Case-control study of heart rate variability and sleep apnea in childhood sickle cell disease

Plamen Bokov1, Bérengère Koehl2,3, Benjamin Dudoignon1

  • 1AP-HP, Robert Debré Hospital, Department of Physiology, INSERM NeuroDiderot, Paris University, Paris, France.

PubMed

Insights

Children with sickle cell disease (SCD) and obstructive sleep apnea (OSA) show unique heart rate variability (HRV) and pharyngeal compliance patterns. Decreased sympathetic modulation appears protective in SCD, unlike in healthy children.

Area of Science:

  • Pediatric Pulmonology
  • Cardiology
  • Genetics

Background:

  • Obstructive sleep apnea (OSA) is prevalent in sickle cell disease (SCD), often without typical risk factors like obesity.
  • Previous research indicated increased pharyngeal compliance correlates with decreased sympathetic modulation in healthy children with OSA.
  • The specific pathophysiology of OSA in SCD requires further investigation, particularly concerning autonomic nervous system function and airway mechanics.

Purpose of the Study:

  • To investigate the association between heart rate variability (HRV) and pharyngeal compliance in children with SCD and OSA.
  • To compare these associations between children with SCD and healthy controls.

Main Methods:

  • A case-control study matched 21 children with SCD and 21 healthy controls (aged 4-18 years) for sex, age, and obstructive apnea-hypopnoea index (OAHI).
  • Polysomnography and acoustic pharyngometry were used to assess sleep apnea, pharyngeal compliance, and HRV from ECG recordings during different sleep states and whole night.
  • Statistical analyses explored relationships between compliance and HRV parameters, specifically SD2, a marker of sympathetic modulation.

Main Results:

  • Children with SCD had lower BMI z-scores and more tonsil hypertrophy than controls.
  • Children with SCD and OSA exhibited lower pharyngeal compliance compared to those without OSA.
  • An inverse relationship between pharyngeal compliance and SD2 (sympathetic modulation) was observed in SCD (R = -0.63, p = 0.002), contrasting with a positive relationship in controls (R = 0.59, p = 0.006).

Conclusions:

  • Pharyngeal compliance and HRV show distinct associations in children with SCD compared to healthy children.
  • Decreased sympathetic modulation may be protective against OSA in SCD, suggesting a unique pathophysiology possibly linked to smooth muscle dysfunction.
  • These findings highlight the need for tailored approaches to managing OSA in pediatric SCD patients.

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