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Clinical correlates of steady‐state oxyhaemoglobin desaturation in children who have sickle cell disease
Author(s) -
Quinn Charles T.,
Ahmad Naveed
Publication year - 2005
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/j.1365-2141.2005.05738.x
Subject(s) - acute chest syndrome , sickle cell anemia , medicine , steady state (chemistry) , hemoglobinopathy , cohort , disease , vaso occlusive crisis , gastroenterology , pediatrics , chemistry
Summary 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 β 0 ‐thalassaemia (S β 0 ), sickle‐haemoglobin C disease (SC), or sickle β + ‐thalassaemia (S β + ) to determine the relationships between steady‐state oxyhaemoglobin saturation (SpO 2 ) and SCD genotype, age, gender, steady‐state haemoglobin (Hb) and reticulocyte count, and rate of acute chest syndrome (ACS). The SS/S β 0 group ( n = 390) had lower mean SpO 2 than the SC/S β + group ( n = 195) (96·3% vs. 98·7%, P < 0·001). Among SS/S β 0 subjects, a decrease in steady‐state SpO 2 correlated with a decrease in Hb, an increase in reticulocytes, older age and male gender. These correlations were not found in the SC/S β + group. Prior ACS did not correlate with steady‐state SpO 2 . A multivariate model explained 45% of the variability in SpO 2 , but only 5% of the variation in SpO 2 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.