Fetal Hemoglobin is Associated with Peripheral Oxygen Saturation in Sickle Cell Disease in Tanzania

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

doi:10.1016/j.ebiom.2017.08.006

Fetal Hemoglobin is Associated with Peripheral Oxygen Saturation in Sickle Cell Disease in Tanzania

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

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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 ≥ 5 years 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.

Original language	English
Journal	EBioMedicine
Early online date	8 Aug 2017
DOIs	https://doi.org/10.1016/j.ebiom.2017.08.006
Publication status	E-pub ahead of print - 8 Aug 2017

Keywords

Fetal hemoglobin (HbF)
Sickle cell disease
Hypoxia
Oxygen saturation
Reticulocytes

UN SDGs

This output contributes to the following UN Sustainable Development Goals (SDGs)

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Fetal Hemoglobin is Associated_NKYA_Publishedonline8August2017_GOLD AAM (CC BY-NC-ND)Accepted author manuscript, 628 KBLicence: CC BY-NC-ND

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@article{a5ccf515cad2401cbaa73f4d40d8a42e,

title = "Fetal Hemoglobin is Associated with Peripheral Oxygen Saturation in Sickle Cell Disease in Tanzania",

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 ≥ 5 years 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.",

keywords = "Fetal hemoglobin (HbF), Sickle cell disease, Hypoxia, Oxygen saturation, Reticulocytes",

author = "Siana Nkya and Josephine Mgaya and Florence Urio and Abel Makubi and Thein, {Swee Lay} and Stephan Menzel and Cox, {Sharon E.} and Newton, {Charles R.} and Kirkham, {Fenella J.} and Mmbando, {Bruno P.} and Julie Makani",

year = "2017",

month = aug,

day = "8",

doi = "10.1016/j.ebiom.2017.08.006",

language = "English",

journal = "EBioMedicine",

issn = "2352-3964",

publisher = "Elsevier BV",

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TY - JOUR

T1 - Fetal Hemoglobin is Associated with Peripheral Oxygen Saturation in Sickle Cell Disease in Tanzania

AU - Nkya, Siana

AU - Mgaya, Josephine

AU - Urio, Florence

AU - Makubi, Abel

AU - Thein, Swee Lay

AU - Menzel, Stephan

AU - Cox, Sharon E.

AU - Newton, Charles R.

AU - Kirkham, Fenella J.

AU - Mmbando, Bruno P.

AU - Makani, Julie

PY - 2017/8/8

Y1 - 2017/8/8

N2 - 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 ≥ 5 years 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.

AB - 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 ≥ 5 years 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.

KW - Fetal hemoglobin (HbF)

KW - Sickle cell disease

KW - Hypoxia

KW - Oxygen saturation

KW - Reticulocytes

UR - http://www.scopus.com/inward/record.url?scp=85028343172&partnerID=8YFLogxK

U2 - 10.1016/j.ebiom.2017.08.006

DO - 10.1016/j.ebiom.2017.08.006

M3 - Article

SN - 2352-3964

JO - EBioMedicine

JF - EBioMedicine

ER -

Fetal Hemoglobin is Associated with Peripheral Oxygen Saturation in Sickle Cell Disease in Tanzania

Abstract

Keywords

UN SDGs

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