Abstract：
:Despite evidence showing an improvement in nutritional outcomes following diagnosis by newborn screening (NBS) for cystic fibrosis (CF), the impact on pulmonary outcomes has been less clear. In this review the approaches to measurement of early lung function and knowledge gained from NBS CF cohorts will be described. Studies which have compared outcomes in those diagnosed by NBS to those diagnosed following symptomatic presentation will be presented. Compiling the evidence base used to evaluate the impact of NBS on pulmonary outcomes has been complicated by improvements in clinical management, infection control practices, as well as public health interventions (such as tobacco smoking bans in public places) that have evolved substantially over recent decades. Forced expiratory volumes have been used as the main outcome but it is important not to draw conclusions for 'early lung function' from tests such as spirometry alone, which lack sensitivity in early lung disease. There is, at present, insufficient evidence to draw firm conclusions about the effect of NBS on early lung function. In an era of highly effective treatments targeting the underlying molecular defect responsible for CF, future opportunities for early initiation of treatment may mean that the impact of NBS on early lung function may yet to be realised.
journal_name
Paediatr Respir Revjournal_title
Paediatric respiratory reviewsauthors
Davies Gdoi
10.1016/j.prrv.2020.08.005subject
Has Abstractpub_date
2020-08-20 00:00:00eissn
1526-0542issn
1526-0550pii
S1526-0542(20)30121-4pub_type
杂志文章,评审abstract：:Most causes of upper airway obstruction are rare in the neonatal period and during infancy. They may, however, cause major respiratory problems either initially or during the first few weeks of life. It is important to recognise these problems at an early stage so that appropriate measures to overcome airway obstructi...
journal_title：Paediatric respiratory reviews
pub_type： 杂志文章,评审
doi：10.1016/j.prrv.2003.10.001
更新日期：2004-03-01 00:00:00
abstract：:Advances in the elucidation of cystic fibrosis transmembrane regulator (CFTR) function have resulted in a greater understanding of the relationship between the CF gene defect and clinical disease. The clinical phenotype is influenced by the class of mutation and possibly by other modifier genes. CFTR regulates the vol...
journal_title：Paediatric respiratory reviews
pub_type： 杂志文章,评审
doi：10.1053/prrv.2000.0029
更新日期：2000-06-01 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：:Comprehensive newborn screening for cystic fibrosis has occurred for more than 25 years in some regions and the results of randomised controlled trials reporting the outcomes have been published. Testing protocols for CF have recently been reviewed and the sensitivity and specificity of these protocols are high. In sp...
journal_title：Paediatric respiratory reviews
pub_type： 杂志文章,评审
doi：10.1016/j.prrv.2008.09.004
更新日期：2008-12-01 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：:One of the most active areas of research in medicine today is stem cell biology. This review introduces the reader to the field of stem cell biology and its therapeutic potential. More importantly, the potential application of stem cell therapy in acute lung injury will be explored. ...
journal_title：Paediatric respiratory reviews
pub_type： 杂志文章,评审
doi：10.1016/j.prrv.2006.03.009
更新日期：2006-06-01 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：: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：: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
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journal_title：Paediatric respiratory reviews
pub_type： 杂志文章,评审
doi：10.1016/j.prrv.2009.10.004
更新日期：2010-03-01 00:00:00
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journal_title：Paediatric respiratory reviews
pub_type： 杂志文章,评审
doi：10.1016/j.prrv.2016.04.003
更新日期：2016-06-01 00:00:00
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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：: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
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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
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journal_title：Paediatric respiratory reviews
pub_type： 杂志文章,评审
doi：10.1016/j.prrv.2012.04.001
更新日期：2012-09-01 00:00:00
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journal_title：Paediatric respiratory reviews
pub_type： 杂志文章,评审
doi：10.1016/j.prrv.2016.05.004
更新日期：2016-09-01 00:00:00
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journal_title：Paediatric respiratory reviews
pub_type： 杂志文章,评审
doi：10.1053/prrv.2000.0059
更新日期：2000-09-01 00:00:00
abstract：:Cough is a common presenting symptom to paediatricians and paediatric respiratory physicians. The causes of chronic cough in childhood are significantly different from those in adults, and a different approach to diagnosis and management is needed. In most cases cause can be identified or a satisfactory label given. T...
journal_title：Paediatric respiratory reviews
pub_type： 杂志文章,评审
doi：10.1016/j.prrv.2005.11.008
更新日期：2006-03-01 00:00:00
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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
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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 major morbidity and mortality associated with cerebral palsy (CP) relates to respiratory compromise. This manifests through repeated pulmonary aspiration, airway colonization with pathogenic bacteria, the evolution of bronchiectasis and sleep disordered breathing. An accurate assessment involving a multidisciplina...
journal_title：Paediatric respiratory reviews
pub_type： 杂志文章,评审
doi：10.1016/j.prrv.2008.10.003
更新日期：2009-03-01 00:00:00
abstract：:Assessing chest wall motion is a basic and vital component in managing the child with respiratory problems, whether these are due to pathology in the lungs, airways, chest wall or muscles. Since the 1960s, clinical assessment has been supplemented with an ever-growing range of technological options for measuring chest...
journal_title：Paediatric respiratory reviews
pub_type： 杂志文章,评审
doi：10.1016/j.prrv.2014.10.006
更新日期：2015-01-01 00:00:00
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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：:Childhood respiratory diseases are accompanied by various forms of epithelial repair and remodelling. Respiratory stem cells are likely to be of great importance in these processes but their identification remains uncertain. By contrast, an enormous effort has been made to identify specific markers of stem cells of ot...
journal_title：Paediatric respiratory reviews
pub_type： 杂志文章,评审
doi：10.1053/prrv.2002.0180
更新日期：2002-03-01 00:00:00
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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：:CFTR modulators are a class of drugs which directly target the defective CFTR protein in cystic fibrosis (CF), improving its function with resultant clinical improvements. Currently these drugs are confined to people with a limited selection of genetic mutations. New modulators are in development which will lead to th...
journal_title：Paediatric respiratory reviews
pub_type： 杂志文章,评审
doi：10.1016/j.prrv.2018.04.003
更新日期：2018-06-01 00:00:00
abstract：:Increasing numbers of children and young adults are now receiving home ventilation. Whereas in some neuromuscular conditions the outcome and quality of life gains are clear cut; in others eg Spinal Muscular Atrophy Type I there are a few outcome studies with conflicting results and it is more difficult to balance the ...
journal_title：Paediatric respiratory reviews
pub_type： 杂志文章,评审
doi：10.1016/j.prrv.2005.06.003
更新日期：2005-09-01 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：: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：: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
