Archives of disease in childhood
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Non-invasive ventilation (NIV) use has increased markedly over the last 10 years. Children being treated with NIV are now a common sight in most paediatric intensive care units and high dependency units and nearly all tertiary respiratory units will look after a cohort of children who use NIV at home. ⋯ Effectiveness of NIV will need to be evaluated on each child as it used. It is important to define the purpose of using NIV in each child, and then determine whether it is effective.
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Intravenous salbutamol is commonly used to treat children with severe asthma unresponsive to inhaled β2-agonist therapy. However, in this setting, there is little clinical trial data demonstrating its effectiveness. Additionally, there are significant concerns that intravenous salbutamol-dosing recommendations for children with acute asthma are excessive, and unnecessarily raise the potential for adverse reactions, such as lactic acidosis and tachycardia which, by increasing respiratory workload, exacerbate respiratory failure. Here, we review salbutamol clinical pharmacology and toxicology, evidence relating to its use in acute asthma and highlight gaps in the evidence base.
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Primary ciliary dyskinesia (PCD) is an inherited autosomal-recessive disorder of motile cilia characterised by chronic lung disease, rhinosinusitis, hearing impairment and subfertility. Nasal symptoms and respiratory distress usually start soon after birth, and by adulthood bronchiectasis is invariable. Organ laterality defects, usually situs inversus, occur in ∼50% of cases. ⋯ This review examines who to refer for diagnostic testing. It describes the limitations surrounding diagnosis using currently available techniques and considers whether recent advances to genotype patients with PCD will lead to genetic testing and screening to aid diagnosis in the near future. It discusses the challenges of monitoring and treating respiratory and ENT disease in children with PCD.