title: Clinical correlates of steady-state oxyhaemoglobin desaturation in children who have sickle cell disease.
abstract: Individuals with sickle cell disease (SCD) may have oxyhaemoglobin desaturation during the steady-state, the causes of which are incompletely known. We studied a cohort of 585 children who have sickle cell anaemia (SS), sickle beta0-thalassaemia (Sbeta0), sickle-haemoglobin C disease (SC), or sickle beta+-thalassaemia (Sbeta+) to determine the relationships between steady-state oxyhaemoglobin saturation (SpO2) and SCD genotype, age, gender, steady-state haemoglobin (Hb) and reticulocyte count, and rate of acute chest syndrome (ACS). The SS/Sbeta0 group (n = 390) had lower mean SpO2 than the SC/Sbeta+ group (n = 195) (96.3% vs. 98.7%, P < 0.001). Among SS/Sbeta0 subjects, a decrease in steady-state SpO2 correlated with a decrease in Hb, an increase in reticulocytes, older age and male gender. These correlations were not found in the SC/Sbeta+ group. Prior ACS did not correlate with steady-state SpO2. A multivariate model explained 45% of the variability in SpO2, but only 5% of the variation in SpO2 was explained by Hb. We conclude that steady-state desaturation is common in individuals with SCD, but it appears to be unrelated to prior episodes of ACS and largely unexplained by chronic anaemia.
authors.affiliation: Division of Hematology-Oncology, Department of Pediatrics, University of Texas Southwestern Medical Center at Dallas, Dallas, TX 75390-9063, USA. charles.quinn@utsouthwestern.edu
authors.forename: Charles T
authors.initials: CT
authors.lastname: Quinn
authors.forename: Naveed
authors.initials: N
authors.lastname: Ahmad
mesh_terms: D000293:Adolescent; D000367:Age Factors; D000755:Anemia, Sickle Cell / Q000097:blood*; D002648:Child; D002675:Child, Preschool; D005260:Female; D005838:Genotype; D006454:Hemoglobins / Q000378:metabolism; D006801:Humans; D008297:Male; D010092:Oximetry; D010108:Oxyhemoglobins / Q000378:metabolism*; D017701:Reticulocyte Count; D012189:Retrospective Studies; D012737:Sex Factors
chemical_list: D006454:Hemoglobins; D010108:Oxyhemoglobins