title: Pulse oximetry in a cohort study of sickle cell disease.
abstract: Oxygen saturation was determined by pulse oximetry in a representative sample of Jamaican patients with steady-state sickle cell disease in a cohort study from birth. There were 220 with homozygous sickle cell (SS) disease and 142 with sickle cell-haemoglobin C (SC) disease aged 9-18 years, and 122 with a normal haemoglobin (AA) genotype aged 15-18 years. Pulse oximetry (SpO2) values were lower in SS disease (mean [95% confidence interval], 92.5 [92.0-93.0]) than in SC disease (96.7[96.5-96.9]) or AA controls (97.1 [96.8-97.3]). Inhalation of 100% oxygen in SS patients with O2 saturations below 90% consistently increased saturation to 99-100%. In SS disease, SpO2 correlated positively with haemoglobin and fetal haemoglobin and negatively with reticulocyte counts but not with MCHC, MCV or bilirubin level. Mean SpO2 in SS subjects with a normal alpha globin gene complement (mean [SD], 91.7 [3.9]%) was lower than in heterozygotes (93.4 [4.0]%) or homozygotes (96.1 [3.0]%) for alpha+ thalassaemia, the effects of alpha-thalassaemia not being explained by differences in haemoglobin or MCHC. In SS disease, SpO2 levels were not associated with age (within this age range), sex, number of sick clinic visits or number of hospital admissions. Higher SpO2 levels were associated with greater height and weight, more frequent painful crises and less frequent acute chest syndrome, but these associations were not significant after adjustment for haemoglobin level. Desaturation is common in steady-state SS disease and knowledge of the individual's steady-state value may be important in the interpreting low values during acute complications.
authors.affiliation: Medical Research Council Laboratories (Jamaica), University of the West Indies, Kingston, Jamaica.
authors.forename: J
authors.initials: J
authors.lastname: Homi
authors.forename: L
authors.initials: L
authors.lastname: Levee
authors.forename: D
authors.initials: D
authors.lastname: Higgs
authors.forename: P
authors.initials: P
authors.lastname: Thomas
authors.forename: G
authors.initials: G
authors.lastname: Serjeant
mesh_terms: D000293:Adolescent; D000367:Age Factors; D000755:Anemia, Sickle Cell / Q000097:blood* / Q000378:metabolism*; D002561:Cerebrovascular Disorders / Q000097:blood / Q000378:metabolism; D002637:Chest Pain / Q000097:blood / Q000378:metabolism; D002648:Child; D015331:Cohort Studies; D005260:Female; D005838:Genotype; D006128:Growth / Q000502:physiology; D006801:Humans; D007361:Intelligence Tests; D008297:Male; D010092:Oximetry / Q000592:standards / Q000706:statistics & numerical data; D010100:Oxygen / Q000008:administration & dosage / Q000097:blood; D012016:Reference Values; D015203:Reproducibility of Results; D012720:Severity of Illness Index; D012737:Sex Factors
chemical_list: D010100:Oxygen