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Fetal hemoglobin is associated with peripheral oxygen saturation in sickle cell disease in Tanzania

Fetal hemoglobin is associated with peripheral oxygen saturation in sickle cell disease in Tanzania
Fetal hemoglobin is associated with peripheral oxygen saturation in sickle cell disease in Tanzania

Fetal hemoglobin (HbF) and peripheral hemoglobin oxygen saturation (SpO2) both predict clinical severity in sickle cell disease (SCD), while reticulocytosis is associated with vasculopathy, but there are few data on mechanisms. HbF, SpO2 and routine clinical and laboratory measures were available in a Tanzanian cohort of 1175 SCD individuals aged≥5years and the association with SpO2 (as response variable transformed to a Poisson distribution) was assessed by negative binomial model with age and sex as covariates. Increase in HbF was associated with increased SpO2 (rate ratio, RR=1.19; 95% confidence intervals [CI] 1.04, 1.37 per natural log unit of HbF; p=0.0004). In univariable analysis, SpO2 was inversely associated with age, reticulocyte count, and log (total bilirubin) and directly with pulse, SBP, hemoglobin, and log(HbF). In multivariable regression log(HbF) (RR 1.191; 95%CI 1.04, 1.37; p=0.013), pulse (RR 1.01; 95%CI 1.00, 1.01; p=0.026), SBP (RR 1.008; 95%CI 1.00, 1.02; p=0.014), and hemoglobin (1.120; 95%CI 1.05, 1.19; p=0.001) were positively and independently associated with SpO2 while reticulocyte count (RR 0.985; 95%CI 0.97, 0.99; p=0.019) was independently inversely associated with SpO2. In SCD, improving SpO2, in part through cardiovascular compensation and associated with reduced reticulocytosis, may be a mechanism by which HbF reduces disease severity.

Journal Article
146-149
Nkya, Siana
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Mgaya, Josephine
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Urio, Florence
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Makubi, Abel
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Thein, Swee Lay
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Menzel, Stephan
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Cox, Sharon E
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Newton, Charles R
ea661613-9a2d-4e14-8d04-2d1c0804a321
Kirkham, Fenella J
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Mmbando, Bruno P
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Makani, Julie
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Nkya, Siana
e31cd803-e327-4cfd-a2f2-63655be855e7
Mgaya, Josephine
740b5114-40dc-4020-bbe0-c54475f34328
Urio, Florence
2147f58c-7cac-45f9-99f5-4dd8b0e14a90
Makubi, Abel
a590c8b2-00de-4f50-867f-c7adb1c23e38
Thein, Swee Lay
496d02c6-084c-4778-ba43-abbcc667e0da
Menzel, Stephan
5c8f3f55-d218-4f7f-8ef2-0fd0ea13d44c
Cox, Sharon E
78996c1a-4d0f-46e1-8357-429a2f04cdfe
Newton, Charles R
ea661613-9a2d-4e14-8d04-2d1c0804a321
Kirkham, Fenella J
1dfbc0d5-aebe-4439-9fb2-dac6503bcd58
Mmbando, Bruno P
204e8234-af85-45e7-842c-9184f50a0122
Makani, Julie
76a145a7-02fc-43ea-a1df-3a52d2004e48

Nkya, Siana, Mgaya, Josephine, Urio, Florence, Makubi, Abel, Thein, Swee Lay, Menzel, Stephan, Cox, Sharon E, Newton, Charles R, Kirkham, Fenella J, Mmbando, Bruno P and Makani, Julie (2017) Fetal hemoglobin is associated with peripheral oxygen saturation in sickle cell disease in Tanzania. EBioMedicine, 23, 146-149. (doi:10.1016/j.ebiom.2017.08.006).

Record type: Article

Abstract

Fetal hemoglobin (HbF) and peripheral hemoglobin oxygen saturation (SpO2) both predict clinical severity in sickle cell disease (SCD), while reticulocytosis is associated with vasculopathy, but there are few data on mechanisms. HbF, SpO2 and routine clinical and laboratory measures were available in a Tanzanian cohort of 1175 SCD individuals aged≥5years and the association with SpO2 (as response variable transformed to a Poisson distribution) was assessed by negative binomial model with age and sex as covariates. Increase in HbF was associated with increased SpO2 (rate ratio, RR=1.19; 95% confidence intervals [CI] 1.04, 1.37 per natural log unit of HbF; p=0.0004). In univariable analysis, SpO2 was inversely associated with age, reticulocyte count, and log (total bilirubin) and directly with pulse, SBP, hemoglobin, and log(HbF). In multivariable regression log(HbF) (RR 1.191; 95%CI 1.04, 1.37; p=0.013), pulse (RR 1.01; 95%CI 1.00, 1.01; p=0.026), SBP (RR 1.008; 95%CI 1.00, 1.02; p=0.014), and hemoglobin (1.120; 95%CI 1.05, 1.19; p=0.001) were positively and independently associated with SpO2 while reticulocyte count (RR 0.985; 95%CI 0.97, 0.99; p=0.019) was independently inversely associated with SpO2. In SCD, improving SpO2, in part through cardiovascular compensation and associated with reduced reticulocytosis, may be a mechanism by which HbF reduces disease severity.

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Accepted/In Press date: 5 August 2017
e-pub ahead of print date: 8 August 2017
Additional Information: Copyright © 2017. Published by Elsevier B.V.
Keywords: Journal Article

Identifiers

Local EPrints ID: 417575
URI: https://eprints.soton.ac.uk/id/eprint/417575
PURE UUID: 00e9a470-5875-46e4-80f2-a359b9010008
ORCID for Fenella J Kirkham: ORCID iD orcid.org/0000-0002-2443-7958

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Date deposited: 05 Feb 2018 17:30
Last modified: 19 Nov 2019 01:51

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Contributors

Author: Siana Nkya
Author: Josephine Mgaya
Author: Florence Urio
Author: Abel Makubi
Author: Swee Lay Thein
Author: Stephan Menzel
Author: Sharon E Cox
Author: Charles R Newton
Author: Fenella J Kirkham ORCID iD
Author: Bruno P Mmbando
Author: Julie Makani

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