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_name
Paediatr Respir Revjournal_title
Paediatric respiratory reviewsauthors
Walshaw MJdoi
10.1016/j.prrv.2019.02.006subject
Has Abstractpub_date
2019-08-01 00:00:00pages
12-14eissn
1526-0542issn
1526-0550pii
S1526-0542(19)30020-Xjournal_volume
31pub_type
杂志文章,评审abstract：:The anatomical and functional development of the lung appears especially vulnerable to a whole range of insults during gestation and the first few years of life. A significant proportion of adult lung disease originates in utero or early infancy. Most publications on this topic are descriptive retrospective studies. A...
journal_title：Paediatric respiratory reviews
pub_type： 杂志文章,评审
doi：10.1016/s1526-0542(02)00311-1
更新日期：2003-03-01 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：:Sudden unexpected death is one of the most frequent ways of dying in the first year of life after the neonatal period. It is however, much less frequent after the first birthday. Investigations into the cause of death are very important, for a significant proportion of these sudden deaths can be explained only after a...
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：: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：: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：: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：: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：: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：: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：: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：:The range of techniques available to assess respiratory muscles weakness has greatly expanded over recent years. Respiratory muscle tests can be divided into non-invasive tests, such as lung function parameters, maximal static pressures and sniff nasal pressure and invasive tests, such as transdiaphragmatic pressures ...
journal_title：Paediatric respiratory reviews
pub_type： 杂志文章,评审
doi：10.1016/s1526-0542(03)00051-4
更新日期：2003-09-01 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：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：:Individually, congenital abnormalities of the lung are rare but collectively they form an important group of conditions. The range of malformations is broad and the clinical impact is very variable, depending on the degree of malformation. This article groups these conditions under their traditional headings and consi...
journal_title：Paediatric respiratory reviews
pub_type： 杂志文章,评审
doi：10.1053/prrv.2000.0072
更新日期：2000-12-01 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
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：: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：: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
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：:The interrupter technique (Rint) is a quick, easy and effort-independent way to obtain indirect measurements of airways resistance in the preschool child. Results may be obtained using a portable Rint machine or a whole-body plethysmograph. Normative data are available and recent studies have improved standardisation ...
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：:There are many important respiratory manifestations of endocrine and metabolic diseases in children. Acute and chronic pulmonary infections are the most common respiratory abnormalities in patients with diabetes mellitus, although cardiogenic and non-cardiogenic pulmonary oedema are also possible. Pseudohypoaldosteron...
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：:Bronchiolitis is a common viral disease that significantly affects infants less than 12 months of age. The purpose of this review is to present a review of the current knowledge of the uses of respiratory support in the management of infants with bronchiolitis presenting to hospital. We electronically searched MEDLINE...
journal_title：Paediatric respiratory reviews
pub_type： 杂志文章,评审
doi：10.1016/j.prrv.2018.10.001
更新日期：2019-04-01 00:00:00
abstract：:Control of the cardiovascular and respiratory systems undergoes rapid maturation during infancy. Sleep is at a lifetime maximum during this period and has a marked influence on cardiorespiratory function. The mechanisms leading to sudden infant death syndrome (SIDS) may include a failure in the neural integration of t...
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
abstract：:Cardiac abnormalities occur in association with many of the neuromuscular disorders that present in childhood. Genetic defects involving the cytoskeleton, nuclear membrane, and mitochondrial function have all been described in patients with skeletal myopathy and cardiac involvement. The most common classes of neuromus...
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
abstract：:Social Robots are used in different contexts and, in healthcare, they are better known as Socially Assistive Robots. In the context of asthma, the use of Socially Assistive Robots has the potential to increase motivation and engagement to treatment. Other positive roles proposed for Socially Assistive Robots are to pr...
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
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：: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：: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
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：: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
