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Age is a predictor of a small decrease in lung function in children with sickle cell anemia

Age is a predictor of a small decrease in lung function in children with sickle cell anemia
Age is a predictor of a small decrease in lung function in children with sickle cell anemia

The longitudinal pattern of lung function in children with sickle cell anemia (SCA) has shown a decrease in FEV1 % predicted, a risk factor for death in adults with SCA, but predictors for this decline are poorly characterized. In a prospective longitudinal multi-center cohort of children with SCA, we tested the hypotheses that: (1) FEV1 % predicted declines over time; and (2) SCA-specific characteristics and therapy predict this decline. At three clinical centers, children with SCA (HbSS or HbSβ0 thalassemia), unselected for respiratory disease, were enrolled in the Sleep and Asthma Cohort (SAC) study. Study-certified pulmonary function technicians performed spirometry and lung volumes. Each assessment was reviewed centrally. Predicted values were determined for TLC, FEV1 , FVC, and FEV1 /FVC ratio. A total of 197 participants, mean age 11.0 years at first testing (range 4-19.3 years), had a minimum of three spirometry measurements, over an average of 4.4 years (range 1.1-6.5 years) from baseline to endpoint. In a multivariable model, FEV1 % predicted declines by 0.3% for every additional year of age (95% CI -0.56 to -0.05, P = .020). Sex, asthma history, hemoglobin, reticulocyte count, white blood cell count, incidence rate of severe acute pain and acute chest syndrome episodes, and hydroxyurea therapy were not associated with a decline in FEV1 % predicted. In a large, rigorously evaluated, prospective cohort of an unselected group of children with SCA, FEV1 % predicted declines minimally over an average of 4 years, and none of the examined disease features predict the decline.

Journal Article
0361-8609
408-415
Willen, Shaina M
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Cohen, Robyn
68312680-d8a7-4a08-9e1e-2eff5e924bfe
Rodeghier, Mark
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Kirkham, Fenella
1dfbc0d5-aebe-4439-9fb2-dac6503bcd58
Redline, Susan S
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Rosen, Carol
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Kirkby, Jane
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DeBaun, Michael R
76559153-80c6-4642-bdf8-672a75570dfe
Willen, Shaina M
ca7aab8e-7548-490c-84c0-6eef8738223b
Cohen, Robyn
68312680-d8a7-4a08-9e1e-2eff5e924bfe
Rodeghier, Mark
fa844b56-b6c4-4dd5-affd-f56b0b23e24e
Kirkham, Fenella
1dfbc0d5-aebe-4439-9fb2-dac6503bcd58
Redline, Susan S
3466f7f2-aa34-4c87-b8f1-37e039f6f0bd
Rosen, Carol
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Kirkby, Jane
f7468439-d939-4b22-9728-af7671014910
DeBaun, Michael R
76559153-80c6-4642-bdf8-672a75570dfe

Willen, Shaina M, Cohen, Robyn, Rodeghier, Mark, Kirkham, Fenella, Redline, Susan S, Rosen, Carol, Kirkby, Jane and DeBaun, Michael R (2018) Age is a predictor of a small decrease in lung function in children with sickle cell anemia. American Journal of Hematology, 408-415. (doi:10.1002/ajh.25003).

Record type: Article

Abstract

The longitudinal pattern of lung function in children with sickle cell anemia (SCA) has shown a decrease in FEV1 % predicted, a risk factor for death in adults with SCA, but predictors for this decline are poorly characterized. In a prospective longitudinal multi-center cohort of children with SCA, we tested the hypotheses that: (1) FEV1 % predicted declines over time; and (2) SCA-specific characteristics and therapy predict this decline. At three clinical centers, children with SCA (HbSS or HbSβ0 thalassemia), unselected for respiratory disease, were enrolled in the Sleep and Asthma Cohort (SAC) study. Study-certified pulmonary function technicians performed spirometry and lung volumes. Each assessment was reviewed centrally. Predicted values were determined for TLC, FEV1 , FVC, and FEV1 /FVC ratio. A total of 197 participants, mean age 11.0 years at first testing (range 4-19.3 years), had a minimum of three spirometry measurements, over an average of 4.4 years (range 1.1-6.5 years) from baseline to endpoint. In a multivariable model, FEV1 % predicted declines by 0.3% for every additional year of age (95% CI -0.56 to -0.05, P = .020). Sex, asthma history, hemoglobin, reticulocyte count, white blood cell count, incidence rate of severe acute pain and acute chest syndrome episodes, and hydroxyurea therapy were not associated with a decline in FEV1 % predicted. In a large, rigorously evaluated, prospective cohort of an unselected group of children with SCA, FEV1 % predicted declines minimally over an average of 4 years, and none of the examined disease features predict the decline.

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Longitudinal_lung_manuscript_AJH_CLEAN (1) - Accepted Manuscript
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Accepted/In Press date: 7 December 2017
e-pub ahead of print date: 11 December 2017
Published date: 7 February 2018
Keywords: Journal Article

Identifiers

Local EPrints ID: 417652
URI: http://eprints.soton.ac.uk/id/eprint/417652
ISSN: 0361-8609
PURE UUID: ff656ebf-ac16-41f1-a0a5-fd25c14d195e
ORCID for Fenella Kirkham: ORCID iD orcid.org/0000-0002-2443-7958

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Date deposited: 08 Feb 2018 17:30
Last modified: 16 Mar 2024 06:10

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Contributors

Author: Shaina M Willen
Author: Robyn Cohen
Author: Mark Rodeghier
Author: Fenella Kirkham ORCID iD
Author: Susan S Redline
Author: Carol Rosen
Author: Jane Kirkby
Author: Michael R DeBaun

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