include patients in all phases along the spectrum of care. The work of international organizations like the Curing Coma Campaign can hopefully minimize international variability in the diagnosis and management of DoC and optimize care.</div>","PeriodicalId":20530,"journal":{"name":"Presse Medicale","volume":"52 2","pages":"Article 104162"},"PeriodicalIF":2.7,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10736479","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 1

The current and future contribution of neuroimaging to the understanding of disorders of consciousness 神经影像学对理解意识障碍的当前和未来贡献

IF 2.7 3区医学

Presse Medicale Pub Date : 2023-06-01 DOI: 10.1016/j.lpm.2022.104163

Naji Alnagger , Paolo Cardone , Charlotte Martial , Steven Laureys , Jitka Annen , Olivia Gosseries

{"title":"The current and future contribution of neuroimaging to the understanding of disorders of consciousness","authors":"Naji Alnagger , Paolo Cardone , Charlotte Martial , Steven Laureys , Jitka Annen , Olivia Gosseries","doi":"10.1016/j.lpm.2022.104163","DOIUrl":"10.1016/j.lpm.2022.104163","url":null,"abstract":"<div>Patients with disorders of consciousness (DoC) represent a group of severely brain-injured patients with varying capacities for consciousness in terms of both wakefulness and awareness. The current state-of-the-art for assessing these patients is through standardised behavioural examinations, but inaccuracies are commonplace. Neuroimaging and electrophysiological techniques have revealed vast insights into the relationships between neural alterations, andcognitive and behavioural features of consciousness in patients with DoC. This has led to the establishment of neuroimaging paradigms for the clinical assessment of DoC patients. Here, we review selected neuroimaging findings on the DoC population, outlining key findings of the dysfunction underlying DoC and presenting the current clinical utility of neuroimaging tools. We discuss that whilst individual brain areas play instrumental roles in generating and supporting consciousness, activation of these areas alone is not sufficient for conscious experience. Instead, for consciousness to arise, we need preserved thalamo-cortical circuits, in addition to sufficient connectivity between distinctly differentiated brain networks, underlined by connectivity both within, and between such brain networks. Finally, we present recent advances and future perspectives in computational methodologies applied to DoC, supporting the notion that progress in the science of DoC will be driven by a symbiosis of these data-driven analyses, and theory-driven research. Both perspectives will work in tandem to provide mechanistic insights contextualised within theoretical frameworks which ultimately inform the practice of clinical neurology.</div>","PeriodicalId":20530,"journal":{"name":"Presse Medicale","volume":"52 2","pages":"Article 104163"},"PeriodicalIF":2.7,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10740145","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 1

Disorders of Consciousness: navigating between nihilism and unrealistic hopes 意识障碍：在虚无主义和不切实际的希望之间徘徊。

IF 2.7 3区医学

Presse Medicale Pub Date : 2023-06-01 DOI: 10.1016/j.lpm.2023.104182

Benjamin Rohaut

引用次数: 0

Epistemological challenges for neural correlates of consciousness: A defense of medical research on consciousness 意识的神经相关性的认识论挑战：对意识医学研究的辩护。

IF 2.7 3区医学

Presse Medicale Pub Date : 2023-06-01 DOI: 10.1016/j.lpm.2023.104183

Armance Neveu , Vincent Degos , Anouk Barberousse

引用次数: 0

Heuristics and biases in medical decision-making under uncertainty: The case of neuropronostication for consciousness disorders 不确定性条件下医疗决策中的启发式和偏见：意识障碍的神经支配案例。

IF 2.7 3区医学

Presse Medicale Pub Date : 2023-06-01 DOI: 10.1016/j.lpm.2023.104181

Camille Lakhlifi , Benjamin Rohaut

{"title":"Heuristics and biases in medical decision-making under uncertainty: The case of neuropronostication for consciousness disorders","authors":"Camille Lakhlifi , Benjamin Rohaut","doi":"10.1016/j.lpm.2023.104181","DOIUrl":"10.1016/j.lpm.2023.104181","url":null,"abstract":"<div>Neuropronostication for consciousness disorders can be very complex and prone to high uncertainty. Despite notable advancements in the development of dedicated scales and physiological markers using innovative paradigms, these technical progressions are often overshadowed by factors intrinsic to the medical environment. Beyond the scarcity of objective data guiding medical decisions, factors like time pressure, fatigue, multitasking, and emotional load can drive clinicians to rely more on heuristic-based clinical reasoning. Such an approach, albeit beneficial under certain circumstances, may lead to systematic error judgments and impair medical decisions, especially in complex and uncertain environments. After a brief review of the main theoretical frameworks, this paper explores the influence of clinicians' cognitive biases on clinical reasoning and decision-making in the challenging context of neuroprognostication for consciousness disorders. The discussion further revolves around developing and implementing various strategies designed to mitigate these biases and their impact, aiming to enhance the quality of care and the patient safety.</div>","PeriodicalId":20530,"journal":{"name":"Presse Medicale","volume":"52 2","pages":"Article 104181"},"PeriodicalIF":2.7,"publicationDate":"2023-06-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41210480","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0