Abstract：
:Whilst substantial progress has been made in the treatment of cystic fibrosis, the disease still carries a significant burden in terms of symptoms, requirement for treatment and early mortality. The last decade has witnessed a new era in the development of small molecule drugs targeting the CFTR protein, which for the first time may provide a truly disease-modifying approach to treatment. This article reviews progress and highlights some of the current and future challenges in CFTR modulator therapies.
journal_name
Paediatr Respir Revjournal_title
Paediatric respiratory reviewsauthors
Harman K,Dobra R,Davies JCdoi
10.1016/j.prrv.2017.03.008subject
Has Abstractpub_date
2018-03-01 00:00:00pages
7-9eissn
1526-0542issn
1526-0550pii
S1526-0542(17)30031-3journal_volume
26pub_type
杂志文章,评审abstract：:NICE produced a guideline for the diagnosis and management of CF (NG78) in October 2017. This paper describes the process of producing the guideline and highlights some of the areas covered by it, including ideas for further research and tools that can be used by purchasers to help improve CF care. ...
journal_title：Paediatric respiratory reviews
pub_type： 杂志文章,评审
doi：10.1016/j.prrv.2019.02.006
更新日期：2019-08-01 00:00:00
abstract：:Pulmonary arterial (PA) hypertension in preterm infant is an important consequence of chronic lung disease of prematurity (CLD) arising mainly due to impaired alveolar development and dysregulated angiogenesis of the pulmonary circulation. Although PA pressure and resistance in these children normalise by school age, ...
journal_title：Paediatric respiratory reviews
pub_type： 杂志文章,评审
doi：10.1016/j.prrv.2012.08.003
更新日期：2013-12-01 00:00:00
abstract：:Despite being the most widely used and vital therapy in neonatology, optimal strategies for the use of oxygen in preterm infants remain controversial. Achieving the balance between attaining adequate tissue oxygenation and avoiding oxygen toxicity is challenging. There remains a paucity of clear evidence based guidanc...
journal_title：Paediatric respiratory reviews
pub_type： 杂志文章,评审
doi：10.1016/j.prrv.2012.12.003
更新日期：2014-06-01 00:00:00
abstract：:Parental smoking has an important impact on asthma and wheezing illnesses in infants and children. In utero exposure is associated with impaired lung growth and wheezing illnesses, particularly in preschool children. Exposure to environmental tobacco smoke is associated with increased wheezing illnesses and increased ...
journal_title：Paediatric respiratory reviews
pub_type： 杂志文章,评审
doi：10.1053/prrv.2001.0141
更新日期：2001-09-01 00:00:00
abstract：:The treatment of interstitial lung disease in children depends on the nature of the underlying pathology. In approximately 50% of cases a specific aetiology can be found such as: chronic viral infection, an auto-immune process, sarcoidosis or alveolar proteinosis. In the remainder, the process is idiopathic and the pa...
journal_title：Paediatric respiratory reviews
pub_type： 杂志文章,评审
doi：10.1016/j.prrv.2004.01.004
更新日期：2004-06-01 00:00:00
abstract：:The thorax consists of the rib cage and the respiratory muscles. It houses and protects the various intrathoracic organs such as the lungs, heart, vessels, esophagus, nerves etc. It also serves as the so-called "respiratory pump" that generates the movement of air into the lungs while it prevents their total collapse ...
journal_title：Paediatric respiratory reviews
pub_type： 杂志文章,评审
doi：10.1016/j.prrv.2013.12.003
更新日期：2014-09-01 00:00:00
abstract：:Asthma is a common disease in paediatrics and adults with a significant morbidity, mortality, and financial burden worldwide. Asthma is now recognized as a heterogeneous disease and emerging clinical and laboratory research has elucidated understanding of asthma's underlying immunology. The future of asthma is classif...
journal_title：Paediatric respiratory reviews
pub_type： 杂志文章,评审
doi：10.1016/j.prrv.2019.08.002
更新日期：2020-11-01 00:00:00
abstract：:Many factors have been implicated in SIDS cases including environmental influences such as sleeping arrangements and smoking. Most recently, cardiac abnormalities have been hypothesised to play a role in some cases, particularly the primary genetic arrhythmogenic disorders such as familial long QT syndrome (LQTS). Bot...
journal_title：Paediatric respiratory reviews
pub_type： 杂志文章,评审
doi：10.1016/j.prrv.2014.09.006
更新日期：2014-12-01 00:00:00
abstract：:Primary ciliary dyskinesia (PCD), also known as immotile-cilia syndrome, is a rare genetic disease that is inherited in an autosomal recessive manner. Several studies have explored certain aspects of PCD in the Arab world, yet much is still lacking in terms of identifying the different characteristics of this disease....
