Paediatric Respiratory Reviews (Paediatr Respir Rev)

Publications in this journal

• Article: Pseudomembranous Colitis in Cystic Fibrosis.

  Prasad Nagakumar
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  ABSTRACT: Cystic fibrosis (CF) patients may require frequent courses of antibiotics and repeated hospital admissions. Although children with CF have high carriage rate for C.difficile, they rarely develop colitis. Pseudomembranous colitis is more common in adult post lung transplant CF patients. Although rare, paseudomembranous colitis should be considered in CF patients presenting with abdominal symptoms even in the absence of diarrhoea.
  Paediatric Respiratory Reviews 04/2013;
• Article: Acute respiratory failure in children.

  Jürg Hammer
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  ABSTRACT: Acute respiratory failure is the most common medical emergency in children. One aim of this review is to discuss the physiologic peculiarities that explain the increased vulnerability of infants and children to any pathology affecting the respiratory tract. The other aim is to highlight the importance of history taking and correct physical examination for early recognition of an impending catastrophic progression of respiratory failure. Under most circumstances, correct physical examination alone allows one to pinpoint the cause to a particular part of the respiratory system and to make the appropriate decisions for a proactive and life-saving management of the critically ill child.
  Paediatric Respiratory Reviews 04/2013;
• Article: Management of acute severe upper airway obstruction in children.

  Andreas Pfleger, Ernst Eber
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  ABSTRACT: There are many causes of acute severe upper airway obstruction (UAO) in children. The timing of symptom onset and the presence of fever will help to distinguish infectious from non-infectious conditions. Signs and symptoms from congenital malformations often present at birth but may also develop over time. The most common cause of UAO in children is croup. Choking on a foreign body also occurs relatively frequently. Evaluation of the child with UAO starts with a detailed history followed by a thorough physical examination, including an assessment of severity. Severe airway obstruction will result in respiratory failure. This situation requires an immediate response. A child with partial airway obstruction may initially have an adequate airway. However, this situation can deteriorate rapidly. Therefore, providing supportive care and mobilizing resources for definitive airway management may be the most appropriate interventions.
  Paediatric Respiratory Reviews 04/2013;
• Article: Management of Chest Trauma in Children.

  Juan A Tovar, Juan J Vazquez
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  ABSTRACT: Chest trauma in children is caused by high-energy blows, due in general to traffic accidents, that involve several other body regions. They occur mainly in the first decade of life and can be penetrating but are more often non-penetrating. Rib fractures and lung contusions, sometimes associated with pneumothorax or haemothorax, are the more usual injuries, but tracheobronchial rupture, cardiac, oesophageal or diaphragmatic injuries may also occur. These injuries are treated with supportive respiratory and haemodynamic measures, drainage of air or blood from the pleural space and, at times, surgical repair of the injured organ(s). Ruptures of the airway may be difficult to treat and occasionally require suture, anastomosis or resection. Oesophageal injuries can be treated conservatively with antibiotics, drainage and parenteral nutrition. Diaphragmatic tears should be repaired operatively. Overall mortality ranges from 6 to 20%. Mortality is high but this is mainly due to the associated presence of extra-thoracic trauma, and particularly to head injuries.
  Paediatric Respiratory Reviews 04/2013;
• Article: Management of status asthmaticus in children.

  Muriel Koninckx, Corinne Buysse, Matthijs de Hoog
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  ABSTRACT: Recent literature on paediatric status asthmaticus (PSA) confirms an increasing percentage of admissions to paediatric intensive care units. PSA is a medical emergency that can be fatal and needs careful and prompt intervention. The severity of PSA is mainly determined by clinical judgement of signs and symptoms. Peak flow measurements and serial lung function measurements are not reliable in PSA. Validated clinically useful instruments are lacking. The three main factors that are involved in the pathophysiology of PSA, bronchoconstriction, mucus plugging and airway inflammation need to be addressed to optimise treatment. Initial therapies include supplementation of oxygen, repetitive administration of rapid acting β2-agonists, inhaled anticholinergics in combination with systemic glucocorticosteroids and intravenous magnesium sulphate. Additional treatment modalities may include methylxanthines, DNase, ketamine, sodium bicarbonate, heliox, epinephrine, non-invasive respiratory support, mechanical ventilation and inhalational anaesthetics.
  Paediatric Respiratory Reviews 04/2013;
• Article: What is the best airway clearance technique in cystic fibrosis?

  Eleanor Main
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  ABSTRACT: The global development of airway clearance techniques (ACTs) for cystic fibrosis (CF) and corresponding research spans over four decades. Five Cochrane reviews synthesising the evidence from a plethora of early short and medium term studies have not uncovered any superior method. Four recent long term RCT studies exposed fundamental shortcomings in the standard RCT trial design and the insensitivity of FEV1 in physiotherapy studies. Strong patient preference, lack of blinding and the requirement for effortful and demanding participation over long intervals will continue to derail efforts to find the best ACT for CF, unless they are addressed in future clinical trials.
  Paediatric Respiratory Reviews 03/2013;
• Article: Respiratory emergencies in children.

