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 volume and composition of airways surface liquid, primarily by controlling chloride ion transport. However, CFTR also regulates other membrane channels and transports other molecules which may be important in mucocilary clearance and innate defence mechanisms. CFTR may also modulate the inflammatory response in respiratory epithelial cells and other inflammatory cells through a range of mechanisms. It is apparent that CFTR dysfunction results in a range of effects which may contribute to the clinical phenotype. These may contribute to the development of clinical disease at different stages of the natural history of cystic fibrosis. A greater understanding of the basic defect and its implications is likely to result in novel therapeutic approaches.
journal_name
Paediatr Respir Revjournal_title
Paediatric respiratory reviewsauthors
McAuley DF,Elborn JSdoi
10.1053/prrv.2000.0029keywords:
subject
Has Abstractpub_date
2000-06-01 00:00:00pages
93-100issue
2eissn
1526-0542issn
1526-0550pii
S1526-0542(00)90029-0journal_volume
1pub_type
杂志文章,评审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：:The term "mucoactive agent" refers to any medication used to improve the clearance of airway secretions. It is not synonymous with the word "mucolytic" as this strictly means a drug that decreases the viscosity of secretions. In many cases, decreased viscosity will adversely affect cough transport. For this reason man...
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
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：:It is reasonable to suggest that Telemedicine could help in the management of chronic diseases by giving patients more flexibility to remain at home with opportunities to forward electronic data to healthcare professionals, reduce hospital emergency attendances and reduce overall costs. The reality, particularly in cy...
journal_title：Paediatric respiratory reviews
pub_type： 杂志文章,评审
doi：10.1016/j.prrv.2017.03.005
更新日期：2018-03-01 00:00:00
abstract：AIM:To describe the respiratory and nutritional supportive care and hospitalisations required in the real-world scenario in children with SMA type 1 treated with nusinersen. METHODS:Single-centre observational cohort study of children with SMA1 commencing nusinersen from November 2016 to September 2018. Motor, respira...
journal_title：Paediatric respiratory reviews
pub_type： 杂志文章,评审
doi：10.1016/j.prrv.2020.09.002
更新日期：2020-09-22 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：: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：:Spinal muscular atrophy (SMA) is a relatively common, profoundly disabling and incurable disease that presents in early childhood with hypotonia, weakness and decreased movement. Without ventilatory support, premature death from respiratory insufficiency is universal in children with spinal muscular atrophy type 1 (SM...
journal_title：Paediatric respiratory reviews
pub_type： 评论,杂志文章,评审
doi：10.1016/j.prrv.2007.10.002
更新日期：2008-03-01 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
更新日期：2020-09-01 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：: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：:Obese adolescents, particularly those with extreme obesity (body mass index > or =40 kg/m(2)), are developing serious medical complications at an unexpectedly high rate. As non-operative approaches to weight loss have shown less than optimal results, paediatric patients are increasingly seeking bariatric surgical inte...
journal_title：Paediatric respiratory reviews
pub_type： 杂志文章,评审
doi：10.1016/j.prrv.2006.08.004
更新日期：2006-12-01 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：: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：: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：:Interpretation of the paediatric chest X-ray may appear intimidating at first but knowledge of a few basic rules and an understanding of how the radiographic appearance may be influenced by age and technique will help the clinician arrive at the correct diagnosis in many cases. A structured and logical approach to X-r...
journal_title：Paediatric respiratory reviews
pub_type： 杂志文章,评审
doi：10.1053/prrv.2000.0018
更新日期：2000-03-01 00:00:00
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. ...
journal_title：Paediatric respiratory reviews
pub_type： 杂志文章,评审
doi：10.1016/j.prrv.2020.08.005
更新日期：2020-08-20 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：: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：: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：: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：:Vocal cord dysfunction is characterised by paradoxical vocal cord adduction that occurs during inspiration, resulting in symptoms of dyspnoea, wheeze, chest or throat tightness and cough. Although the condition is well described in children and adults, confusion with asthma often triggers the use of an aggressive trea...
journal_title：Paediatric respiratory reviews
pub_type： 杂志文章,评审
doi：10.1016/j.prrv.2007.05.004
更新日期：2007-06-01 00:00:00
abstract：:Renal disease is relatively rare in cystic fibrosis even though the cystic fibrosis transmembrane regulator (CFTR) protein is expressed in abundance in the kidney. Aberrant CFTR expression probably explains the subtle abnormalities in renal concentrating and diluting ability described in cystic fibrosis and possibly t...
journal_title：Paediatric respiratory reviews
pub_type： 杂志文章
doi：
更新日期：2002-06-01 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：: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：:The term atelectasis describes a state of collapsed and non-aerated region of the lung parenchyma, which is otherwise normal. This pathological condition is usually associated with several pulmonary and chest disorders and represents a manifestation of the underlying disease, not a disease per se. Atelectasis may occu...
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：:Foreign body aspiration is a common accident in children and represents an important cause of morbidity and mortality. Diagnosis of this condition demands a high degree of suspicion since physical examination and basic radiology exams have low sensitivity. It is more frequent in children younger than 3 years of age, p...
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
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：:Bronchopulmonary dysplasia (BPD) is a common complication of preterm birth. Chest imaging is important in making the diagnosis of BPD, and in assessing for complications. More recently computerised tomography (CT) scanning has provided insights in to the pathophysiology of BPD. Studies in infants, young and school age...
journal_title：Paediatric respiratory reviews
pub_type： 杂志文章,评审
doi：10.1016/j.prrv.2010.05.005
更新日期：2010-09-01 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
