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Severe obstructive sleep disorders in Prader-Willi syndrome patients in southern Italy

Abstract

Sleep-related disordered breathing (SDB) is very common in paediatric patients affected by Prader-Willi Syndrome (PWS). However, data addressing SBD patterns and their management are lacking. The aim of the present study was to analyse SDB features in 14 PWS patients (age range, 8 months-17 years). Polygraphic registration (PG) during a 12-h nocturnal sleep was performed in all patients. Obstructive and central apnoea indices and oxygen saturation (SpO2) were recorded along with demographic and clinical data. Obstructive sleep apnoea (OSA) was diagnosed in 13/14 patients (92.9%); the mean obstructive apnoea-hypopnea index (OAHI) was 7.6 ± 4.2 events/h with a mean central apnoea index (CAI) of 0.7 ± 1.04 events/h. Time spent with SpO2 < 90% was of 0.02% [range 0–23%], with a mean oxygen desaturation index of 12.1 ± 6.9 events/h. No correlation was found between OAHI and body mass index (mean BMI 28 ± 9.8 kg/m2 and BMI z-score 2.7 ± 1.7).

Conclusion: OSA was the predominant sleep-related disorder in our PWS patients, not associated with age or obesity, and appeared more severe than previously reported. Further studies addressing the underlying mechanisms are necessary in larger study populations to better design the most appropriate clinical approach.

What is Known:
Sleep-related patterns and their management are very limited in patients with Prader-Willi syndrome.
What is New:
Severe obstructive sleep apnoea is the most frequent sleep-related disorder in our case series.

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Abbreviations

BMI:

Body mass index

CAI:

Central apnoea index

CSA:

Central sleep apnoea

GH:

Growth hormone

OAHI:

Obstructive apnoea-hypopnea index

OSA:

Obstructive sleep apnoea

PG:

Polygraphic registration

PWS:

Prader-Willi syndrome

SDB:

Sleep-disordered breathing

SpO2 :

Oxygen saturation

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Authors

Contributions

All coauthors attended the initial meeting and participated in the discussion of topics and in the design of the consensus paper. AC wrote the initial draft of the manuscript which was then circulated to all coauthors. Comments and annotations of all coauthors were included in subsequent drafts. The final version of the manuscript was approved by all coauthors.

Corresponding author

Correspondence to Angelo Canora.

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Conflict of interest

The authors declare that they have no conflict of interest.

Ethical approval

All procedures performed in this study were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

Statement of informed consent

The participants/their caregivers gave informed consent.

Additional information

Communicated by Peter de Winter

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Cite this article

Canora, A., Franzese, A., Mozzillo, E. et al. Severe obstructive sleep disorders in Prader-Willi syndrome patients in southern Italy. Eur J Pediatr 177, 1367–1370 (2018). https://doi.org/10.1007/s00431-017-3083-9

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Keywords

  • Prader-Willi syndrome
  • Sleep
  • Obstructive sleep apnoea