Abstract
Primary ciliary dyskinesia (PCD) is a rare disease causing motile cilia dysfunction, recurrent airway infection, and bronchiectasis. Airway infection management strategies are borrowed from cystic fibrosis. The aim of this study is to describe the management of airway infection with Pseudomonas aeruginosa ( PA) in children and adults with PCD across European centers. An online survey questionnaire was sent electronically using SurveyMonkey® to 55 PCD centers in 36 European countries. Fifty-two responded from 43 centers in 26 countries, a response rate of 70%. Most (89%) countries did not have written guidelines for PCD management. Airway sampling for infection detection at each clinic visit was more likely when follow-up was frequent. Eighty-seven percent of centers chose to treat the first PA isolate, most prescribing combined oral ciprofloxacin and inhaled colistimethate sodium (43%, n = 18). The preferred treatment for chronic infection with PA was nebulized colistimethate in 51% ( n = 22). In summary, considerable variation exists across European centers in the frequency of patient follow-up and airway sampling for infection, treatment goals, and the management of PA infection. Few centers had written guidelines for PCD man...Continue Reading
References
Sep 21, 1991·Lancet·N H ValeriusN Høiby
Sep 7, 1996·Lancet·K ChengC A Hart
May 1, 1997·Pediatric Pulmonology·B FrederiksenN Høiby
Oct 23, 2001·Archives of Disease in Childhood·A C EquiA Bush
Dec 6, 2003·American Journal of Respiratory and Critical Care Medicine·Peadar G NooneMichael R Knowles
Oct 7, 2004·Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society·Tim W R LeeJames M Littlewood
Sep 29, 2007·Pediatric Pulmonology·T F MoriartyM M Tunney
Oct 7, 2008·Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society·Tacjana PresslerUNKNOWN Scandinavian CF Study Consortium
Apr 18, 2009·Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society·J S ElbornC Bertram
Jun 10, 2010·The European Respiratory Journal·C E KuehniUNKNOWN ERS Task Force on Primary Ciliary Dyskinesia in Children
Dec 30, 2011·Respiratory Medicine·Laura WhiteJay Suntharalingam
Jan 28, 2012·The European Respiratory Journal·Marie-Pierre F StrippoliUNKNOWN ERS Task Force onPrimary Ciliary Dyskinesia in Children
Sep 27, 2013·Journal of Clinical Microbiology·Geraint B RogersJane S Lucas
Oct 5, 2013·Chest·Malena Cohen-CymberknohEitan Kerem
Jan 15, 2014·Pediatric Pulmonology·Marco MaglioneFrancesca Santamaria
May 27, 2014·Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society·Alan R SmythUNKNOWN European Cystic Fibrosis Society
Nov 6, 2014·Acta Oto-laryngologica·Mikkel Christian AlaninChristian von Buchwald
Sep 6, 2015·Clinical Microbiology and Infection : the Official Publication of the European Society of Clinical Microbiology and Infectious Diseases·M C AlaninH K Johansen
Sep 30, 2015·Pediatric Pulmonology·Adam J ShapiroUNKNOWN Genetic Disorders of Mucociliary Clearance Consortium
May 5, 2016·Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society·Malena Cohen-CymberknohEitan Kerem
Jun 12, 2016·The European Respiratory Journal·Anand ShahMichael R Loebinger
Jul 7, 2016·Thorax·Justine Frija-MassonBernard Maître
Apr 1, 2017·PloS One·Alissa S HanshewSusan L Thibeault
Jul 27, 2017·Expert Review of Respiratory Medicine·Jane S LucasWoolf T Walker
Sep 11, 2017·The European Respiratory Journal·Eva PolverinoJames D Chalmers
Sep 28, 2017·Respiratory Medicine·Malena Cohen-CymberknohEitan Kerem
Citations
Dec 22, 2019·International Journal of Molecular Sciences·Giuseppantonio MaisettaGiovanna Batoni
Sep 24, 2020·ERJ Open Research·Suzanne CrowleyJane S Lucas
Jun 25, 2020·BMC Proceedings·Laura E GardnerClaire Hogg
Sep 26, 2020·Pneumologie·J RaidtH Omran
Feb 6, 2021·The European Respiratory Journal·Myrofora GoutakiUNKNOWN BEAT-PCD Clinical Research Collaboration
Aug 6, 2021·ERJ Open Research·June K MarthinKim G Nielsen