Fetal Hemoglobin is Associatedwith Peripheral Oxygen Saturation in Sickle Cell Disease in Tanzania

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dc.contributor.authorNkya, Siana
dc.contributor.authorMgaya, Josephine
dc.contributor.authorUrio, Florence
dc.contributor.authorMakubi, Abel
dc.contributor.authorThein, Swee L
dc.contributor.authorMenzel, Stephan
dc.contributor.authorCox, Sharon E.
dc.contributor.authorNewton, Charles R
dc.contributor.authorKirkham, Fenella J
dc.contributor.authorMmbando, Bruno P
dc.contributor.authorMakani, Julie
dc.date.accessioned2019-05-07T09:33:55Z
dc.date.available2019-05-07T09:33:55Z
dc.date.issued2017
dc.description.abstractFetal 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 laboratorymeasureswere available in a Tanzanian cohort of 1175 SCD individuals aged ≥ 5 years and the associationwith 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.en_US
dc.identifier.doi10.1016/j.ebiom.2017.08.006.
dc.identifier.urihttp://hdl.handle.net/20.500.11810/5209
dc.language.isoenen_US
dc.publisherEBioMedicineen_US
dc.subjectFetal hemoglobin (HbF) Sickle cell disease Hypoxia Oxygen saturation Reticulocytesen_US
dc.titleFetal Hemoglobin is Associatedwith Peripheral Oxygen Saturation in Sickle Cell Disease in Tanzaniaen_US
dc.typeJournal Articleen_US
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