journal_title：Paediatric respiratory reviews
pub_type： 杂志文章,评审
doi：10.1016/j.prrv.2018.09.002
更新日期：2019-02-01 00:00:00
abstract：:Asthma is a heterogeneous disease and it is therefore unrealistic to expect that inhaled corticosteroids (ICS) would be appropriate first line preventer therapy for all children with asthma. There is good theoretical and clinical trial evidence demonstrating that leukotriene receptor antagonists (LTRAs) are more effec...
journal_title：Paediatric respiratory reviews
pub_type： 评论,杂志文章
doi：10.1016/j.prrv.2011.05.008
更新日期：2011-12-01 00:00:00
abstract：:Bronchiolitis is a common disease in infancy, mostly due to Respiratory Syncytial Virus and Rhinovirus. In addition to acute infection, viral bronchiolitis is responsible for sequelae including recurrent wheezing and asthma. The analysis of the viral characteristics and of the pathogenesis of the infection shows diffe...
journal_title：Paediatric respiratory reviews
pub_type： 杂志文章,评审
doi：10.1016/j.prrv.2016.11.006
更新日期：2017-09-01 00:00:00
abstract：:With increasing survival in cystic fibrosis (CF) there is an increasing need to deal with the desires of CF patients to become parents. In the context of 98% male infertility in CF, new techniques offer the prospect of successful parenthood. For females, successful pregnancy is possible but careful planning is require...
journal_title：Paediatric respiratory reviews
pub_type： 杂志文章,评审
doi：10.1016/s1526-0542(02)00184-7
更新日期：2002-09-01 00:00:00
abstract：:Conflict between the demands of the environment in which a child lives and the child's intrinsic sleep signature, produces stress-behaviour difficulties in the child. Distorted development, especially distorted brain development, often leads to distorted intrinsic sleep signatures. The sleep-behaviour outcome, arising...
journal_title：Paediatric respiratory reviews
pub_type： 杂志文章,评审
doi：10.1016/j.prrv.2013.08.001
更新日期：2014-03-01 00:00:00
abstract：:Cystic fibrosis related diabetes (CFRD) is a common complication of cystic fibrosis, caused by a fall in insulin secretion with age in individuals with pancreatic insufficiency. CFRD is associated with worse clinical status and increased mortality. Treatment of CFRD with insulin results in sustained improvements in lu...
journal_title：Paediatric respiratory reviews
pub_type： 杂志文章
doi：10.1016/j.prrv.2013.02.002
更新日期：2013-05-01 00:00:00
abstract：:During the first year of life and particularly the first 6 months autonomic control of the cardio-respiratory system is still undergoing maturation and infants are at risk of cardio-respiratory instability. These instabilities are most marked during sleep, which is important as infants spend the majority of each 24 ho...
journal_title：Paediatric respiratory reviews
pub_type： 杂志文章,评审
doi：10.1016/j.prrv.2013.02.012
更新日期：2014-06-01 00:00:00
abstract：:With the advent of improved antenatal imaging over the past 10 years, the diagnosis, assessment and management of congenital cystic lung abnormalities have changed. These were once considered the exclusive domain of the surgeon, who had the authority to operate on all congenital cystic lung abnormalities regardless of...
journal_title：Paediatric respiratory reviews
pub_type： 杂志文章,评审
doi：10.1016/j.prrv.2006.10.001
更新日期：2007-03-01 00:00:00
abstract：:The current global strategy for control of tuberculosis, Directly Observed Treatment, Short-course (DOTS) focuses, as a priority, on the management of patients with sputum smear positive tuberculosis. As tuberculosis in children is rarely sputum smear positive, this has led many to believe that children are not import...
journal_title：Paediatric respiratory reviews
pub_type： 杂志文章,评审
doi：10.1016/s1526-0542(04)90027-9
更新日期：2004-01-01 00:00:00
abstract：:Community-acquired pneumonia remains a common and serious illness, which affects children of all age groups. The spectrum of causative organisms is wide and it differs according to the age of the patients. With the advent of new and improved diagnostic techniques our understanding of the aetiology of the disease has b...
journal_title：Paediatric respiratory reviews
pub_type： 杂志文章,评审
doi：10.1016/s1526-0542(04)90037-1
更新日期：2004-01-01 00:00:00
abstract：:During sleep changes in central and peripheral neurological pathways and muscle tone result in unique vulnerabilities in the respiratory system. Abnormalities of the respiratory system that are not apparent in wakefulness can become evident during particular sleep states, making overnight polysomnography (PSG) a valua...
journal_title：Paediatric respiratory reviews
pub_type： 杂志文章,评审
doi：10.1016/j.prrv.2008.01.003
更新日期：2008-09-01 00:00:00
abstract：INTRODUCTION:Corticosteroids can be used prenatally to mature the fetal lungs and postnatally to treat or prevent chronic lung disease (CLD). Randomised controlled trials have been performed to evaluate the benefits and risks of perinatal corticosteroid therapy. METHODS:Systematic reviews of randomised controlled tria...
