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Published on: October 22, 2016
Hypoventilation disproportionate to OSAS severity in children with Prader-Willi syndrome
Francois Abel1, Hui-Leng Tan2, Valentina Negro2
1Department of Paediatric Respiratory Medicine, Great Ormond Street Hospital, London, UK.
Insights
Children with Prader-Willi syndrome (PWS) and obstructive sleep apnea (OSA) experience longer periods of hypercapnia during sleep. This occurs disproportionately with increasing severity of airway obstruction compared to non-syndromic children.
Area of Science:
- Pediatric Pulmonology
- Sleep Medicine
- Genetics
Background:
- Prader-Willi syndrome (PWS) is a complex genetic disorder.
- Obstructive sleep apnea syndrome (OSAS) is common in children with PWS.
- Hypercapnia during sleep is a concern in pediatric OSAS.
Purpose of the Study:
- To investigate if children with PWS and OSAS have higher proportions of total sleep time (TST) with hypercapnia.
- To compare children with PWS and OSAS to non-syndromic children with similar obstructive apnea-hypopnea index (OAHI).
Main Methods:
- A cross-sectional study was conducted in two tertiary care hospitals.
- 48 children with PWS and 92 controls with snoring underwent polygraphy.
- Transcutaneous CO2 (PtcCO2) levels and %TST with PtcCO2 >50 mmHg were analyzed.
- Multiple linear regression tested the interaction between PWS, OSAS severity, and hypoventilation duration.
Main Results:
- No significant difference in %TST with PtcCO2 >50 mmHg was found between the two groups overall.
- A significant interaction (p<0.01) was observed between PWS and OSAS severity regarding hypoventilation duration.
- Children with PWS showed disproportionately longer %TST with PtcCO2 >50 mmHg as OSAS severity increased.
Conclusions:
- Increasing upper airway obstruction in children with PWS is associated with longer periods of hypoventilation.
- This effect is more pronounced in PWS patients compared to non-syndromic children with similar obstructive events.
- Findings highlight a specific vulnerability in PWS patients regarding sleep-related breathing.
Objective:
To test the hypothesis that children with Prader-Willi syndrome (PWS) and obstructive sleep apnoea syndrome (OSAS) have hypercapnia for higher proportion of total sleep time (TST) than non-syndromic children with similar obstructive apnoea-hypopnoea index (OAHI).
Design:
Cross-sectional study.
Setting:
Two tertiary care hospitals.
Patients:
Patients with PWS and non-syndromic children with snoring who underwent polygraphy and were of similar age, body mass index (BMI) z-score and OAHI.
Main Outcome Measure:
The two groups were compared regarding %TST with transcutaneous CO2 (PtcCO2) >50 mm Hg. The interaction between PWS diagnosis and OSAS severity (OAHI <1 episode/h vs 1-5 episodes/h vs >5 episodes/h) regarding %TST with PtcCO2 >50 mm Hg was tested using multiple linear regression.
Results:
48 children with PWS and 92 controls were included (median age 2.3 (range 0.2-14.1) years vs 2.2 (0.3-15.1) years; BMI z-score 0.7±1.9 vs 0.8±1.7; median OAHI 0.5 (0-29.5) episodes/h vs 0.5 (0-33.9) episodes/h; p>0.05). The two groups did not differ in %TST with PtcCO2 >50 mm Hg (median 0% (0-100%) vs 0% (0-81.3%), respectively; p>0.05). However, the interaction between PWS and OSAS severity with respect to duration of hypoventilation was significant (p<0.01); the estimated mean differences of %TST with PtcCO2 >50 mm Hg between children with PWS and controls for OAHI <1 episode/h, 1-5 episodes/h and >5 episodes/h were +0.2%, +1% and +33%, respectively.
Conclusion:
Increasing severity of upper airway obstruction during sleep in children with PWS is accompanied by disproportionately longer periods of hypoventilation when compared with non-syndromic children with similar frequency of obstructive events.
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