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Published on: November 5, 2019
Sleep pathology characterization in sickle cell disease: case-control study
Maria Inês Mascarenhas1, Helena Cristina Loureiro, Teresa Ferreira
1Pediatric Department, Hospital Prof. Doutor Fernando Fonseca, Lisbon, Portugal; Sickle Cell Disease Pediatric Group Pediatric Department, Hospital Prof. Doutor Fernando Fonseca, Lisbon, Portugal.
Insights
Children with sickle cell disease (SCD) experience lower minimum blood oxygen levels during sleep compared to peers, despite similar sleep apnea severity. Sleep evaluations are crucial for preventing complications in these children.
Area of Science:
- Pediatric Sleep Medicine
- Hematology
- Respiratory Medicine
Background:
- Children and adolescents with sickle cell disease (SCD) exhibit a higher prevalence of sleep disorders, including obstructive sleep apnea syndrome (OSAS).
- Nocturnal hypoxemia in SCD patients poses risks for vaso-occlusive crises and other disease-related morbidities.
- Understanding sleep disturbances in SCD is critical for comprehensive patient management.
Purpose of the Study:
- To compare polysomnography (PSG) findings in children with SCD against a control group of children with suspected OSAS but without SCD.
- To identify specific sleep-related differences between pediatric SCD patients and a matched control group.
Main Methods:
- A retrospective study design was employed, comparing clinical and PSG parameters between two groups of children.
- Statistical analysis included descriptive statistics and t-tests, with a significance level set at P < 0.05.
- The study involved 65 children with SCD and 65 age- and gender-matched controls with suspected OSAS.
Main Results:
- No significant differences were observed in sleep architecture, including sleep phase percentages, sleep efficiency, and sleep latency between the SCD and control groups.
- Children with SCD demonstrated significantly lower mean SpO2 and minimum SpO2 levels compared to controls (P < 0.01).
- Enuresis was reported significantly more frequently in the SCD group (35.4%) than in the control group (6.2%, P < 0.01).
Conclusions:
- Sleep architecture is comparable between children with and without SCD, but minimum SpO2 is significantly reduced in SCD patients.
- Despite similar apnea-hypopnea index (AHI) values, lower SpO2 in SCD children highlights a critical issue requiring attention.
- Routine sleep evaluation is essential for SCD children to prevent associated complications and comorbidities.
Background:
Children and adolescents with sickle cell disease (SCD) have a higher incidence of sleep pathology and obstructive sleep apnea syndrome (OSAS). The nocturnal hypoxemia is a risk to vaso-occlusive crisis among other SCD morbidities. Our aim was to compare polysomnography (PSG) results in a sample of children with SCD with a sample of children with suspected OSAS without SCD.
Design And Methods:
A retrospective study compared clinical and PSG parameters. A descriptive analysis and t-test were done considering P < 0.05 as significant.
Results:
PSG was done in 65 children with SCD and 65 control-children. Control sample was selected to be equal to SCD sample considering gender (53.8% were male), age (mean age was 9.4 years (SD ± 4.6) and AHI (mean 3.57 events/hr). Mean efficiency, latency and percentage of sleep phases in both groups showed no statistically significant differences. Mean SpO2 and minimum SpO2 were lower in SCD group and it was statistically significant (P < 0.01). Enuresis was more frequent in the SCD children group (35.4% vs. 6.2%, P < 0.01).
Conclusion:
Comparing children with and without SCD, sleep architecture was similar in both groups and minimum SpO2 was significantly lower in SCD children although both groups had a similar AHI. This is an important issue in these children, so it is essential to have a sleep evaluation in order to prevent complications and co-morbidities.

