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Upper airway genioglossal activity in children with sickle cell disease
Jingtao Huang1, Swaroop J Pinto, Julian L Allen
1Sleep Center, University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA.
Insights
Children with sickle cell disease (SCD) and obstructive sleep apnea syndrome (OSAS) show weaker airway reflexes than those without OSAS. This suggests impaired neuromuscular responses contribute to upper airway collapse in pediatric SCD patients with OSAS.
Area of Science:
- Pediatric Pulmonology
- Sleep Medicine
- Hematology
Background:
- Obstructive sleep apnea syndrome (OSAS) is more prevalent in pediatric sickle cell disease (SCD) patients.
- Not all SCD patients develop OSAS, suggesting contributing factors beyond SCD itself.
- A blunted neuromuscular response to airway pressure changes may increase susceptibility to upper airway collapse in SCD patients with OSAS.
Purpose of the Study:
- To investigate the hypothesis that SCD patients with OSAS exhibit a blunted neuromuscular response to negative airway pressure during sleep.
- To compare the upper airway reflex responses between children with SCD and OSAS versus children with SCD without OSAS.
Main Methods:
- The study involved 4 children with SCD and OSAS and 18 children with SCD without OSAS.
- Pressure-flow measurements and genioglossal electromyography (EMGgg) were recorded during sleep.
- Two techniques were used to induce subatmospheric nasal pressure, and changes in EMGgg activity were analyzed to determine the slope of EMGgg vs. nasal pressure (EMGgg-P(N)) and critical closing pressure (Pcrit).
Main Results:
- The slope of EMGgg-P(N) was more negative in SCD controls (without OSAS) than in SCD OSAS patients when using the activated technique.
- SCD controls showed a significantly greater difference in the slope of EMGgg-P(N) between activated and hypotonic techniques compared to SCD OSAS patients.
- Critical closing pressure (Pcrit) was more negative in SCD controls than SCD OSAS patients with the activated technique, indicating greater airway stability.
Conclusions:
- Children with SCD but without OSAS demonstrate more robust upper airway reflexes compared to those with SCD and OSAS.
- This suggests that impaired neuromuscular control of the upper airway plays a role in the development of OSAS in pediatric SCD patients.
Study Objectives:
The prevalence of obstructive sleep apnea syndrome (OSAS) in sickle cell disease (SCD) has been reported to be higher than that in the general pediatric population. However, not all subjects with SCD develop OSAS. We hypothesized that SCD patients with OSAS have a blunted neuromuscular response to subatmospheric pressure loads during sleep, making them more likely to develop upper airway collapse.
Design:
Subjects with SCD with and without OSAS underwent pressure-flow measurements during sleep using intraoral surface electrodes to measure genioglossal EMG (EMGgg). Two techniques were applied to decrease the nasal pressure (P(N)) to subatmospheric levels, resulting in an activated and relatively hypotonic upper airway. The area under the curve of the inspiratory EMGgg moving time average was analyzed. EMGgg activity was expressed as a percentage of baseline. Changes in EMGgg in response to decrements in nasal pressure were expressed as the slope of the EMGgg vs. nasal pressure (slope of EMGgg-P(N)).
Setting:
Sleep laboratory.
Participants:
4 children with SCD and OSAS and 18 children with SCD but without OSAS.
Results:
THE MAJOR FINDINGS OF THIS STUDY WERE: (1) using the activated but not the hypotonic technique, the slope of EMGgg-P(N) was more negative in SCD controls than SCD OSAS; (2) the slope of EMGgg-P(N) was significantly lower using the activated technique compared to the hypotonic technique in SCD controls only; (3) similarly, the critical closing pressure, Pcrit, was more negative using the activated technique than the hypotonic technique in SCD controls but not in SCD OSAS.
Conclusion:
This preliminary study has shown that children with SCD but without OSAS have more prominent upper airway reflexes than children with SCD and OSAS.
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