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 implications of screening. This paper reviews the evidence on the psychosocial issues raised by newborn screening for cystic fibrosis, in particular the issues of parental attitudes to screening, the evidence from families with an affected infant, the evidence from families with a carrier infant and the lessons for service delivery.
journal_name
Paediatr Respir Revjournal_title
Paediatric respiratory reviewsauthors
Parsons EP,Bradley DMdoi
10.1016/s1526-0542(03)00086-1keywords:
subject
Has Abstractpub_date
2003-12-01 00:00:00pages
285-92issue
4eissn
1526-0542issn
1526-0550pii
S1526054203000861journal_volume
4pub_type
杂志文章,评审abstract::The lung is continually at risk of exposure to noxious environmental agents and respiratory pathogens. An elaborate series of defence mechanisms have been developed to protect the airways from these insults. The lower respiratory tract is protected by local mucociliary mechanisms that involve the integration of the ci...
journal_title:Paediatric respiratory reviews
pub_type: 杂志文章,评审
doi:10.1053/prrv.2000.0009
更新日期:2000-03-01 00:00:00
abstract::Despite fundamental advances in the research on respiratory syncytial virus (RSV) since its initial identification almost 60 years ago, recurring failures in developing vaccines and pharmacologic strategies effective in controlling the infection have allowed RSV to become a leading cause of global infant morbidity and...
journal_title:Paediatric respiratory reviews
pub_type: 杂志文章,评审
doi:10.1016/j.prrv.2015.05.005
更新日期:2015-09-01 00:00:00
abstract::There are multiple aetiologies for childhood bronchiectasis unrelated to cystic fibrosis. Some of these aetiologies, such as those predisposing to recurrent lung infections, e.g. immunodeficiencies, require treatment of the underlying condition and disease-specific pulmonary pathogens within the airway. Regardless of ...
journal_title:Paediatric respiratory reviews
pub_type: 杂志文章
doi:10.1016/j.prrv.2010.10.012
更新日期:2011-06-01 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::Cystic fibrosis (CF) respiratory infection is characterised by the presence of typical human bacterial pathogens such as Pseudomonas aeruginosa, Haemophilus influenzae and Staphylococcus aureus. Less typical pathogens such as Burkholderia, Stenotrophomonas, Achromobacter, Pandorea and Ralstonia have emerged as problem...
journal_title:Paediatric respiratory reviews
pub_type: 杂志文章,评审
doi:10.1016/j.prrv.2014.04.006
更新日期:2014-06-01 00:00:00
abstract::Asthma is one of the commonest chronic conditions in children and can occasionally be fatal. Little has changed regarding the risk factors for children dying from asthma in the last 30 years. The majority of deaths from asthma occur in children from socio-economically disadvantaged backgrounds. These should be prevent...
journal_title:Paediatric respiratory reviews
pub_type: 杂志文章,评审
doi:10.1016/j.prrv.2018.02.001
更新日期:2018-06-01 00:00:00
abstract::Primary Ciliary Dyskinesia (PCD) is an autosomal recessive genetic condition affecting the function of motile cilia. The upper respiratory tract is lined with ciliated epithelium and hence a hallmark of PCD is the development, from the neonatal period onwards, of persisting secretion retention and suppurative infectio...
journal_title:Paediatric respiratory reviews
pub_type: 杂志文章,评审
doi:10.1016/j.prrv.2015.09.006
更新日期:2016-03-01 00:00:00
abstract::Dysfunctional breathing, hyperventilation and vocal cord dysfunction are frequently seen in children and adults. The prevalence is unknown. There are no standardized diagnostic criteria, and for now, effective exclusion of organic disease leaves the diagnosis of dysfunctional breathing. Therapy is mainly focussed on e...
journal_title:Paediatric respiratory reviews
pub_type: 杂志文章,评审
doi:10.1016/j.prrv.2010.09.003
更新日期:2011-03-01 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.2016.08.008
更新日期:2017-01-01 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.2005.09.011
更新日期:2005-12-01 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::Accumulating data suggest that prenatal compromises leading to intrauterine growth restriction (IUGR) increase the risk for respiratory deficiencies after birth. In this respect, a growing body of epidemiological evidence in infants, children and adults indicates that small for gestational (SGA) birth weight can adver...
