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 the majority of patients with CF becoming eligible for treatment. CFTR modulators are currently considered contraindicated in patients with a solid organ transplant. This excludes many patients who may benefit from the multisystem effects of CFTR modulator treatment. In this review, we discuss issues regarding drug interactions, organ transplantation and CFTR modulation.
journal_name
Paediatr Respir Revjournal_title
Paediatric respiratory reviewsauthors
Mitchell RM,Jones AM,Barry PJdoi
10.1016/j.prrv.2018.04.003subject
Has Abstractpub_date
2018-06-01 00:00:00pages
6-8eissn
1526-0542issn
1526-0550pii
S1526-0542(18)30057-5journal_volume
27pub_type
杂志文章,评审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：:Breaking the news to parents that their child has cystic fibrosis [CF] is most frequently given in the first few weeks of the baby's life as a result of newborn screening. This is optimal to reduce morbidity but can have a significant impact on the parents' mental wellbeing and the parent-child relationship. Parent fe...
journal_title：Paediatric respiratory reviews
pub_type： 杂志文章,评审
doi：10.1016/j.prrv.2020.04.006
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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
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journal_title：Paediatric respiratory reviews
pub_type： 杂志文章,评审
doi：10.1016/j.prrv.2014.03.001
更新日期：2014-06-01 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(02)00265-8
更新日期：2002-12-01 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.2012.05.006
更新日期：2013-09-01 00:00:00
abstract：:The obstructive sleep apnoea syndrome (OSAS) is common in children and results in several complications when untreated. Considering that children are undergoing important brain development, recent research has focused on neurocognitive and behavioural (NCB) complications of OSAS. Studies have shown that treatment of p...
journal_title：Paediatric respiratory reviews
pub_type： 杂志文章,评审
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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：10.1016/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
doi：10.1016/j.prrv.2018.03.005
更新日期：2018-09-01 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.2006.04.198
更新日期：2006-01-01 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.2011.01.004
更新日期：2012-03-01 00:00:00
abstract：:Air pollution exposure has increased extensively in recent years and there is considerable evidence that exposure to particulate matter can lead to adverse respiratory outcomes. The health impacts of exposure to air pollution during the prenatal period is especially concerning as it can impair organogenesis and organ ...
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
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
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journal_title：Paediatric respiratory reviews
pub_type： 杂志文章,评审
doi：10.1016/j.prrv.2020.11.001
更新日期：2020-12-05 00:00:00
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journal_title：Paediatric respiratory reviews
pub_type： 杂志文章,评审
doi：
更新日期：2003-09-01 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.2007.07.002
更新日期：2007-09-01 00:00:00
abstract：:The Clinical and Functional Translation of CFTR (CFTR2) project presents a novel approach to clinical and functional annotation of mutations identified in disease-causing genes. Phenotype and genotype information on approximately 40,000 cystic fibrosis (CF) patients were collected from registries and large clinics. Th...
journal_title：Paediatric respiratory reviews
pub_type： 杂志文章
doi：10.1016/j.prrv.2013.01.006
更新日期：2013-05-01 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
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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.2009.12.002
更新日期：2010-12-01 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
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journal_title：Paediatric respiratory reviews
pub_type： 杂志文章,评审
doi：10.1016/j.prrv.2004.04.011
更新日期：2004-09-01 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.2006.03.009
更新日期：2006-06-01 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
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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
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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
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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：:There are hardly any controlled studies in paediatric tracheostomy care; instead, most established standards, procedures and details have been elaborated at the bedside by trial and error. Once the appropriate tube is chosen, tube care consists of tube change, fixation, management of secretions, humidification of insp...
journal_title：Paediatric respiratory reviews
pub_type： 杂志文章,评审
doi：10.1016/j.prrv.2006.06.003
更新日期：2006-09-01 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：:A systematic review was performed (i) to describe the reported overall rate of progression of CF lung disease quantified as FEV1%predicted decline with age, (ii) to summarise identified influencing risk factors and (iii) to review methods used to analyse CF lung disease progression data. A search of publications provi...
journal_title：Paediatric respiratory reviews
pub_type： 杂志文章,评审
doi：10.1016/j.prrv.2016.03.002
更新日期：2016-09-01 00:00:00
