title: Diagnosis, monitoring, and treatment of primary ciliary dyskinesia: PCD foundation consensus recommendations based on state of the art review.
abstract: Primary ciliary dyskinesia (PCD) is a genetically heterogeneous, rare lung disease resulting in chronic oto-sino-pulmonary disease in both children and adults. Many physicians incorrectly diagnose PCD or eliminate PCD from their differential diagnosis due to inexperience with diagnostic testing methods. Thus far, all therapies used for PCD are unproven through large clinical trials. This review article outlines consensus recommendations from PCD physicians in North America who have been engaged in a PCD centered research consortium for the last 10 years. These recommendations have been adopted by the governing board of the PCD Foundation to provide guidance for PCD clinical centers for diagnostic testing, monitoring, and appropriate short and long-term therapeutics in PCD patients.
authors.affiliation: Department of Pediatrics, Montreal Children's Hospital, McGill University, Quebec, Canada.
authors.forename: Adam J
authors.initials: AJ
authors.lastname: Shapiro
authors.affiliation: Department of Pathology and Laboratory Medicine, University of North Carolina School of Medicine, Marsico Lung Institute, Chapel Hill, North Carolina.
authors.forename: Maimoona A
authors.initials: MA
authors.lastname: Zariwala
authors.affiliation: Department of Pediatrics, Washington University School of Medicine, St. Louis, Missouri.
authors.forename: Thomas
authors.initials: T
authors.lastname: Ferkol
authors.affiliation: Department of Pediatrics, Riley Hospital for Children, Indiana University, Indianapolis, Indiana.
authors.forename: Stephanie D
authors.initials: SD
authors.lastname: Davis
authors.affiliation: Department of Pediatrics, Children's Hospital Colorado and University of Colorado School of Medicine, Aurora, Colorado.
authors.forename: Scott D
authors.initials: SD
authors.lastname: Sagel
authors.affiliation: Department of Pediatrics, The Hospital for Sick Children and University of Toronto, Toronto, Ontario, Canada.
authors.forename: Sharon D
authors.initials: SD
authors.lastname: Dell
authors.affiliation: Department of Pediatrics, Seattle Children's Hospital and University of Washington, Seattle, Washington.
authors.forename: Margaret
authors.initials: M
authors.lastname: Rosenfeld
authors.affiliation: National Heart, Lung, and Blood Institute, Bethesda, Maryland.
authors.forename: Kenneth N
authors.initials: KN
authors.lastname: Olivier
authors.affiliation: Department of Pediatrics, Stanford University, Palo Alto, California.
authors.forename: Carlos
authors.initials: C
authors.lastname: Milla
authors.affiliation: Department of Otolaryngology, Montreal Children's Hospital, McGill University, Montreal, Quebec, Canada.
authors.forename: Sam J
authors.initials: SJ
authors.lastname: Daniel
authors.affiliation: Department of Otolaryngology-Head and Neck Surgery, University of North Carolina School of Medicine, Chapel Hill, North Carolina.
authors.forename: Adam J
authors.initials: AJ
authors.lastname: Kimple
authors.affiliation: The PCD Foundation-President, Minneapolis, Minnesota.
authors.forename: Michele
authors.initials: M
authors.lastname: Manion
authors.affiliation: Department of Medicine, University of North Carolina, Marsico Lung Institute, Chapel Hill, North Carolina.
authors.forename: Michael R
authors.initials: MR
authors.lastname: Knowles
authors.affiliation: Department of Pediatrics, University of North Carolina, Marsico Lung Institute, Chapel Hill, North Carolina.
authors.forename: Margaret W
authors.initials: MW
authors.lastname: Leigh
mesh_terms: D001706:Biopsy; D001944:Breath Tests; D002908:Chronic Disease; D019468:Disease Management; D005820:Genetic Testing; D006801:Humans; D007252:Influenza Vaccines / Q000627:therapeutic use; D007619:Kartagener Syndrome / Q000175:diagnosis* / Q000628:therapy*; D008854:Microscopy, Electron; D018715:Microscopy, Video; D009656:North America; D022242:Pneumococcal Vaccines / Q000627:therapeutic use; D022261:Respiratory Syncytial Virus Vaccines / Q000627:therapeutic use; D012138:Respiratory Therapy / Q000379:methods
chemical_list: D007252:Influenza Vaccines; D022242:Pneumococcal Vaccines; D022261:Respiratory Syncytial Virus Vaccines
keywords: PCD Foundation; PCD, kartagener; consensus statement; primary ciliary dyskinesia