journal_title:Paediatric respiratory reviews
pub_type: 杂志文章,评审
doi:10.1016/j.prrv.2012.10.001
更新日期:2013-12-01 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.2010.09.008
更新日期:2011-03-01 00:00:00
abstract::Sickle cell disease (SCD), caused by a mutation in the β-globin gene HBB, is widely distributed in malaria endemic regions. Cardiopulmonary complications are major causes of morbidity and mortality. Hemoglobin SS (Hb SS) represents a large proportion of SCD in the Americas, United Kingdom, and certain regions of Afric...
journal_title:Paediatric respiratory reviews
pub_type: 杂志文章,评审
doi:10.1016/j.prrv.2013.11.003
更新日期:2014-03-01 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::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::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
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::Significant technological advancements have been achieved in radiology with the introduction of a branch speciality known as interventional radiology. Radiology has moved into the therapeutic arena, performing minimally invasive diagnostic and therapeutic procedures. The interventional radiologist can treat certain va...
journal_title:Paediatric respiratory reviews
pub_type: 杂志文章,评审
doi:10.1053/prrv.2000.0121
更新日期:2001-06-01 00:00:00
abstract::Regular exercise and habitual physical activity are important for patients with cystic fibrosis (CF). Research has demonstrated the benefits of aerobic, anaerobic, and strength exercise training programs for health and quality of life, however, the CF patient is faced with unique barriers and challenges to participati...
journal_title:Paediatric respiratory reviews
pub_type: 杂志文章,评审
doi:10.1016/j.prrv.2009.04.001
更新日期:2009-09-01 00:00:00
abstract::Severe acute respiratory syndrome (SARS) is a newly described respiratory infection with pandemic potential. The causative agent is a new strain of coronavirus most likely originating from wild animals. This disease first emerged in November 2002 in Guangdong Province, China. Early in the outbreak the infection had be...
journal_title:Paediatric respiratory reviews
pub_type: 杂志文章,评审
doi:10.1016/j.prrv.2004.07.011
更新日期:2004-12-01 00:00:00
abstract::Respiratory viruses cause a number of clinical 'syndromes' in the intensive care unit with different viruses being able to produce similar clinical pictures. Our main presenting problems are upper airway (e.g. croup and tracheitis), lower airway with intrapulmonary shunt (e.g. bronchitis and pneumonia), lower airway w...
journal_title:Paediatric respiratory reviews
pub_type: 杂志文章,评审
doi:
更新日期:2003-09-01 00:00:00
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...
journal_title:Paediatric respiratory reviews
pub_type: 杂志文章,评审
doi:10.1016/j.prrv.2017.03.008
更新日期:2018-03-01 00:00:00
abstract::Chylothorax is the accumulation of chyle in the pleural space, as a result of damage to the thoracic duct. Chyle is milky fluid enriched with fat secreted from the intestinal cells and lymphatic fluid. Chylothorax in children, is most commonly seen as a complication of cardiothoracic surgery but may occur in newborns ...
journal_title:Paediatric respiratory reviews
pub_type: 杂志文章,评审
doi:10.1016/j.prrv.2009.06.008
更新日期:2009-12-01 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::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::The burden of asthma among children is high in Australia compared with many other countries. Recent data show that 14-16% of children report a diagnosis of asthma that remains a problem. Boys, children under the age of 5 years and urban indigenous children experience a greater burden of asthma than other children. Mor...
journal_title:Paediatric respiratory reviews
pub_type: 杂志文章,评审
doi:10.1016/j.prrv.2004.11.004
更新日期:2005-03-01 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::Inhaled nitric oxide (iNO) is a selective pulmonary vasodilator. In randomised trials, iNO increased gas exchange modestly and had no effect on the outcome (survival, chronic lung disease, CLD) in very low birth-weight (VLBW) infants. NO is an important component of the innate immune system and a lipid- soluble free r...
journal_title:Paediatric respiratory reviews
pub_type: 杂志文章,评审
doi:10.1016/s1526-0542(04)90047-4
更新日期:2004-01-01 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