Objective: Pulmonary complications including acute chest syndrome, are leading causes of sickle cell disease (SCD) related morbidity and mortality. Prior studies have shown that patients with pulmonary complications have evidence of pulmonary involvement reflected in lung functions trends as early as childhood. Spirometry is the current standard for measuring lung function. Growing evidence suggests that lung clearance index (LCI) which is a commonly reported parameter of multiple breath washout (MBW) tests, is more sensitive than spirometry measurements in the early identification of pulmonary changes in pediatric patients. The aim of our study was to determine if this relationship between LCI and spirometry existed within the pediatric sickle cell population. Study Design: This is a cross-sectional, retrospective study to compare LCI to spirometry measurements in children with SCD. Charts were reviewed of clinic encounters at Phoenix Children’s Hospital from March 1, 2013 – June 30, 2017. Spirometry and MBW measurements were collected from 23 patients between the ages of 5 years – 22 years. The MBW utilized sulfur hexafluoride (SF6) as the tracer gas. Demographics and SCD variant (e.g. HbSS, HbSC, etc.) for each encounter were also collected. Results: Our results show that LCI correlates to FEV1% predicted (Spearman’s coefficient -0.44, p = 0.003) and FEF25-75% (Spearman’s coefficient -0.49, p <0.001) over time. Based on demographics, LCI is affected by weight (p = 0.046) but not age or height. When comparing LCI and FEV1% predicted, abnormal LCI results were noted to occur even in the presence of normal FEV1% predicted measurements. Conclusions: Our data support LCI correlating with spirometry measurements, but more studies are necessary to explore whether LCI can be used as a screening test to detect pulmonary changes in young children with SCD. Earlier monitoring of lung function will allow for preventative therapies and delayed progression of pulmonary dysfunction.
| Published in | American Journal of Pediatrics (Volume 8, Issue 2) |
| DOI | 10.11648/j.ajp.20220802.12 |
| Page(s) | 56-62 |
| Creative Commons |
This is an Open Access article, distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution and reproduction in any medium or format, provided the original work is properly cited. |
| Copyright |
Copyright © The Author(s), 2022. Published by Science Publishing Group |
Spirometry, Pediatrics, Sickle Cell, Pulmonary Function Tests
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APA Style
Sophia Williams, Monica Chaung, Paul Kang. (2022). Lung Clearance Index to Detect Early Pulmonary Changes in Children with Sickle Cell Disease. American Journal of Pediatrics, 8(2), 56-62. https://doi.org/10.11648/j.ajp.20220802.12
ACS Style
Sophia Williams; Monica Chaung; Paul Kang. Lung Clearance Index to Detect Early Pulmonary Changes in Children with Sickle Cell Disease. Am. J. Pediatr. 2022, 8(2), 56-62. doi: 10.11648/j.ajp.20220802.12
AMA Style
Sophia Williams, Monica Chaung, Paul Kang. Lung Clearance Index to Detect Early Pulmonary Changes in Children with Sickle Cell Disease. Am J Pediatr. 2022;8(2):56-62. doi: 10.11648/j.ajp.20220802.12
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Download@article{10.11648/j.ajp.20220802.12,
author = {Sophia Williams and Monica Chaung and Paul Kang},
title = {Lung Clearance Index to Detect Early Pulmonary Changes in Children with Sickle Cell Disease},
journal = {American Journal of Pediatrics},
volume = {8},
number = {2},
pages = {56-62},
doi = {10.11648/j.ajp.20220802.12},
url = {https://doi.org/10.11648/j.ajp.20220802.12},
eprint = {https://article.sciencepublishinggroup.com/pdf/10.11648.j.ajp.20220802.12},
abstract = {Objective: Pulmonary complications including acute chest syndrome, are leading causes of sickle cell disease (SCD) related morbidity and mortality. Prior studies have shown that patients with pulmonary complications have evidence of pulmonary involvement reflected in lung functions trends as early as childhood. Spirometry is the current standard for measuring lung function. Growing evidence suggests that lung clearance index (LCI) which is a commonly reported parameter of multiple breath washout (MBW) tests, is more sensitive than spirometry measurements in the early identification of pulmonary changes in pediatric patients. The aim of our study was to determine if this relationship between LCI and spirometry existed within the pediatric sickle cell population. Study Design: This is a cross-sectional, retrospective study to compare LCI to spirometry measurements in children with SCD. Charts were reviewed of clinic encounters at Phoenix Children’s Hospital from March 1, 2013 – June 30, 2017. Spirometry and MBW measurements were collected from 23 patients between the ages of 5 years – 22 years. The MBW utilized sulfur hexafluoride (SF6) as the tracer gas. Demographics and SCD variant (e.g. HbSS, HbSC, etc.) for each encounter were also collected. Results: Our results show that LCI correlates to FEV1% predicted (Spearman’s coefficient -0.44, p = 0.003) and FEF25-75% (Spearman’s coefficient -0.49, p 1% predicted, abnormal LCI results were noted to occur even in the presence of normal FEV1% predicted measurements. Conclusions: Our data support LCI correlating with spirometry measurements, but more studies are necessary to explore whether LCI can be used as a screening test to detect pulmonary changes in young children with SCD. Earlier monitoring of lung function will allow for preventative therapies and delayed progression of pulmonary dysfunction.},
year = {2022}
}
TY - JOUR T1 - Lung Clearance Index to Detect Early Pulmonary Changes in Children with Sickle Cell Disease AU - Sophia Williams AU - Monica Chaung AU - Paul Kang Y1 - 2022/04/14 PY - 2022 N1 - https://doi.org/10.11648/j.ajp.20220802.12 DO - 10.11648/j.ajp.20220802.12 T2 - American Journal of Pediatrics JF - American Journal of Pediatrics JO - American Journal of Pediatrics SP - 56 EP - 62 PB - Science Publishing Group SN - 2472-0909 UR - https://doi.org/10.11648/j.ajp.20220802.12 AB - Objective: Pulmonary complications including acute chest syndrome, are leading causes of sickle cell disease (SCD) related morbidity and mortality. Prior studies have shown that patients with pulmonary complications have evidence of pulmonary involvement reflected in lung functions trends as early as childhood. Spirometry is the current standard for measuring lung function. Growing evidence suggests that lung clearance index (LCI) which is a commonly reported parameter of multiple breath washout (MBW) tests, is more sensitive than spirometry measurements in the early identification of pulmonary changes in pediatric patients. The aim of our study was to determine if this relationship between LCI and spirometry existed within the pediatric sickle cell population. Study Design: This is a cross-sectional, retrospective study to compare LCI to spirometry measurements in children with SCD. Charts were reviewed of clinic encounters at Phoenix Children’s Hospital from March 1, 2013 – June 30, 2017. Spirometry and MBW measurements were collected from 23 patients between the ages of 5 years – 22 years. The MBW utilized sulfur hexafluoride (SF6) as the tracer gas. Demographics and SCD variant (e.g. HbSS, HbSC, etc.) for each encounter were also collected. Results: Our results show that LCI correlates to FEV1% predicted (Spearman’s coefficient -0.44, p = 0.003) and FEF25-75% (Spearman’s coefficient -0.49, p 1% predicted, abnormal LCI results were noted to occur even in the presence of normal FEV1% predicted measurements. Conclusions: Our data support LCI correlating with spirometry measurements, but more studies are necessary to explore whether LCI can be used as a screening test to detect pulmonary changes in young children with SCD. Earlier monitoring of lung function will allow for preventative therapies and delayed progression of pulmonary dysfunction. VL - 8 IS - 2 ER -
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