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 the increased incidence of nephrocalcinosis. It also provides a hypothesis for the important differences in renal handling of some drugs. As the prognosis improves for patients with cystic fibrosis, secondary renal complications, for example glomerulonephritis and amyloidosis, are likely to become more prevalent. There are also a number of potentially nephrotoxic drugs used in the management of cystic fibrosis.
journal_name
Paediatr Respir Revjournal_title
Paediatric respiratory reviewsauthors
Stephens SE,Rigden SPkeywords:
subject
Has Abstractpub_date
2002-06-01 00:00:00pages
135-8issue
2eissn
1526-0542issn
1526-0550pii
S1526055002000124journal_volume
3pub_type
杂志文章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::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
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::This paper describes a patient whose decline over two years was precipitous, from an active independent life with lung function (FEV1) above 50% to requiring transplantation. The main pathogen on sputum culture throughout that period was Scediosporium apiosperum. The epidemiology pathogenicity and treatment of this fu...
journal_title:Paediatric respiratory reviews
pub_type: 杂志文章
doi:10.1016/j.prrv.2013.02.004
更新日期:2013-05-01 00:00:00
abstract::Since the discovery of the CF gene over a decade ago, several groups worldwide have explored the potential of gene therapy, the insertion of a normal copy of the gene into the respiratory epithelium. Both viral and synthetic gene transfer agents have been designed to this end, although problems with repeat application...
journal_title:Paediatric respiratory reviews
pub_type: 杂志文章,评审
doi:10.1016/j.prrv.2006.04.214
更新日期:2006-01-01 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::Despite being the most widely used and vital therapy in neonatology, optimal strategies for the use of oxygen in preterm infants remain controversial. Achieving the balance between attaining adequate tissue oxygenation and avoiding oxygen toxicity is challenging. There remains a paucity of clear evidence based guidanc...
journal_title:Paediatric respiratory reviews
pub_type: 杂志文章,评审
doi:10.1016/j.prrv.2012.12.003
更新日期:2014-06-01 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::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::Bacterial 'colonisation' of the nasopharynx by potential bacterial pathogens is frequent in early childhood and is frequent as part of a dynamic process in which the microbiota of the oral and nasopharynx are established. New understanding recognizes this process is evolving and that competition and likely regulation ...
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
abstract::During the first year of life and particularly the first 6 months autonomic control of the cardio-respiratory system is still undergoing maturation and infants are at risk of cardio-respiratory instability. These instabilities are most marked during sleep, which is important as infants spend the majority of each 24 ho...
journal_title:Paediatric respiratory reviews
pub_type: 杂志文章,评审
doi:10.1016/j.prrv.2013.02.012
更新日期:2014-06-01 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
doi:10.1016/j.prrv.2016.05.004
更新日期:2016-09-01 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::Non-invasive ventilation (NIV) provides significant advantages to the paediatrician who cares for children with chronic ventilatory insufficiency and who would like to manage these children in the home environment. There is a growing experience in the use of NIV in the management of children with upper airway obstruct...
journal_title:Paediatric respiratory reviews
pub_type: 杂志文章,评审
doi:10.1053/prrv.2000.0035
更新日期:2000-06-01 00:00:00
abstract::With increasing survival in cystic fibrosis (CF) there is an increasing need to deal with the desires of CF patients to become parents. In the context of 98% male infertility in CF, new techniques offer the prospect of successful parenthood. For females, successful pregnancy is possible but careful planning is require...
journal_title:Paediatric respiratory reviews
pub_type: 杂志文章,评审
doi:10.1016/s1526-0542(02)00184-7
更新日期:2002-09-01 00:00:00
abstract::Allergic bronchopulmonary aspergillosis (ABPA) is a severe complication in children, adolescents and adults with cystic fibrosis (CF), the prevalence of which ranges from 6-25%. The disease is the result of the colonisation of the respiratory tract by fungi of the genus Aspergillus, commonly Aspergillus fumigatus, and...
journal_title:Paediatric respiratory reviews
pub_type: 杂志文章,评审
doi:10.1016/j.prrv.2005.09.003
更新日期:2006-03-01 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::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::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::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
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::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::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::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::There have been major advances in the surgery for oesophageal atresia (OA) and tracheo-oesophageal fistula(TOF) with survival now exceeding 90%. The standard open approach to OA and distal TOF has been well described and essentially unchanged for the last 60 years. Improved survival in recent decades is most attributa...
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
abstract::This paper is an attempt to convey in a lucid way how to go about writing a scientific article for publication in an appropriate journal. Topics covered are: a) reasons to write a paper b) types of papers c) asking a question and formulating an hypothesis d) the complex series of steps necessary before you begin your ...
journal_title:Paediatric respiratory reviews
pub_type: 杂志文章
doi:10.1016/j.prrv.2011.02.004
更新日期:2012-06-01 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::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::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::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