Clinical and laboratory observationDaytime pulse oximeter measurements do not predict incidence of pain and acute chest syndrome episodes in sickle cell anemia
Section snippets
Patient Population
The Cooperative Study for Sickle Cell Disease (CSSCD) study design has been reported previously.6, 7 A total of 151 African American children from the CSSCD with hemoglobin SS (HbSS) were enrolled in the study before age 6-months and had pulse oximetry data available for review. A total of 21 subjects had a missing pulse oximetry reading, and/or pain or ACS event. Hence, 86% (130 of 151) children were included in the study cohort for the final analysis. Subjects with HbSS were enrolled in this
Demographics
A total of 130 participants met criteria and were included in the study. The cohort had a mean age of 9.8 years (range, 4.8 to 15.7 years) and included 65 males (50%). The mean total follow-up was 13.0 years. The mean and median follow-up after SpO2 measurement were 3.5 years and 3.1 years, respectively. The mean PRE-SpO2 follow-up was 9.5 years; the median was 9.2 years. The mean and median SpO2 in the cohort were 94.1% and 95%, respectively, with a range of 75% to 100% (Figure).
No Association Between SpO2 Measurement and Pain Rate
During
Discussion
Our results showed no correlation between SpO2 and the subsequent sickle cell disease related morbidity. These findings are similar to those of Homi et al.2 In addition, no specific daytime SpO2 threshold was associated with higher incidence of pain or acute chest syndrome episodes.
Recurrent ACS episodes have been implicated as a cause of low baseline SpO2. Rackoff,8 in a study of 86 children with HbSS, determined that a history of ACS and age greater than 5 years were both associated with
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Cited by (21)
Early initiation of inhaled corticosteroids does not decrease acute chest syndrome morbidity in pediatric patients with sickle cell disease
2018, Blood Cells, Molecules, and DiseasesCitation Excerpt :Murine models of SCD have shown steady state differences in the lungs compared to control mice including higher markers of vascular injury and inflammatory cells that increase upon exposure to hypoxia [17]. Clinically, steady state pulse oxygen saturation in children [18] and adults [19] with SCD is reduced, resting and exertional alveolar-arterial oxygen gradient is widened [19], and patients with SCD but without asthma have a higher prevalence of airway hyper-reactivity/-responsiveness than ethnically matched controls [20]. The overlap between intrinsic lung disease in SCD and ACS symptoms, with asthma physiology and acute asthma exacerbations, makes a formal diagnosis of asthma difficult in patients with SCD.
A systematic review of the literature for severity predictors in children with sickle cell anemia
2017, Blood Cells, Molecules, and DiseasesCitation Excerpt :Sibling history of asthma was also found to be associated with a higher pain rate in the CSSCD newborn cohort [44]. Pulse oximetry levels, however, were not associated with the number of VOE or ACS in the newborn cohort of the CSSCD [45]. Polymorphisms of bilirubin UDP gluronosyltransferase 1A1 (UGT1A1) and genes associated with vascular disease were performed on banked samples from patients enrolled in the CSSCD.
Recommendations for long-term home oxygen therapy in children and adolescents
2013, Jornal de PediatriaQuestions in the management of sickle cell
2006, Journal of PediatricsNocturnal hypoxemia measured by polysomnogram is associated with acute chest syndrome in pediatric sickle cell disease
2021, Journal of Clinical Sleep Medicine
The Cooperative Study of Sickle Cell Disease was conducted and supported by the NHLBI in collaboration with site investigators. This manuscript was not prepared in collaboration with investigators of the Cooperative Study of Sickle Cell Disease and does not necessarily reflect the opinions or views of the Cooperative Study of Sickle Cell Disease or NHLBI.
Funded in part by the Doris Duke Charitable Foundation and by the National Institutes of Health, National Heart, Lung and Blood Institute (NHLBI) (grants NO1-HB47099, NO1-HB47110, and RO1-HL79937).