title: Multiple-Breath Washout as a Lung Function Test in Cystic Fibrosis. A Cystic Fibrosis Foundation Workshop Report.
abstract: The lung clearance index (LCI) is a lung function parameter derived from the multiple-breath washout (MBW) test. Although first developed 60 years ago, the technique was not widely used for many years. Recent technological advances in equipment design have produced gains in popularity for this test among cystic fibrosis (CF) researchers and clinicians, particularly for testing preschool-aged children. LCI has been shown to be feasible and sensitive to early CF lung disease in patients of all ages from infancy to adulthood. A workshop was convened in January 2014 by the North American Cystic Fibrosis Foundation to determine the readiness of the LCI for use in multicenter clinical trials as well as clinical care. The workshop concluded that the MBW text is a valuable potential outcome measure for CF clinical trials in preschool-aged patients and in older patients with FEV1 in the normal range. However, gaps in knowledge about the choice of device, gas, and standardization across systems are key issues precluding its use as a clinical trial end point in infants. Based on the current evidence, there are insufficient data to support the use of LCI or MBW parameters in the routine clinical management of patients with CF.
authors.affiliation: 1 Division of Respiratory Medicine, Department of Pediatrics, Physiology, and Experimental Medicine, The Research Institute, Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.
authors.forename: Padmaja
authors.initials: P
authors.lastname: Subbarao
authors.affiliation: 2 Lucile Salter Packard Children's Hospital, Stanford University, Palo Alto, California.
authors.forename: Carlos
authors.initials: C
authors.lastname: Milla
authors.affiliation: 3 Great Ormond Street Hospital, London, United Kingdom.
authors.forename: Paul
authors.initials: P
authors.lastname: Aurora
authors.affiliation: 4 Royal Brompton Hospital, Imperial College London, London, United Kingdom.
authors.forename: Jane C
authors.initials: JC
authors.lastname: Davies
authors.affiliation: 5 Riley Hospital for Children at IU Health, Indiana University School of Medicine, Indianapolis, Indiana.
authors.forename: Stephanie D
authors.initials: SD
authors.lastname: Davis
authors.affiliation: 6 Telethon Kids Institute, University of Western Australia, Perth, Australia.
authors.forename: Graham L
authors.initials: GL
authors.lastname: Hall
authors.affiliation: 7 Seattle Children's Research Institute, University of Washington, Seattle, Washington.
authors.forename: Sonya
authors.initials: S
authors.lastname: Heltshe
authors.affiliation: 8 University Children's Hospital Basel (UKBB), Basel, Switzerland.
authors.forename: Philipp
authors.initials: P
authors.lastname: Latzin
authors.affiliation: 9 Queen Silvia Children's Hospital, University of Gothenburg, Gothenburg, Sweden.
authors.forename: Anders
authors.initials: A
authors.lastname: Lindblad
authors.affiliation: 10 Washington University School of Medicine, St. Louis, Missouri.
authors.forename: Jessica E
authors.initials: JE
authors.lastname: Pittman
authors.affiliation: 11 Children's Hospital at Westmead, University of Sydney, Sydney, Australia.
authors.forename: Paul D
authors.initials: PD
authors.lastname: Robinson
authors.affiliation: 12 Seattle Children's Hospital, University of Washington, Seattle, Washington.
authors.forename: Margaret
authors.initials: M
authors.lastname: Rosenfeld
authors.affiliation: 13 University Children's Hospital Zurich, Zurich, Switzerland.
authors.forename: Florian
authors.initials: F
authors.lastname: Singer
authors.affiliation: 14 Children's Hospital of Iowa, University of Iowa, Iowa City, Iowa; and.
authors.forename: Tim D
authors.initials: TD
authors.lastname: Starner
authors.affiliation: 1 Division of Respiratory Medicine, Department of Pediatrics, Physiology, and Experimental Medicine, The Research Institute, Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada.
authors.forename: Felix
authors.initials: F
authors.lastname: Ratjen
authors.affiliation: 15 Arizona Health Science Center, University of Arizona, Tucson, Arizona.
authors.forename: Wayne
authors.initials: W
authors.lastname: Morgan
mesh_terms: D001944:Breath Tests / Q000295:instrumentation / Q000379:methods; D002675:Child, Preschool; D003550:Cystic Fibrosis / Q000175:diagnosis* / Q000503:physiopathology; D018450:Disease Progression; D045268:Flowmeters; D005541:Forced Expiratory Volume; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008991:Monitoring, Physiologic / Q000379:methods; D017063:Outcome Assessment, Health Care; D011379:Prognosis; D015203:Reproducibility of Results; D012129:Respiratory Function Tests / Q000379:methods
keywords: cystic fibrosis; lung clearance index; multiple-breath washout; pulmonary function tests