Respirology : official journal of the Asian Pacific Society of Respirology
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Neuromuscular diseases represent a heterogeneous group of disorders of the muscle, nerve or neuromuscular junction. The respiratory muscles are rarely spared in neuromuscular diseases even if the type of muscle involvement, severity and time course greatly varies among the different diseases. Diagnosis of respiratory muscle weakness is crucial because of the importance of respiratory morbidity and mortality. ⋯ The measurement of oesogastric pressures can be helpful as they allow the diagnosis and quantification of paradoxical breathing, as well as the assessment of the strength of the inspiratory and expiratory muscles by means of the oesophageal pressure during a maximal sniff and of the gastric pressure during a maximal cough. Sleep assessment should also be part of the respiratory evaluation of children with neuromuscular disease with at least the recording of nocturnal gas exchange if polysomnography is not possible or unavailable. This improvement in the assessment of respiratory muscle performance may increase our understanding of the respiratory pathophysiology of the different neuromuscular diseases, improve patient care, and guide research and innovative therapies by identifying and validating respiratory parameters.
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Review Meta Analysis
Endobronchial ultrasound-guided transbronchial needle biopsy for the diagnosis of intrathoracic lymph node metastases from extrathoracic malignancies: a meta-analysis and systematic review.
Intrathoracic lymph node metastases in patients with extrathoracic malignancies are a common clinical manifestation. Several studies evaluating intrathoracic lymph node metastases in patients with extrathoracic malignancy by using the endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) have been reported. The objective of this meta-analysis is to investigate the diagnostic value of EBUS-TBNA for diagnosing intrathoracic lymph node metastases in patients with extrathoracic malignancies. ⋯ The overall DOR was 179.77 (95% CI: 66.29-487.50). The area under the SROC curve and the diagnostic accuracy were 0.9247 and 0.8588, respectively. Evidence gathered from studies of moderate quality reveals a high degree of diagnostic accuracy of EBUS-TBNA for diagnosing intrathoracic lymph node metastases in patients with extrathoracic malignancies.
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The approach to management of malignant pleural effusions (MPE) has changed over the past few decades. The key goals of MPE management are to relieve patient symptoms using the least invasive means and in the most cost-effective manner. There is now a realization that patient-reported outcome measures should be the primary goal of MPE treatment, and this now is the focus in most clinical trials. ⋯ An unprecedented number of clinical trials are now underway to improve various aspects of MPE care. However, choosing an optimal intervention for MPE in an individual patient remains a challenge due to our limited understanding of the underlying pathophysiology of breathlessness in MPE and a lack of predictors of survival and pleurodesis outcome. This review provides an overview of common pleural interventional procedures used for MPE management, controversies and limitations of current practice, and areas of research most needed to improve practice in future.
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Substantial gains in understanding the pathophysiologic mechanisms underlying asthma have been made using preclinical mouse models. However, because asthma is a complex, heterogeneous syndrome that is rarely due to a single allergen and that often presents in the absence of atopy, few of the promising therapeutics that demonstrated effectiveness in mouse models have translated into new treatments for patients. This has resulted in an urgent need to characterize T helper (Th) 2-low, non-eosinophilic subsets of asthma, to study models that are resistant to conventional treatments such as corticosteroids and to develop therapies targeting patients with severe disease. ⋯ These models utilize more physiologically relevant sensitizing agents, exacerbating factors and allergens, as well as incorporate time points that better reflect the natural history and chronicity of clinical asthma. Importantly, some models better represent non-classical asthma endotypes that facilitate the study of non-Th2-driven pathology and resemble the complex nature of clinical asthma, including corticosteroid resistance. Placing mouse asthma models into the context of human asthma endotypes will afford a more relevant approach to the understanding of pathophysiological mechanisms of disease that will afford the development of new therapies for those asthmatics that remain difficult to treat.
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The last decade has seen a significant advance in understanding about early lung disease in cystic fibrosis (CF). As studies that have measured lung function in preschool years are conducted in association with surveillance of infection, inflammation and early structural changes, and emerging longitudinal data become available, a better insight into the very early onset and nature of such lung disease is emerging. ⋯ Lung function measurement in CF is potentially an important assessment tool and is used in routine clinical practice in several centres already. Results of studies from lung function tests that, on the basis of their underpinning physiology, are viewed as being best suited currently for the early detection of lung disease in CF are reviewed.