Lung Clearance Index as an Outcome Measure for Clinical Trials in Young Children with Cystic Fibrosis. A Pilot Study Using Inhaled Hypertonic Saline

Subbarao, Padmaja; Stanojevic, Sanja; Brown, Meghan; Jensen, Renée; Rosenfeld, Margaret; Davis, Stephanie D.; Brumback, Lyndia C.; Gustafsson, Per; Ratjen, Félix

doi:10.1164/rccm.201302-0219oc

Cited by 151 publications

(114 citation statements)

References 29 publications

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“…To date, one single-center study examined the usefulness of LCI (measured by respiratory mass spectrometry) in an interventional study in infants and preschool children (50). This add-on pilot study to the Infant Study of Inhaled Saline (ISIS) in CF trial (51) attempted MBW measurements in all 27 infant and preschool subjects participating in the Toronto center.…”

Section: Clinical Trialsmentioning

confidence: 99%

Multiple-Breath Washout as a Lung Function Test in Cystic Fibrosis. A Cystic Fibrosis Foundation Workshop Report

et al. 2015

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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 FEV 1 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.

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Section: Clinical Trialsmentioning

confidence: 99%

Multiple-Breath Washout as a Lung Function Test in Cystic Fibrosis. A Cystic Fibrosis Foundation Workshop Report

et al. 2015

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“…It is essential to have surrogate markers in order to define the disease severity status [3], to detect the lung destruction in earlier stages [4,5] or to estimate the effectiveness of a treating intervention [6][7][8][9][10][11][12]. An appropriate outcome measure has to meet some criteria: It must reflect the presence and severity of the disease.…”

Section: Introductionmentioning

confidence: 99%

“…MBW is a non-invasive and safe test for assessing ventilation inhomogeneity. LCI is the most common index of uneven ventilation distribution and has recently been proposed as a sensitive marker for the detection of early pulmonary changes in CF [35][36][37] and the assessment of treatment effectiveness [6][7][8][9][10][11][12]. LCI seems to allow earlier detection of disease, compared to FEV 1 [36,37].…”

Section: Introductionmentioning

confidence: 99%

Is Lung Clearance Index (LCI) Affected by the Severity of Lung Disease in CF?

Avramidou¹,

Hatziagorou²,

Kampouras³

et al. 2017

J Pulm Respir Med

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“…As in previous years, the argument for bringing the CT scan and MBW forward as clinical end-points and relating these investigations to the various clinical outcomes is still hot topic [54]. In two studies, SHEIKH and co-workers [55,56] showed that HRCT during general anaesthesia and infant lung function testing (raised volume thoraco-abdominal compression technique) during sedation improved after intravenous antibiotic therapy in at total of 49 infants (21 in one study and 28 in the other) with CF at a mean age of 21 weeks, underscoring that untreated CF is a progressive disease.…”

Section: Cystic Fibrosismentioning

confidence: 99%