BackgroundMucopolysaccharidosis type II (MPSII) patients frequently suffer from dyspnoea caused by restrictive airway disease due to skeletal abnormalities as well as glycosaminoglycans (GAG) accumulation at different levels of the airway, including the trachea. In this study we describe the extent of the tracheal and bronchial narrowing, the changes in airway diameter during respiration and the effects of these obstructions on respiratory function in adult MPSII patients.MethodsFive adult MPSII patients (mean age 40 years) were included. Pulmonary function tests and in- and expiratory chest CT scans were obtained. Cross-sectional areas of trachea and main bronchi were measured at end-inspiration and -expiration and percentage collapse was calculated.ResultsThere was diffuse narrowing of the entire intra-thoracic trachea and main bronchi and severe expiratory collapse of the trachea in all patients. At 1 cm above the aortic arch the median % collapse of the trachea was 68 (range 60 to 77 %), at the level of the aortic arch 64 (range 21–93 %), for the main bronchi this was 58 (range 26–66 %) on the left and 44 (range 9–76 %) on the right side. The pulmonary function tests showed that this airway collapse results in obstructive airway disease in all patients, which was severe (forced expiratory volume <50 % of predicted) in four out of five patients.ConclusionIn adult MPS II patients, central airways diameters are strikingly reduced and upon expiration there is extensive collapse of the trachea and main bronchi. This central airways obstruction explains the severe respiratory symptoms in MPSII patients.Electronic supplementary materialThe online version of this article (doi:10.1186/s13023-016-0425-z) contains supplementary material, which is available to authorized users.

There is a growing number of patients being treated with long-term home mechanical ventilation (HMV). This poses a challenge for the healthcare system because in-hospital resources are decreasing. The application of digital health to assist HMV care might help. In this narrative review we discuss the evidence for using telemonitoring to assist in initiation and follow-up of patients on long-term HMV. We also give an overview of available technology and discuss which parameters can be measured and how often this should be done. To get a telemonitoring solution implemented in clinical practice is often complex; we discuss which factors contribute to that. We discuss patients’ opinions regarding the use of telemonitoring in HMV. Finally, future perspectives for this rapidly growing and evolving field will be discussed.

In 2007, the European Respiratory Society (ERS) delivered the Noninvasive Ventilation (NIV) course in Hannover, Germany, for the first time. This teaching course was to become the birth of a successful tradition in the delivery of NIV education by ERS. In the following 10 years, the Hannover NIV course has been held on four occasions, in 2009, 2011, 2014 and, just recently in 2017. Each of these courses have been fully booked with around 80 delegates. Such was the popularity of the course and the rapidly expanding knowledge in this area of acute NIV and home mechanical ventilation (HMV) that, in 2014, the ERS agreed to deliver both a “basic concepts” and an “advanced concepts” course. The Hannover NIV course focused on the basic concepts, whereas the NIV course held for the first time in November 2015 in Milan, Italy, focused on advanced concepts.

No abstract