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Published on: November 5, 2019
Sleep-disordered breathing and transcranial Dopplers in sickle cell disease
Nira A Goldstein1, Roni Keller, Kathy Rey
1Division of Pediatric Otolaryngology, State University of New York Downstate Medical Center, 450 Clarkson Avenue, Brooklyn, NY 11203, USA. ngoldstein@downstate.edu
Insights
Sleep-disordered breathing is common in children with sickle cell disease, affecting nearly a quarter of patients. However, this study found no link between sleep problems and increased cerebrovascular risk, as measured by transcranial Doppler.
Area of Science:
- Pediatric Hematology
- Sleep Medicine
- Neurology
Background:
- Sickle cell disease (SCD) is a genetic blood disorder associated with various complications.
- Sleep-disordered breathing (SDB) is increasingly recognized in children with SCD.
- Cerebrovascular complications, such as stroke, are a major concern in pediatric SCD.
Purpose of the Study:
- To determine the prevalence of SDB in children with SCD.
- To investigate the association between SDB and high-risk transcranial Doppler (TCD) velocities, a marker for cerebrovascular risk.
Main Methods:
- A cross-sectional study was conducted at a tertiary care academic medical center.
- Sixty-four children (aged 2-14 years) with SCD (genotype SS or Sβ(0)-thalassemia) and no history of stroke were enrolled.
- Sleep-disordered breathing was assessed using the Pediatric Sleep Questionnaire and overnight polysomnography for children who snored. Transcranial Doppler (TCD) ultrasonography was performed to measure blood flow velocities.
Main Results:
- The prevalence of snoring was 37.5%, positive polysomnography findings were 23.7%, and positive Pediatric Sleep Questionnaire scores were 21.9%.
- No significant differences in TCD velocities or the frequency of high-risk TCD findings were observed between children with and without SDB.
- Specifically, comparisons between nonsnorers, children with snoring but negative polysomnography, and children with snoring and positive polysomnography showed no significant differences in TCD risk (P=.91 and P=.66).
Conclusions:
- There is a high prevalence of snoring and SDB in children with sickle cell disease.
- The study's findings do not support an association between SDB and increased cerebrovascular risk (high-risk TCD velocities) in this pediatric population.
- Further research may be needed to fully understand the relationship between sleep and cerebrovascular health in children with SCD.
Objectives:
To determine the prevalence of sleep-disordered breathing in children with sickle cell disease and whether there is an association of sleep-disordered breathing with high-risk transcranial Doppler ultrasonography (TCD) velocities. Study
Design:
Cross-sectional.
Setting:
Tertiary care academic medical center.
Patients:
Sixty-four children (aged 2-14 years) selected for eligible genotype (type SS or Sβ(0)-thalassemia) and no history of stroke.
Interventions:
Parents completed the Pediatric Sleep Questionnaire. Overnight polysomnography was performed for children with snoring. The TCD was performed or existing results were obtained for all children; for children who underwent transfusion therapy, readings prior to the transfusion were analyzed. Children with abnormal or conditional TCD (flow velocity ≥170 cm/s in any vessel) were considered high risk.
Main Outcome Measures:
Prevalence of sleep-disordered breathing and TCD velocity and frequency of high-risk TCD in patients with and without sleep-disordered breathing.
Results:
The prevalence of snoring was 37.5% (95% CI, 26.7%-49.8%), the prevalence of positive polysomnography findings was 23.7% (14.6%-36.1%), and the prevalence of positive Pediatric Sleep Questionnaire scores was 21.9% (13.4%-33.6%). There was no significant difference in TCD velocity or number of patients with high-risk TCD between nonsnorers and children with snoring but negative polysomnography findings and children with snoring and positive polysomnography findings (P = .91 and P = .66, respectively) or between nonsnorers and snorers with a negative Pediatric Sleep Questionnaire score and snorers with a positive Pediatric Sleep Questionnaire score (P = .76 and P = .33, respectively).
Conclusion:
There is a high prevalence of snoring and sleep-disordered breathing among children with sickle cell disease, but our results do not support an association with cerebrovascular risk.
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