  Ernst Eber
  Paediatric Respiratory Reviews 03/2013;
• Article: Asthma medication delivery: Mists and myths.

  Bart L Rottier, Bruce K Rubin
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  ABSTRACT: Asthma is usually treated with inhaled corticosteroids (ICS) and bronchodilators generated from pressurized metered dose inhalers (pMDI), dry powder inhalers (DPI), or nebulizers. The target areas for ICS and beta 2-agonists in the treatment of asthma are explained. Drug deposition not only depends on particle size, but also on inhalation manoeuvre. Myths regarding inhalation treatments lead to less than optimal use of these delivery systems. We discuss the origin of many of these myths and provide the background and evidence for rejecting them.
  Paediatric Respiratory Reviews 03/2013;
• Article: Respiratory difficulties and breathing disorders in achondroplasia.

  S Afsharpaiman, A Saburi, Karen A Waters
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  ABSTRACT: Respiratory difficulties and breathing disorders in achondroplasia are thought to underlie the increased risk for sudden infant death and neuropsychological deficits seen in this condition. This review evaluates literature regarding respiratory dysfunctions and their sequelae in patients with achondroplasia. The limited number of prospective studies of respiratory disease in achondroplasia means that observational studies and case series provide a large proportion of the data regarding the spectrum of respiratory diseases in achondroplasia and their treatments. Amongst clinical respiratory problems described, snoring is the commonest observed abnormality, but the reported incidence of obstructive sleep apnoea (OSA) shows wide variance (10% to 75%). Reported treatments of OSA include adenotonsillectomy, the use of CPAP, and surgical improvement of the airway, including mid-face advancement. Otolaryngologic manifestations are also common. Respiratory failure due to small thoracic volumes is reported, but uncommon. Mortality rate at all ages was 2.27 (CI: 1.7-3.0) with age-specific mortality increased at all ages. Sudden death was most common in infants and children. Cardiovascular events are the main cause of mortality in adults. Despite earlier recognition and treatment of respiratory complications of achondroplasia, increased mortality rates and other complications remain high. Future and ongoing evaluation of the prevalence and impact of respiratory disorders, particularly OSA, in achondroplasia is recommended.
  Paediatric Respiratory Reviews 03/2013;
• Article: Cardio-respiratory control during sleep in infancy.

  Rosemary S C Horne
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  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 24hours in sleep. Sleep state has a marked effect on the cardio-respiratory system with instabilities being more common in active sleep compared to quiet sleep. Responses to hypoxia are also immature during infancy and may make young infants more vulnerable to cardio-respiratory instability. It has been proposed that an inability to respond appropriately to a life threatening event underpins the Sudden Infant Death Syndrome (SIDS). The major risk factors for SIDS, prone sleeping and maternal smoking, both impair cardio-respiratory control in normal healthy term infants.
  Paediatric Respiratory Reviews 03/2013;
• Article: Biomarkers in Bronchopulmonary Dysplasia.

  Anita Bhandari, Vineet Bhandari
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  ABSTRACT: Bronchopulmonary dysplasia (BPD) is a complex disorder secondary to gene-environment interactions, and is the commonest chronic lung disease in infancy. There is no specific or effective treatment available to date for BPD. Since the aetiopathogenesis of BPD is multifactorial, involving diverse molecular signaling pathways, a variety of biomarkers detected in biological fluids have been proposed for early identification of infants predisposed to BPD. This review will be restricted to biomarker studies in human infants, conducted mostly in the last decade. The majority of the studies have been conducted using blood, urine or tracheal aspirate samples. Despite the multitude of biomarkers proposed, most studies have been conducted in small numbers of infants, with few being replicated by independent investigators. Confirmatory studies with adequate sample sizes and assessment of the role of putative biomarkers in the aetiology of BPD in developmentally appropriate animal models and human lungs with BPD will enhance the potential for therapeutic interventions. Genomic and proteomic approaches have the greatest potential to significantly advance the field of biomarkers in BPD.
  Paediatric Respiratory Reviews 03/2013;
• Article: Pulmonary Functional Magnetic Resonance Imaging for Paediatric Lung Disease.

  Miranda Kirby, Harvey O Coxson, Grace Parraga
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  ABSTRACT: A better understanding of the anatomic structure and physiological function of the lung is fundamental to understanding the pathogenesis of pulmonary disease and how to design and deliver better treatments and measure response to intervention. Magnetic resonance imaging (MRI) with the hyperpolarised noble gases helium-3 ((3)He) and xenon-129 ((129)Xe) provides both structural and functional pulmonary measurements, and because it does not require the use of x-rays or other ionising radiation, offers the potential for intensive serial and longitudinal studies in paediatric patients. These facts are particularly important in the evaluation of chronic lung diseases such as asthma and cystic fibrosis- both of which can be considered paediatric respiratory diseases with unmet therapy needs. This review discusses MRI-based imaging methods with a focus on hyperpolarised gas MRI. We also discuss the strengths and limitations as well as the future work required for clinical translation towards paediatric respiratory disease.
  Paediatric Respiratory Reviews 03/2013;
• Article: Diabetes in Cystic Fibrosis.

