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Current opinions on non-invasive ventilation as a treatment for chronic obstructive pulmonary disease

imagePurpose of reviewThis review examines the current reports, the evidence and the issues surrounding the use of non-invasive ventilation (NIV) for the treatment of chronic obstructive pulmonary disease (COPD) in both the acute and domiciliary setting. Recent findingsWith the increasing use of NIV, more recent studies have focused on investigating the outcomes of our current practice. Although overall morbidity and mortality outcomes in the acute setting have improved, patients who initially stabilize but then deteriorate during an acute exacerbation of COPD have a poor prognosis. The focus must be on phenotyping this high-risk group to investigate other potential rescue treatments, including extracorporeal carbon dioxide removal. Indeed, phenotyping appears to favour the obese COPD patient, which may have a protective role in reducing the risk of NIV failure and recurrent hospital admissions. Randomized controlled trial evidence to support the use of NIV in a domiciliary setting as a treatment for COPD is awaited, and until the data from a number of ongoing clinical trials are available, the wide variation in global practice will continue. Increased understanding of patient ventilator asynchrony has improved domiciliary NIV set up, which is expected to enhance the tolerability of NIV, promoting patient adherence. SummaryNIV is the established standard of care to treat acute hypercapnic exacerbations of COPD postoptimal medical management. NIV as a long-term treatment for COPD remains controversial based on the evidence from the published randomized controlled trials. With increasing experience of NIV therapy, patient outcomes are improving; however, further work is still required to better characterize and target the patients who will most benefit from NIV.

Treatment strategies for complex behavioral insomnia in children with neurodevelopmental disorders

imagePurpose of reviewThis review describes recent research in pediatric behavioral insomnias in neurodevelopmental disorders and their treatment. Recent findingsInsomnia in children with autism spectrum disorder (ASD) and other neurodevelopmental disorders (NDDs) is typically complex, chronic, and difficult to adequately control. Abnormalities in genetic and/or epigenetic regulation of sleep/wakefulness and its timing predispose patients with NDD to insomnia, although poor sleep hygiene, maladaptive associations, and limit-setting are likely to contribute. Parents are agents for change in problematic sleep behaviors in patients with NDD. We review the benefits of behavioral therapies and melatonin to treat sleep problems in children with NDD. Problematic sleep is so prevalent in some neurodevelopmental syndromes (Rett, Angelman, Williams, and Smith–Magenis) that it is part of their diagnostic criteria. SummaryChildren and adolescents with neurological disorders frequently have complex sleep disorders that require treatment. Understanding the basic pathology and treatment strategies provides an opportunity to improve well being and quality of life in those affected by NDD and their families.

Modifying disease in cystic fibrosis: current and future therapies on the horizon

imagePurpose of reviewRecent therapies directed at proximal targets within cystic fibrosis (CF) pathophysiology hold potential to modulate disease. This review highlights recent clinical trials and future therapies focused on these early steps of disease. Recent findingsRecent approval of a CF transmembrane conductance regulator (CFTR) protein modulator, ivacaftor (Kalydeco), has ignited a wave of investigations for other modulators directed at CFTR mutation classes. Gene replacement therapy continues to be pursued at a slower pace in early phase clinical trials. Airway surface liquid strategies such as dry-powder mannitol and alternate ion channel regulation are discussed as genotype-independent methods of early modulation. SummaryThe breadth of therapies for early targets of CF holds considerable hope to modify the natural history of this disease. Ongoing focus to develop novel markers of early disease state is paramount. The progress of drug development requires concurrent attention on a spectrum of targets to achieve maximal impact.

Restless legs syndrome: update on pathogenesis

imagePurpose of reviewMuch recent progress has been made in understanding restless legs syndrome (RLS), focusing mainly on genetic predisposition and dysregulation of iron metabolism and the dopaminergic system. We provide in this review an update of the most recent scientific advances on the pathophysiology of primary RLS. Recent findingsGenome-wide association studies identified six genetic variants including MEIS1 and BTBD9 with potential relationships with iron. Brain iron level is low in RLS and neuropathological studies have shown significant decreases in dopamine D2 receptors in the putamen that correlated with RLS severity, and increased tyrosine hydroxylase in the substantia nigra. An overly activated dopaminergic system was reported in both animal and cell models of iron insufficiency thus suggesting that in at least a subgroup of RLS patients altered iron metabolism plays a role in the disorder. Also, dysregulation of iron uptake and storage within brain microvessels was recently reported and might play a role in a subgroup of RLS patients. SummaryRLS is a genetically heterogeneous complex trait with high prevalence but large phenotype variability. Current theories of RLS pathophysiology emphasize brain iron deficiency with abnormal dopaminergic consequences, together with a strong underlying genetic background.

Narcolepsy and H1N1 vaccination: a link?

imagePurpose of reviewA number of European countries have reported a dramatic increase in the rates of childhood narcolepsy with cataplexy in children immunized with a split-virion adjuvanted swine flu vaccine. Here, we review the strengths and weaknesses of these epidemiological studies and possible neuroimmunological mechanisms. Recent findingsInitial concerns of a 13-fold increased relative risk of narcolepsy were raised by the Scandinavian health protection agencies in 2010. Subsequent retrospective studies support these findings in Canada, France, Ireland, England and Denmark. The cases are predominantly young children who present with severe and rapid onset of cataplexy as well as narcolepsy often within a few weeks of vaccination. The proposed mechanism for postvaccination narcolepsy is one in which an environmental trigger causes or enhances an antibody-mediated autoimmune response in patients with a preexisting genetic susceptibility. However, there have not yet been any reports of specific autoimmunity, either antibody or T-cell-mediated. SummaryThere is a strong association between narcolepsy and H1N1 vaccination. However, whether this reflects a true increase in affected individuals or a hastening of disease onset in individuals who would otherwise have developed narcolepsy later will become clear in the coming years. The pathological explanation of this association and narcolepsy is likely to be autoimmune, although supportive evidence is lacking.Video abstract available: See the Video Supplementary Digital Content 1 (http://links.lww.com/COPM/A9).

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