Arigliani, Michele;
Kirkham, Fenella J;
Sahota, Sati;
Riley, Mollie;
Liguoro, Ilaria;
Castriotta, Luigi;
Gupta, Atul;
... Aurora, Paul; + view all
(2022)
Lung Clearance Index May Detect Early Peripheral Lung Disease in Sickle Cell Anemia.
Annals of the American Thoracic Society
, 19
(9)
pp. 1507-1515.
10.1513/AnnalsATS.202102-168OC.
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Abstract
RATIONALE: Chronic lung injury is common in sickle cell anemia (SCA) and worsens outcomes. Sensitive lung function tests might predict reversible disease that might benefit from therapeutic interventions. OBJECTIVE: To evaluate whether Lung Clearance Index (LCI), Sacin & Scond, measuring global, intracinar & conductive ventilation inhomogeneity respectively, are more frequently abnormal than lung volumes in young people with SCA. METHODS: Nitrogen multiple breath washout, spirometry and body plethysmography were cross-sectionally evaluated at steady state in subjects with SCA (hemoglobin SS) and healthy controls aged 8-21 years from London, UK. RESULTS: 35 patients (51% boys; mean±SD 16.4±3.5 years) and 31 controls (48% boys; 16.2±3.2 years) were tested. There were significant differences between the study and control group in mean LCI (mean difference 0.42 units, 95%CI 0.22 to 0.63, p = 0.0001), Sacin (mean difference 0.014 units, 95%CI 0.001 to 0.026, p = 0.04), FEV1 (mean difference -0.79 z-scores, 95%CI -1.28 to -0.30, p = 0.002), FVC (mean difference -0.80 z-scores, 95%CI -1.28 to -0.31) and TLC (mean difference -0.79 z-scores, 95%CI -1.25 to -0.29), but not in Scond and FEV1/FVC ratio. While 29% (10/35) of patients had LCI >95th percentile of controls, 23% (8/35) had abnormal FEV1 (<5th of the reference population). CONCLUSION: Lung clearance index detected slightly more abnormalities than lung volumes in young people with SCA. Significant differences with controls in LCI and Sacin but not in Scond and FEV1/FVC ratio suggest that the lung function changes were most likely due to patchy peripheral lung disease.
| Type: | Article |
|---|---|
| Title: | Lung Clearance Index May Detect Early Peripheral Lung Disease in Sickle Cell Anemia |
| Location: | United States |
| Open access status: | An open access version is available from UCL Discovery |
| DOI: | 10.1513/AnnalsATS.202102-168OC |
| Publisher version: | https://doi.org/10.1513/AnnalsATS.202102-168OC |
| Language: | English |
| Additional information: | This version is the author accepted manuscript. For information on re-use, please refer to the publisher’s terms and conditions. |
| UCL classification: | UCL UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > UCL GOS Institute of Child Health > Developmental Neurosciences Dept UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > UCL GOS Institute of Child Health UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences |
| URI: | https://discovery.ucl.ac.uk/id/eprint/10144360 |
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