الفهرس 
S ICKLE C ELL D ISEASE IN P EDIATRICSOnly 14 pages are availabe for public view
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Abstract
Abstract
Background: Sleep disordered breathing (SDB), a common underdiagnosed sequela of sickle cell disease (SCD) has been linked to the frequency of vaso-occlusive crises, cardiac abnormalities and central nervous system events. Objective: To determine the frequency of SDB in patients with SCD and its association to SCD-related complications. Methods: Thirty children and adolescents with SCD were evaluated using overnight polysomnography after completing the modified STOP-Bang questionnaire. Transcranial doppler (TCD) and assessment of tricuspid regurgitation velocity (TRV) were performed. Results: The mean age of the studied cohort was 10.2 years, with male: female ratio 1.7:1. Six patients (20%) had high-risk for obstructive sleep apnea (OSA), while nine (30%) were at intermediate-risk. Sleep apnea defined as apnea (AHI) > 1 event/hour was found among 18/30 (60%) subjects (14 males and 4 females). Patients with AHI > 5 (moderate to severe OSA) had significantly higher TRV (p = 0.007) and left MCA flow velocity (p = 0.049) when compared to those with AHI < 5. Patients with AHI > 5 were at higher risk of OSA according to the modified STOP-Bang questionnaire (p = 0.02). AHI positively correlated with TRV (r = 0.53, p = 0.003), right MCA flow velocity (r = 0.45, p = 0.013) and left MCA flow velocity (r = 0.55, p = 0.002), and negatively correlated to BMI-SDS (r = -0.48, p = 0.008). Conclusion: Sleep apnea in patients with SCD is associated with risks of pulmonary hypertension and cerebral blood flow alterations which highlights the need for early diagnosis and management.