The estimated prevalence of ventilator-dependent individuals in Europe is 6.6 per 100 000 people. The increasing number and costs of these complex patients make present health organisations largely insufficient to face their needs. As a consequence, their burden lays mostly over families. The need to reduce healthcare costs and to increase safety has prompted the development of tele-monitoring for home ventilatory assistance. A European Respiratory Society Task Force produced a literature research based statement on commonly accepted clinical criteria for indications, follow-up, equipment, facilities, legal and economic issues of telemonitoring of these patients. Many remote health monitoring systems are available, ensuring safety, feasibility, effectiveness, sustainability and flexibility to face different patients' needs. The legal problems associated with tele-monitoring are still controversial. National and European Union (EU) governments should develop guidelines and ethical, legal, regulatory, technical, administrative standards for remote medicine. The economic advantages, if any, of this new approach must be compared to a "gold standard" of home care that is very variable among different European countries and within each European country. Much more research is needed before considering tele-monitoring a real improvement in the management of these patients.
This article has supplementary material available from erj.ersjournals.com ; The diagnosis of primary ciliary dyskinesia is often confirmed with standard, albeit complex and expensive, tests. In many cases, however, the diagnosis remains difficult despite the array of sophisticated diagnostic tests. There is no "gold standard" reference test. Hence, a Task Force supported by the European Respiratory Society has developed this guideline to provide evidence-based recommendations on diagnostic testing, especially in light of new developments in such tests, and the need for robust diagnoses of patients who might enter randomised controlled trials of treatments. The guideline is based on pre-defined questions relevant for clinical care, a systematic review of the literature, and assessment of the evidence using the GRADE (Grading of Recommendations, Assessment, Development and Evaluation) approach. It focuses on clinical presentation, nasal nitric oxide, analysis of ciliary beat frequency and pattern by high-speed video-microscopy analysis, transmission electron microscopy, genotyping and immunofluorescence. It then used a modified Delphi survey to develop an algorithm for the use of diagnostic tests to definitively confirm and exclude the diagnosis of primary ciliary dyskinesia; and to provide advice when the diagnosis was not conclusive. Finally, this guideline proposes a set of quality criteria for future research on the validity of diagnostic methods for primary ciliary dyskinesia. ; J.S. Lucas, K.G. Nielsen, C.E Kuehni, C. Hogg, M.W Leigh and H. Omran received funding from the European Union Seventh Framework Programme (FP7/2007–2013) under grant agreement n8305404 (BESTCILIA). J.S. Lucas, L. Behan, W.T. Walker and S.A. Collins were supported by the NIHR Respiratory Biomedical Research Unit at the University Hospital Southampton NHS Foundation Trust (Southampton, UK) and AAIR Charity. A. Bush is an NIHR Senior Investigator and was supported by the NIHR Respiratory Disease Biomedical Research Unit at the Royal Brompton and Harefield NHS Foundation Trust and Imperial College London (both London, UK). M. Goutaki was supported by the following national grants: Bernese Lung League, Milena- Pro Kartagener foundation and Swiss National Foundation 32003B_162820/1. S. Dell and M.W. Leigh received funding from the NIH (U54HL096458) through the Genetic Disorders of Mucociliary Clearance Consortium, an initiative of the NIH Office of Rare Diseases Research at the National Center for Advancing Translational Science, and the National Heart, Lung and Blood Institute. ; Peer-reviewed ; Post-print
