Abstract:
INTRODUCTION:Cystic fibrosis (CF) outcomes and survival have improved over the last century primarily due to advancements in antibiotics, nutritional, and pulmonary therapies. Reviewed here are the significant unmet needs that exist for individuals with CF. Areas covered: With the recent development of medications that address the underlying defect in the CF protein, there is hope that there will be continued improvement in CF outcomes. However, there remains a need to prevent or stop progression of CF-related complications, as the CF protein is important to several body systems. As end stage lung disease is the primary cause of mortality in CF, a need exists for advancements in pulmonary therapies to reduce time burden, identification of best practices for the treatment of pulmonary exacerbations, further development of anti-infective and anti-inflammatory therapies, and appropriately timed referral for lung transplantation at end-stage lung disease. Extra-pulmonary complications are increasingly recognized and better understanding of such problems as CF related liver disease is needed. Expert commentary: While CFTR modulators are available for the majority of CF patients, there remains a need for effective therapies to address infection, inflammation, irreversible lung disease, and extrapulmonary complications of CF.
journal_name
Expert Rev Respir Medjournal_title
Expert review of respiratory medicineauthors
West NE,Flume PAdoi
10.1080/17476348.2018.1483723subject
Has Abstractpub_date
2018-07-01 00:00:00pages
585-593issue
7eissn
1747-6348issn
1747-6356journal_volume
12pub_type
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journal_title:Expert review of respiratory medicine
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doi:10.1586/17476348.2016.1164603
更新日期:2016-01-01 00:00:00
abstract::Introduction: Neuromotor control of diaphragm muscle and the recovery of diaphragm activity following spinal cord injury have been narrowly focused on ventilation. By contrast, the understanding of neuromotor control for non-ventilatory expulsive/straining maneuvers (including coughing, defecation, and parturition) is...
journal_title:Expert review of respiratory medicine
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journal_title:Expert review of respiratory medicine
pub_type: 杂志文章,评审
doi:10.1586/ers.10.23
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journal_title:Expert review of respiratory medicine
pub_type: 杂志文章,评审
doi:10.1586/17476348.2015.996134
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abstract::Long-term oxygen therapy (LTOT) has been shown to reduce pulmonary hypertension and improve survival in patients with chronic obstructive pulmonary disease and resting hypoxemia (reduced arterial partial pressure of oxygen ≤55 mmHg). However, the benefit of its use for chronic pulmonary diseases other than chronic obs...
journal_title:Expert review of respiratory medicine
pub_type: 杂志文章,评审
doi:10.1586/ers.12.69
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journal_title:Expert review of respiratory medicine
pub_type: 杂志文章
doi:10.1080/17476348.2018.1424544
更新日期:2018-03-01 00:00:00
abstract:INTRODUCTION:Interstitial lung diseases (ILD) include a broad range of diffuse parenchymal lung disorders of known and unknown etiologies. Patients with ILD can experience acute exacerbations (AE) which are associated with extremely high morbidity and mortality. Little is known about the etiology of AEs, and whether in...
journal_title:Expert review of respiratory medicine
pub_type: 杂志文章,评审
doi:10.1080/17476348.2018.1446831
更新日期:2018-04-01 00:00:00
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journal_title:Expert review of respiratory medicine
pub_type: 杂志文章
doi:10.1080/17476348.2020.1724096
更新日期:2020-04-01 00:00:00
abstract:INTRODUCTION:Lung Clearance Index, measured using the multiple breath washout (MBW) technique, may be a useful test in infants with Cystic Fibrosis (CF). However, the requirement for specialised equipment and a number of important technical and methodological considerations relevant to testing in infants have complicat...
journal_title:Expert review of respiratory medicine
pub_type: 杂志文章,评审
doi:10.1080/17476348.2017.1269604
更新日期:2017-01-01 00:00:00
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journal_title:Expert review of respiratory medicine
pub_type: 杂志文章
doi:10.1586/ers.09.47
更新日期:2009-12-01 00:00:00
abstract::Introduction: Knowledge of the pathophysiology of cough has continued to advance over recent decades. Establishing anatomic-diagnostic protocols, based on the anatomy and distribution of vagus nerve pathways regulating the cough reflex, was the first breakthrough in modern clinical medicine for chronic cough. The unme...
journal_title:Expert review of respiratory medicine
pub_type: 杂志文章
doi:10.1080/17476348.2020.1713102
更新日期:2020-03-01 00:00:00
abstract:INTRODUCTION:In sarcoidosis, muscle involvement is common, but mostly asymptomatic. Currently, little is known about respiratory muscle and diaphragm involvement and function in patients with sarcoidosis. Reduced inspiratory muscle strength and/or a reduced diaphragm function may contribute to exertional dyspnea, fatig...
journal_title:Expert review of respiratory medicine
pub_type: 杂志文章,评审
doi:10.1080/17476348.2018.1480940
更新日期:2018-07-01 00:00:00
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journal_title:Expert review of respiratory medicine
pub_type: 杂志文章,评审
doi:10.1080/17476348.2018.1522254
更新日期:2018-11-01 00:00:00
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journal_title:Expert review of respiratory medicine
pub_type: 杂志文章,评审
doi:10.1586/ers.11.34
更新日期:2011-06-01 00:00:00
abstract:INTRODUCTION:Lymphangioleiomyomatosis (LAM) is a destructive lung disease affecting primarily women. LAM is caused by inactivating mutations in the tuberous sclerosis complex (TSC) genes, resulting in hyperactivation of mechanistic/mammalian target of rapamycin complex 1 (mTORC1). Over the past five years, there have b...
