The purpose of this study of sickle cell disease (SCD) was to determine whether arteriopathy, measurable as intracranial vessel signal loss on magnetic resonance angiography (MRA), was associated with low nocturnal hemoglobin oxygen saturation (SpO 2) or hemolytic rate, measurable as reticulocytosis or unconjugated hyperbilirubinemia.
Ninety-five East London children with SCD without prior stroke had overnight pulse oximetry, of whom 47 (26 boys, 39 hemoglobin SS; mean age 9.1 ± 3.1 years) also had MRA, transcranial Doppler (TCD), steady-state hemoglobin, and reticulocytes within 34 months. Two radiologists blinded to the other data graded arteriopathy on MRA as 0 (none) or as increasing severity grades 1, 2, or 3.
Grades 2 or 3 arteriopathy (n = 24; 2 with abnormal TCD) predicted stroke/TIA compared with grades 0 and 1 (log-rank χ 2 [1, n = 47] = 8.1, p = 0.004). Mean overnight SpO 2 correlated negatively with reticulocyte percentage ( r = −0.387; p = 0.007). Despite no significant differences across the degrees of arteriopathy in genotype, mean overnight SpO 2 was higher ( p < 0.01) in those with grade 0 (97.0% ± 1.6%) than those with grades 2 (93.9 ± 3.7%) or 3 (93.5% ± 3.0%) arteriopathy. Unconjugated bilirubin was not associated but reticulocyte percentage was lower ( p < 0.001) in those with grade 0 than those with grades 2 and 3 arteriopathy. In multivariable logistic regression, lower mean overnight SpO 2 (odds ratio 0.50, 95% confidence interval 0.26–0.96; p < 0.01) predicted arteriopathy independent of reticulocyte percentage (odds ratio 1.47, 95% confidence interval 1.15–1.87; p = 0.003).
(1) Commercial Shire/ViroPharma travel to Zurich for meeting about buccal Midazolam for epilepsy (2) Commercial Eisai travel to Lyon for meeting about Eslicarbezepine, an anticonvulsant (3) Commercial Eisai travel to Helsinki for meeting about Eslicarbezepine, an anticonvulsant (4) Non-commercial European Haematology Association travel from Madrid to give an invited talk (5) Non-commercial European Haematology Association travel to Cairo to give an invited talk (6) Non-commercial American Hematology Society travel to Washington to participate in guidelines committee
(1) Commercial Shire/Viropharma re buccal Midazolam (2) Commercial Eisai re Eslicarbezepine
National Institutes for Health Research (UK) grant PB_PG_1112_29099
(1) Great Ormond Street Charity grant V4615 (2) Action Medical Research grant GN2509
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