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Published on: June 14, 2020
Upper airway lymphoid tissue size in children with sickle cell disease
Temima Strauss1, Sanghun Sin1, Carole L Marcus2
1Division of Respiratory and Sleep Medicine, The Children's Hospital at Montefiore and Albert Einstein College of Medicine, Bronx, NY.
Insights
Children with sickle cell disease (SCD) have smaller upper airways due to enlarged lymphoid tissues, increasing their risk for obstructive sleep apnea syndrome (OSAS). This study highlights a key factor contributing to OSAS in pediatric SCD patients.
Area of Science:
- Pediatric Pulmonology
- Hematology
- Medical Imaging
Background:
- Obstructive sleep apnea syndrome (OSAS) is more prevalent in children with sickle cell disease (SCD) than in the general pediatric population.
- Enlarged adenoids and tonsils are suspected contributors to OSAS in children with SCD.
Purpose of the Study:
- To investigate the association between lymphoid tissue size and upper airway dimensions in children with SCD.
- To evaluate the prevalence of OSAS in children with SCD and its correlation with upper airway characteristics.
Main Methods:
- Magnetic Resonance Imaging (MRI) was used to assess upper airway and lymphoid tissue volumes in 36 children with SCD and 36 age-matched controls.
- Polysomnography was performed to diagnose OSAS and evaluate respiratory parameters.
Main Results:
- Children with SCD exhibited significantly smaller upper airway volumes and larger adenoid, tonsil, and lymph node sizes compared to controls.
- OSAS was diagnosed in 19.4% of children with SCD, with a positive correlation between apnea-hypopnea index and upper airway lymphoid tissue size.
- Children with SCD showed lower oxygen saturation nadir, higher peak end-tidal CO2, and increased arousals.
Conclusions:
- Children with SCD have reduced upper airway size, primarily due to lymphoid tissue overgrowth.
- This anatomical predisposition likely explains the increased susceptibility to OSAS in pediatric SCD patients.
Background:
The prevalence of obstructive sleep apnea syndrome (OSAS) is higher in children with sickle cell disease (SCD) as compared with the general pediatric population. It has been speculated that overgrowth of the adenoid and tonsils is an important contributor.
Methods:
The current study used MRI to evaluate such an association. We studied 36 subjects with SCD (aged 6.9 ± 4.3 years) and 36 control subjects (aged 6.6 ± 3.4 years).
Results:
Compared with control subjects, children with SCD had a significantly smaller upper airway (2.8 ± 1.2 cm(3) vs 3.7 ± 1.6 cm(3), P < .01), and significantly larger adenoid (8.4 ± 4.1 cm(3) vs 6.0 ± 2.2 cm(3), P < .01), tonsils (7.0 ± 4.3 cm(3) vs 5.1 ± 1.9 cm(3), P < .01), retropharyngeal nodes (3.0 ± 1.9 cm(3) vs 2.2 ± 0.9 cm(3), P < .05), and deep cervical nodes (15.7 ± 5.7 cm(3) vs 12.7 ± 4.0 cm(3), P < .05). Polysomnography showed that 19.4% (seven of 36) of children with SCD had OSAS compared with 0% (zero of 20) of control subjects (P < .05) and that in children with SCD the apnea-hypopnea index correlated positively with upper airway lymphoid tissues size (r = 0.57, P < 001). In addition, children with SCD had lower arterial oxygen saturation nadir (84.3% ± 12.3% vs 91.2% ± 4.2%, P < .05), increased peak end-tidal CO(2) (53.4 ± 8.5 mm Hg vs 42.3 ± 5.3 mm Hg, P < .001), and increased arousals (13.7 ± 4.7 events/h vs 10.8 ± 3.8 events/h, P < .05).
Conclusions:
Children with SCD have reduced upper airway size due to overgrowth of the surrounding lymphoid tissues, which may explain their predisposition to OSAS.
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