journal_title:Expert review of respiratory medicine
pub_type: 杂志文章,评审
doi:10.1080/17476348.2018.1409622
更新日期:2018-02-01 00:00:00
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journal_title:Expert review of respiratory medicine
pub_type: 杂志文章,评审
doi:10.1586/ers.10.85
更新日期:2011-02-01 00:00:00
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journal_title:Expert review of respiratory medicine
pub_type: 杂志文章,评审
doi:10.1080/17476348.2018.1481393
更新日期:2018-07-01 00:00:00
abstract::Introduction: The forced expiratory flow between 25% and 75% of forced vital capacity (FEF25-75) is a spirometry parameter that may be useful in many clinical settings. Values <65% of predicted have been defined as abnormal.Areas covered: The current report discusses the clinical value of FEF25-75 in different setting...
journal_title:Expert review of respiratory medicine
pub_type: 杂志文章,评审
doi:10.1080/17476348.2019.1674649
更新日期:2019-12-01 00:00:00
abstract:INTRODUCTION:Cystic fibrosis-related diabetes (CFRD) is the end-point of a spectrum of glucose abnormalities in cystic fibrosis that begins with early insulin deficiency and ultimately results in accelerated nutritional decline and loss of lung function. Current diagnostic and management regimens are unable to entirely...
journal_title:Expert review of respiratory medicine
pub_type: 杂志文章,评审
doi:10.1080/17476348.2016.1190646
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journal_title:Expert review of respiratory medicine
pub_type: 杂志文章,评审
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更新日期:2011-08-01 00:00:00
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journal_title:Expert review of respiratory medicine
pub_type: 杂志文章,评审
doi:10.1080/17476348.2018.1475234
更新日期:2018-06-01 00:00:00
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journal_title:Expert review of respiratory medicine
pub_type: 杂志文章
doi:10.1586/ers.09.2
更新日期:2009-04-01 00:00:00
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journal_title:Expert review of respiratory medicine
pub_type: 杂志文章
doi:10.1586/ERS.09.21
更新日期:2009-06-01 00:00:00
abstract:INTRODUCTION:N-acetyl-l-cysteine (NAC), a derivative of the naturally occurring amino acid l-cysteine, is a mucolytic agent that may also act as an antioxidant by providing cysteine intracellularly for increased production of glutathione. It is also used for the treatment of acetaminophen overdose. Areas covered: The r...
journal_title:Expert review of respiratory medicine
pub_type: 杂志文章,评审
doi:10.1080/17476348.2018.1495562
更新日期:2018-08-01 00:00:00
abstract::Objectives: Pleurodesis is an important management option to palliate breathlessness in patients with malignant pleural effusion (MPE). This systematic review aimed to examine available literature for studies investigating factors that predict pleurodesis outcome.Methods: The healthcare databases advanced search (HDAS...
journal_title:Expert review of respiratory medicine
pub_type: 杂志文章
doi:10.1080/17476348.2020.1746647
更新日期:2020-06-01 00:00:00
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journal_title:Expert review of respiratory medicine
pub_type: 杂志文章,评审
doi:10.1586/17476348.2016.1141053
更新日期:2016-01-01 00:00:00
abstract::During the last decade, there have been major advances in knowledge of the effects of oxygen therapy in patients with acute exacerbations of chronic obstructive pulmonary disease. This includes a randomised controlled trial of oxygen therapy in the pre-hospital setting, which showed that high concentration oxygen ther...
journal_title:Expert review of respiratory medicine
pub_type: 杂志文章,评审
doi:10.1586/17476348.2015.1016503
更新日期:2015-06-01 00:00:00
abstract::Introduction: Cystic fibrosis (CF) is a complex, multi-system, genetic disease affecting over 70,000 people worldwide. The underlying defect is a mutation in the CFTR gene. Dysfunctional CFTR protein results in abnormal anion movement across epithelial membranes in affected organs. There has been a paradigm shift in C...
journal_title:Expert review of respiratory medicine
pub_type: 杂志文章,评审
doi:10.1080/17476348.2020.1682998
更新日期:2020-01-01 00:00:00
abstract::Chronic obstructive pulmonary disease is an inflammatory disorder characterized by airflow limitation. Its cardinal symptom is dyspnea, which develops gradually with even low levels of exercise. Bronchodilators (BDs) are the most effective drugs for relieving dyspnea. Two main types of BD are currently available, β-mi...
journal_title:Expert review of respiratory medicine
pub_type: 杂志文章,评审
doi:10.1586/ers.13.18
更新日期:2013-04-01 00:00:00
abstract:BACKGROUND:Approximately 14% of UK hospital in-patients receive supplemental oxygen therapy, only 57% have valid prescriptions. Oxygen must be optimally prescribed to ensure maximal therapeutic response whilst minimizing adverse outcomes (including fatality). This study investigates prescription compliance. METHODS:Al...
journal_title:Expert review of respiratory medicine
pub_type: 杂志文章
doi:10.1080/17476348.2021.1826316
更新日期:2020-10-14 00:00:00