Pulse oximetry in a cohort study of sickle cell disease.

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.

Adolescent blood pressure in a cohort study of sickle cell disease.

BACKGROUND: Previous studies on low blood pressure in patients with homozygous sickle cell (SS) disease have sought new hypotheses on the mechanism of low blood pressure but have not analyzed the role of known determinants such as weight. METHODS: Blood pressure has been measured by an automated oscillometric method in 220 patients with SS disease, 144 with sickle cell-hemoglobin C disease (both groups aged, 9.5 to 18.5 years) and 122 control subjects with a normal hemoglobin genotype (aged 16.0 to 18.5 years) participating in a cohort study from birth. RESULTS: Significant age-related increases in systolic and mean arterial pressure occurred in sickle cell-hemoglobin C disease but not in SS disease. Further analyses were confined to a subgroup of 51 patients with SS, 41 patients with sickle cell-hemoglobin C, and 97 subjects with normal hemoglobin, aged 16.0 to 18.5 years in whom simultaneous measurements of height, weight, arm circumference, and hematologic test results were also available. Crude analyses showed significantly lower systolic, diastolic, and mean arterial pressure in SS disease compared with control subjects with normal hemoglobin, but further analysis showed the systolic difference to be confined to males and all differences disappeared after adjustment for weight. No differences occurred in sickle cell-hemoglobin C disease. CONCLUSIONS: These results suggest that the lower blood pressure in SS disease is attributable to low weight and that no further mechanisms need be postulated.