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 neuromuscular disorders with cardiac manifestations are the muscular dystrophies- Duchenne, Becker, limb-girdle and Emery Dreifuss. Friedreich Ataxia and myotonic dystrophy also have important cardiac involvement. The type and extent of cardiac manifestations are specific to the type of neuromuscular disorder. The most common cardiac findings include dilated or hypertrophic cardiomyopathy, atrioventricular conduction defects, atrial fibrillation and ventricular arrhythmias. Screening for cardiac involvement should be performed in all children with neuromuscular disorders that have the potential for cardiac involvement. This review discusses the cardiac findings associated with specific neuromuscular disorders and outlines the indications for evaluation and treatment.
journal_name
Paediatr Respir Revjournal_title
Paediatric respiratory reviewsauthors
Hsu DTdoi
10.1016/j.prrv.2009.10.004subject
Has Abstractpub_date
2010-03-01 00:00:00pages
35-8issue
1eissn
1526-0542issn
1526-0550pii
S1526-0542(09)00072-4journal_volume
11pub_type
杂志文章,评审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：: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：:Since preschool wheezing is the common expression of several heterogeneous disorders, identification of children at risk for persistent asthma is particularly challenging. To date, efforts to predict the outcome of preschool wheeze have mainly relied on predictive rules consisting of simple clinical and laboratory par...
journal_title：Paediatric respiratory reviews
pub_type： 杂志文章,评审
doi：10.1016/j.prrv.2012.08.004
更新日期：2013-03-01 00:00:00
abstract：:Increasing numbers of children and young adults are now receiving home ventilation. Whereas in some neuromuscular conditions the outcome and quality of life gains are clear cut; in others eg Spinal Muscular Atrophy Type I there are a few outcome studies with conflicting results and it is more difficult to balance the ...
journal_title：Paediatric respiratory reviews
pub_type： 杂志文章,评审
doi：10.1016/j.prrv.2005.06.003
更新日期：2005-09-01 00:00:00
abstract：:Pneumonia is highly prevalent in both developed and developing countries. In this review we list the main organisms affecting children with pneumonia and we propose a summary of the best possible diagnostic and therapeutic measures. ...
journal_title：Paediatric respiratory reviews
pub_type： 杂志文章,评审
doi：10.1016/j.prrv.2006.04.221
更新日期：2006-01-01 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：: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：: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：: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：:The major morbidity and mortality associated with cerebral palsy (CP) relates to respiratory compromise. This manifests through repeated pulmonary aspiration, airway colonization with pathogenic bacteria, the evolution of bronchiectasis and sleep disordered breathing. An accurate assessment involving a multidisciplina...
journal_title：Paediatric respiratory reviews
pub_type： 杂志文章,评审
doi：10.1016/j.prrv.2008.10.003
更新日期：2009-03-01 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：:Since experienced clinicians spend a substantial proportion of their time teaching and supervising trainees, a working knowledge of adult learning is of value. This knowledge will not only make you a more effective teacher, but will be of benefit to you when you are learning new information. There are a number of impo...
journal_title：Paediatric respiratory reviews
pub_type： 杂志文章,评审
doi：10.1016/j.prrv.2007.11.009
更新日期：2008-06-01 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
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：: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：: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：: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：: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：:The development and phenotypic expression of allergic airway disease depends on a complex interaction between genetic and several environmental factors, such as exposure to food, inhalant allergens and non-specific adjuvant factors (e.g. tobacco smoke, air pollution and infections). The first months of life seem to be...
journal_title：Paediatric respiratory reviews
pub_type： 杂志文章,评审
doi：10.1016/s1526-0542(03)00026-5
更新日期：2003-06-01 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：: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 obstructive sleep apnea syndrome is common and its prevalence is expected to increase with the current obesity epidemic. If left untreated, it is associated with important morbidity such as growth failure, neurocognitive impairment, systemic and pulmonary hypertension, and endothelial dysfunction. Recent research ...
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：: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：: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：: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：:Public awareness of the impact of air quality on health is increasing worldwide. Indoor and outdoor air pollutants impair children's growing lungs, and increase the risk of respiratory infections. In many cities, children face indoor air pollution from fuels used for cooking and heating, as well as outdoor pollution f...
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
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：:Childhood pulmonary TB (PTB) is under diagnosed, in part due to difficulties in obtaining microbiological confirmation. However, given the poor specificity of clinical diagnosis, microbiological confirmation and drug susceptibility testing is important in guiding appropriate therapy especially in the context of drug r...
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：: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：: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