journal_title：Paediatric respiratory reviews
pub_type： 杂志文章,评审
doi：10.1016/s1526-0542(04)90057-7
更新日期：2004-01-01 00:00:00
abstract：:During airflight, cabins are pressurised to 8000ft (2438m) leading to an effective FiO2 of 0.15. This leads to a fall in oxygen saturation in all passengers, and especially those with underlying lung disease. The hypoxic challenge test using a body plethysmograph can predict a need for supplemental oxygen during airfl...
journal_title：Paediatric respiratory reviews
pub_type： 杂志文章,评审
doi：10.1016/j.prrv.2016.05.002
更新日期：2017-01-01 00:00:00
abstract：:Since January 2020, there has been a worldwide pandemic of COVID-19, caused by a novel coronavirus-severe acute respiratory syndrome coronavirus 2. The United States has been particularly affected, with the largest number of confirmed cases in a single country in the world. Healthcare systems for adults as well as chi...
journal_title：Paediatric respiratory reviews
pub_type： 杂志文章,评审
doi：10.1016/j.prrv.2020.06.002
更新日期：2020-09-01 00:00:00
abstract：:Newborn screening for cystic fibrosis remains controversial because there is still little agreement that prophylactic interventions provide substantial long-term benefits. In such situations, where there are some medical benefits and the costs are not prohibitive, it is important to consider the psychosocial implicati...
journal_title：Paediatric respiratory reviews
pub_type： 杂志文章,评审
doi：10.1016/s1526-0542(03)00086-1
更新日期：2003-12-01 00:00:00
abstract：:The obstructive sleep apnea syndrome is common and its prevalence is expected to increase with the current obesity epidemic. If left untreated, it is associated with important morbidity such as growth failure, neurocognitive impairment, systemic and pulmonary hypertension, and endothelial dysfunction. Recent research ...
journal_title：Paediatric respiratory reviews
pub_type： 杂志文章,评审
doi：10.1016/j.prrv.2012.05.006
更新日期：2013-09-01 00:00:00
abstract：:Pediatric pulmonary arterial hypertension (PAH) is an uncommon disease that can occur in neonates, infants, and children, and is associated with high morbidity and mortality. Despite advances in treatment strategies over the last two decades, the underlying structural and functional changes to the pulmonary arterial c...
journal_title：Paediatric respiratory reviews
pub_type： 杂志文章,评审
doi：10.1016/j.prrv.2015.05.003
更新日期：2015-09-01 00:00:00
abstract：:Primary ciliary dyskinesia (PCD) is a rare, autosomal recessive condition. The signs and symptoms are due to congenital abnormalities of ciliary structure and function, resulting in impaired mucociliary clearance. This affects the ciliated epithelium lining the nose, sinuses, Eustachian tube and airways. As a conseque...
journal_title：Paediatric respiratory reviews
pub_type： 杂志文章,评审
doi：10.1016/j.prrv.2008.10.001
更新日期：2009-06-01 00:00:00
abstract：:Significant improvement in the survival of patients with CF has been achieved in the last decades. The improved clinical status of the patients is mainly the result of a better understanding of the natural course of infection and inflammation in CF that has led to the implementation of strategies that increase the lif...
journal_title：Paediatric respiratory reviews
pub_type： 杂志文章,评审
doi：10.1016/j.prrv.2017.06.004
更新日期：2017-09-01 00:00:00
abstract：:Our aim was to obtain knowledge of how meteorological conditions affect community epidemics of respiratory syncytial virus (RSV) infection. We recorded year-round RSV activity in nine cities that differ markedly in geographic location and climate. We correlated local weather conditions with weekly or monthly RSV cases...
journal_title：Paediatric respiratory reviews
pub_type： 杂志文章,评审
doi：10.1016/S1526-0542(09)70004-1
更新日期：2009-06-01 00:00:00
abstract：:Radiological evaluation of the airway has been used as a screening tool and an adjunct to endoscopy for many years. It provides non-invasive data on the structure of the airway, often avoiding the risk of general anaesthesia. Standard radiographs provide some information on the intricate anatomy of the paediatric airw...
journal_title：Paediatric respiratory reviews
pub_type： 杂志文章
doi：10.1053/prrv.2000.0101
更新日期：2001-03-01 00:00:00
abstract：:Chronic cough has been variably defined as a cough lasting longer than 3, 4 or 8 weeks. Many post viral or pertussis like illnesses are associated with prolonged coughing that resolves over time. Management involves first trying to make a diagnosis and identify the presence of any underlying condition. Targeted treatm...
journal_title：Paediatric respiratory reviews
pub_type： 杂志文章,评审
doi：10.1016/j.prrv.2012.05.002
更新日期：2013-06-01 00:00:00
