%0 Journal Article
%T Hematological and Genetic Predictors of Daytime Hemoglobin Saturation in Tanzanian Children with and without Sickle Cell Anemia
%A Sharon E. Cox
%A Julie Makani
%A Charles R. Newton
%A Andrew M. Prentice
%A Fenella J. Kirkham
%J ISRN Hematology
%D 2013
%R 10.1155/2013/472909
%X Low hemoglobin oxygen saturation (SpO2) is common in Sickle Cell Anemia (SCA) and associated with complications including stroke, although determinants remain unknown. We investigated potential hematological, genetic, and nutritional predictors of daytime SpO2 in Tanzanian children with SCA and compared them with non-SCA controls. Steady-state resting pulse oximetry, full blood count, transferrin saturation, and clinical chemistry were measured. Median daytime SpO2 was 97% (IQ range 94¨C99%) in SCA (N = 458), lower ( ) than non-SCA (median 99%, IQ range 98¨C100%; N = 394). Within SCA, associations with SpO2 were observed for hematological variables, transferrin saturation, body-mass-index z-score, hemoglobin F (HbF%), genotypes, and hemolytic markers; mean cell hemoglobin (MCH) explained most variability ( , Adj ). In non-SCA only age correlated with SpO2. -thalassemia 3.7 deletion highly correlated with decreased MCH (Pearson correlation coefficient 0.60, ). In multivariable models, lower SpO2 correlated with higher MCH ( -coefficient 0.32, ) or with decreased copies of -thalassemia 3.7 deletion ( -coefficient 1.1, ), and independently in both models with lower HbF% ( -coefficient 0.15, ) and Glucose-6-Phosphate Dehydrogenase genotype ( -coefficient 1.12, ). This study provides evidence to support the hypothesis that effects on red cell rheology are important in determining SpO2 in children with SCA. Potential mechanisms and implications are discussed. 1. Introduction Hemoglobin oxygen desaturation in the absence of acute illness is common in children with Sickle Cell Anemia (SCA), and is associated with higher cerebral blood flow velocities [1, 2], and with risk of complications including stroke [3]. The underlying mechanisms of hemoglobin oxygen desaturation in SCA are poorly understood but may involve the severity of anemia [4] as well as differences in hemoglobin oxygen affinity compared to hemoglobin A (HbA), with increased expression of 2.3 DPG in hemoglobin S (HbS) resulting in a right-shifted hemoglobin oxygen affinity curve and other differences in red cell physiology [5]. Other potential causes include a history of acute chest syndrome and reduced pulmonary [6] and cardiac function [7]. Coinheritance of alpha-thalassemia deletions and glucose-6-phosphate deficiency (G6PD) may affect the degree of anemia [8, 9] whilst alpha-thalassemia status modifies red cell indices [10¨C12] and rheology [13], as can iron status [14]. We therefore investigated potential hematological, genetic, and nutritional predictors of daytime hemoglobin oxygen saturation
%U http://www.hindawi.com/journals/isrn.hematology/2013/472909/