Clinical correlates of steady-state oxyhaemoglobin desaturation in children who have sickle cell disease

Br J Haematol. 2005 Oct;131(1):129-34. doi: 10.1111/j.1365-2141.2005.05738.x.


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.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Adolescent
  • Age Factors
  • Anemia, Sickle Cell / blood*
  • Child
  • Child, Preschool
  • Female
  • Genotype
  • Hemoglobins / metabolism
  • Humans
  • Male
  • Oximetry
  • Oxyhemoglobins / metabolism*
  • Reticulocyte Count
  • Retrospective Studies
  • Sex Factors


  • Hemoglobins
  • Oxyhemoglobins