ERS clinical practice guidelines: high-flow nasal cannula in acute respiratory failure.

Background: High-flow nasal cannula (HFNC) has become a frequently used noninvasive form of respiratory support in acute settings; however, evidence supporting its use has only recently emerged. These guidelines provide evidence-based recommendations for the use of HFNC alongside other noninvasive forms of respiratory support in adults with acute respiratory failure (ARF).

Materials And Methodology: The European Respiratory Society task force panel included expert clinicians and methodologists in pulmonology and intensive care medicine.

Long-Term Mechanical Insufflation-Exsufflation Cough Assistance in Neuromuscular Disease: Patterns of Use and Lessons for Application.

Background: Mechanical insufflation-exsufflation (MI-E) devices increase expiratory air flow and thereby promote increased cough peak flow (CPF) in conjunction with a cough. There is little research looking at long-term use of MI-E in subjects with neuromuscular disease (NMD), and no long-term study has reported CPF, MI-E device settings, and adherence.

Methods: We evaluated 181 patient records (130 adults, 51 children) of individuals who received a MI-E device from our center between February 2014 and February 2018.

Sleep disordered breathing and ventilatory support in children with Down syndrome.

Study Objectives: Obstructive sleep apnoea (OSAS) in children with Down syndrome (DS) is now well recognized, but other forms of sleep disordered breathing (SDB) in this population are less well described. Anecdotally, respiratory support for SDB treatment in this population is not easily tolerated. We aimed to characterize the types of SDB in children with DS referred to a tertiary respiratory center and to assess the effectiveness and adherence to respiratory support.

Airway clearance techniques in neuromuscular disorders: A state of the art review.

This is a unique state of the art review written by a group of 21 international recognized experts in the field that gathered during a meeting organized by the European Neuromuscular Centre (ENMC) in Naarden, March 2017. It systematically reports the entire evidence base for airway clearance techniques (ACTs) in both adults and children with neuromuscular disorders (NMD). We not only report randomised controlled trials, which in other systematic reviews conclude that there is a lack of evidence base to give an opinion, but also include case series and retrospective reviews of practice.

Long term non-invasive ventilation in children: impact on survival and transition to adult care.

Background: The number of children receiving domiciliary ventilatory support has grown over the last few decades driven largely by the introduction and widening applications of non-invasive ventilation. Ventilatory support may be used with the intention of increasing survival, or to facilitate discharge home and/or to palliate symptoms. However, the outcome of this intervention and the number of children transitioning to adult care as a consequence of longer survival is not yet clear.

The addition of mechanical insufflation/exsufflation shortens airway-clearance sessions in neuromuscular patients with chest infection.

Background: Mechanical insufflation/exsufflation (in-exsufflation) increases peak cough flow and may improve sputum clearance. No studies have compared current respiratory physiotherapy practice (manual percussion, shaking, and assisted cough) plus mechanical in-exsufflation to current physiotherapy practice without in-exsufflation in noninvasive-ventilation (NIV) dependent neuromuscular patients with an acute respiratory-tract infection. We hypothesized that airway clearance in these patients would be more effective with in-exsufflation, compared to without in-exsufflation.

Randomised trial of inpatient versus outpatient initiation of home mechanical ventilation in patients with nocturnal hypoventilation.

Background: Long-term home mechanical ventilation (HMV) is usually initiated in hospital. Admission to hospital has resource implications and may not be reimbursable in some healthcare systems.

Methods: Twenty-eight stable neuromuscular and chest wall disease patients with nocturnal hypoventilation (transcutaneous carbon dioxide (TcCO(2) >6.