  Nicola Bridges
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  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 lung function and nutrition. While clinical experience with insulin treatment in CF has increased, the selection of who to treat and glycaemic targets remain unclear.
  Paediatric Respiratory Reviews 03/2013;
• Article: The destructive combination of Scediosporium apiosperum lung disease and exuberant inflammation in cystic fibrosis.

  Georgina K Russell, Radha Gadhok, Nicholas J Simmonds
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  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 fungal pathogen are discussed.
  Paediatric Respiratory Reviews 03/2013;
• Article: Tolerance & resistance to β2-agonist bronchodilators.

  Robert P Yim, Anastassios C Koumbourlis
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  ABSTRACT: For the past half-century, β2-agonists have been the mainstay of treatment of the bronchoconstriction associated with asthma. Although their usefulness in reversing acute bronchospasm remains undiminished, there is increasing evidence that chronic use may lead to development of tolerance and thus, potentially increasing morbidity and even mortality. In addition, genetic studies have shown that certain individuals carrying specific mutations may be prone to developing resistance to β2-agonists regardless of the duration of treatment. This article reviews the current evidence regarding the underlying mechanisms that cause or contribute to the development of the resistance, as well as the strategies for the evaluation and management of patients who are at risk for or have developed tolerance to β2-agonists.
  Paediatric Respiratory Reviews 03/2013;
• Article: Adults with Cystic Fibrosis Should be Treated at a Specialist Centre.

  Andrew M Jones
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  ABSTRACT: Cystic fibrosis is a complex multisystem disease. It is recommended that clinical care for adults with cystic fibrosis should be delivered through specialist centres staffed by multidisciplinary teams and assisted by appropriate support services. The centre should be able to meet the national service specifications for adult CF care including the provision of emergency cover and advice for patients, 24hours a day, 7 days a week. Specialist centre care has been shown to be associated with increased patient satisfaction and improved clinical outcomes for adults with CF.
  Paediatric Respiratory Reviews 03/2013;
• Article: New molecular virus detection methods and their clinical value in lower respiratory tract infections in children.

  Tuomas Jartti, Maria Söderlund-Venermo, Klaus Hedman, Olli Ruuskanen, Mika J Mäkelä
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  ABSTRACT: During the past decade, several new respiratory viruses and their subgroups have been discovered. All these new viruses, as well as previously known respiratory viruses, can be detected by sensitive PCR methods, which have become popular in the diagnostic workup of respiratory viral infections. Currently, respiratory viruses can be detected in up to 95% of children with lower respiratory tract illness. On the other hand, virus detection rates in asymptomatic children are also high (up to 68%), as are coinfection rates in symptomatic children (up to 43%) and justified concerns of causality have been raised. Imposing progress has been made in developing multiplex quantitative PCR assays; here, several primer sets are run within a single PCR mixture. These PCR assays give a better understanding of the dominant viral infection, of viral infections that may be incipient and of any waning infections than does a single-target PCR. Multiplex PCR assays are also gaining popularity due to their cost-effectiveness and short throughput time compared to multiple single-target PCRs. Our understanding of the indications of virus PCRs and our ability to interpret the results from a clinical point of view have improved. This paper reviews the progress in PCR assays and discusses their role in the diagnosis of lower respiratory tract infections in children.
  Paediatric Respiratory Reviews 03/2013; 14(1):38-45.
• Article: Perinatal exposure to nicotine and implications for subsequent obstructive lung disease.

  Gert S Maritz
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  ABSTRACT: Many diseases are due to gene-environment or epigenetic-environment interactions resulting in a change in the program that controls tissue structure and function. Changes in the in utero and external environment during perinatal development due to parental smoking, or nicotine exposure, may reduce the capacity of the offspring to protect themselves against environmental stressors. Nicotine is genotoxic and also induces reactive oxygen species [ROS] production. It also reduces the antioxidant capacity of the lung. The lungs of the offspring are therefore developing in an environment of an oxidant-antioxidant imbalance with the concomitant adverse effects of the oxidants and nicotine on cell integrity. Consequently, they are more prone to develop respiratory diseases such as asthma and emphysema later in life. The use of NRT by pregnant or lactating females is therefore not an appropriate strategy to quit smoking.
  Paediatric Respiratory Reviews 03/2013; 14(1):3-8.
• Article: Cystic Fibrosis Carrier Screening.

  John Massie, Martin B Delatycki
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  ABSTRACT: Carrier screening for cystic fibrosis (CF) has been available since the early 1990s, yet there are few programs, and none funded as part of a national health care strategy. The aim of this paper is to provide a description of carrier screening for CF and examine the progress that has been made towards the establishment of universal population-based carrier screening programs. This is an evidence based commentary on population-based carrier screening for CF. Peak body recommendations were examined and existing programs for CF carrier screening are reviewed. The attitudes from the non-CF community, CF healthcare professionals and people with CF were studied. Data from health economic assessments is examined. The future of carrier screening for CF in the context of advancing genetic technologies and potentially curative therapies is considered.
  Paediatric Respiratory Reviews 03/2013;