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European Journal of Pediatrics

, Volume 177, Issue 9, pp 1367–1370 | Cite as

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

  • Angelo Canora
  • Adriana Franzese
  • Enza Mozzillo
  • Valentina Fattorusso
  • Marialuisa Bocchino
  • Alessandro Sanduzzi
Short Communication

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.

Keywords

Prader-Willi syndrome Sleep Obstructive sleep apnoea 

Abbreviation

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

Notes

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.

Compliance with ethical standards

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.

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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Section of Respiratory Disease, Department of Clinical Medicine and Surgery, Monaldi HospitalUniversity “Federico II” NaplesNaplesItaly
  2. 2.Departments of Translational and Medical SciencesUniversity “Federico II” NaplesNaplesItaly

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