Paediatric respiratory reviews
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Paediatr Respir Rev · Jun 2016
ReviewPathomechanisms of Congenital Cystic Lung Diseases: Focus on Congenital Cystic Adenomatoid Malformation and Pleuropulmonary Blastoma.
It is well established that a number of birth defects are associated with improper formation of the respiratory tract. Important progress has been made in the identification of components of the regulatory networks controlling lung morphogenesis. ⋯ Congenital cystic lung diseases consist of a heterogeneous group of rare lung diseases mainly detected prenatally and characterized by airway dilatation. Despite their apparent phenotypic heterogeneity, these malformations are proposed to be related to a common malformation sequence occurring during lung branching morphogenesis.
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The increase in allergic airways disease has been linked to modern urbanization and lifestyle. Recent evidence suggests that the associated reduction in microbial exposure, reduction in dietary fibre intake and increased antibiotic use may cause early dysbiosis in infancy, which predisposes to immune dysregulation and allergic airways disease later in life. This implies that there may be a window of opportunity for primary prevention strategies aimed to protect or restore the microbiome early in life and thereby decrease the risk of developing allergic airways disease. Alternatively, strategies that correct dysbiosis may aid in the treatment of established allergic airways disease.
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Diagnosing primary ciliary dyskinesia is difficult. With no reference standard, a combination of tests is needed; most tests require expensive equipment and specialist scientists. ⋯ We particularly focus on use of high-speed video analysis, transmission electron microscopy, nasal nitric oxide and genetic testing. We discuss the international efforts that are in place to advance the evidence base for diagnostic tests.
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Non-invasive ventilation is useful to treat some forms of respiratory failure. Hence, the number of patients receiving this treatment is steadily increasing. Considerable conceptual and technical progress has been made in the last years by manufacturers concerning this technique. ⋯ The goal of this review is to deal with latest advances in ventilatory modes and features available for non-invasive ventilation. We present a comprehensive analysis of new modes of ventilator assistance committed to treat respiratory failure (hybrid modes) and central and complex sleep apnea (adaptive servo ventilation), and of new modes of triggering and cycling (neurally adjusted ventilatory assist). Technical aspects, modes of operation and settings of these new features as well as an exhaustive review of published data, their benefits and limits, and the potential place of these devices in clinical practice, are discussed.