| 1. |
- Amin, Reshma, et al.
(författare)
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Hypertonic saline improves the LCI in paediatric patients with CF with normal lung function.
- 2010
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Ingår i: Thorax. - : BMJ. - 1468-3296 .- 0040-6376. ; 65:5, s. 379-83
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Tidskriftsartikel (refereegranskat)abstract
- BACKGROUND AND AIMS Sensitive outcome measures to assess the efficacy of therapeutic interventions in patients with cystic fibrosis (CF) with mild lung disease are currently lacking. Our objective was to study the ability of the lung clearance index (LCI), a measure of ventilation inhomogeneity, to detect a treatment response to hypertonic saline inhalation in paediatric patients with CF with normal spirometry. METHODS In a crossover trial, 20 patients with CF received 4 weeks of hypertonic saline (HS) and isotonic saline (IS) in a randomised sequence separated by a 4 week washout period. The primary end point was the change in the LCI due to HS versus IS. RESULTS Baseline characteristics including the LCI were not significantly different between both study periods. Four weeks of twice-daily HS inhalation significantly improved the LCI compared with IS (1.16, 95% CI 0.26 to 2.05; p=0.016), whereas other outcome measures such as spirometry and quality of life failed to reach statistical significance. Randomisation order had no significant impact on the treatment effect. CONCLUSIONS The LCI, but not spirometry was able to detect a treatment effect from HS inhalation in patients with CF with mild disease and may be a suitable tool to assess early intervention strategies in this patient population. Clinical trial number NCT00635141.
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| 2. |
- Amin, Reshma, et al.
(författare)
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The effect of dornase alfa on ventilation Inhomogeneity in patients with Cystic Fibrosis.
- 2011
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Ingår i: The European respiratory journal. - : European Respiratory Society (ERS). - 1399-3003 .- 0903-1936. ; 37:4, s. 806-812
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Tidskriftsartikel (refereegranskat)abstract
- Outcome measures to assess therapeutic interventions in cystic fibrosis (CF) patients with mild lung disease are lacking. Our aim was to determine if the Lung Clearance Index (LCI) can detect a treatment response to dornase alfa in paediatric CF patients with normal spirometry. CF patients between 6 and 18 years of age with FEV1% ≥80% predicted were eligible. In a crossover design, 17 patients received 4 weeks of dornase alfa and placebo in a randomized sequence separated by a 4-weekwashout period. The primary endpoint was the change in LCI from dornase alfa versus placebo. A mixed model approach incorporating period-dependent baselines was used. This trial was registered with ClinicalTrials.gov, number NCT00557089. The mean age±SD was 10.32±3.35 years. Dornase alfa improved LCI versus placebo (0.90±1.44, p=0.022). Forced expiratory flow at 25-75% expired volume (FEF25-75) measured by percent predicted and z-scores also improved in subjects on dornase alfa (6.1%±10.34, p=0.03; and 0.28 z-score±0.46, p=0.03). Dornase alfa significantly improved LCI. Therefore the LCI may be a suitable tool to assess early intervention strategies in this patient population.
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| 3. |
- Subbarao, Padmaja, et al.
(författare)
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Multiple-Breath Washout as a Lung Function Test in Cystic Fibrosis. A Cystic Fibrosis Foundation Workshop Report.
- 2015
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Ingår i: Annals of the American Thoracic Society. - 2325-6621. ; 12:6, s. 932-9
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Tidskriftsartikel (refereegranskat)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.
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