BSPED2024 Poster Presentations Obesity 1 (7 abstracts)
Retrospective review of sleep disordered breathing in children with prader-willi syndrome
Maryam Sahibqran 1,2 , Angela Lucas-Herald 1 , Rebecca Lennon 2 , Elise Buchanan 2 , Paul Burns 2 , Phil Davies 2 , Ross Langley 2 & M. Guftar. Shaikh 1
1Developmental Endocrinology Research Group, Royal Hospital for Children, Glasgow, United Kingdom; 2Department of Respiratory Medicine, Hospital for Children, Glasgow, United Kingdom
Aim: Longitudinal study of the prevalence of sleep-disordered breathing in children with Prader-Will Syndrome (PWS) on Growth hormone (GH) therapy.
Background: Growth Hormone (GH) therapy is routinely used in the management of children with Prader-Willi Syndrome (PWS) to improve growth and body composition. However, sleep disordered breathing (SDB) may be a consequence of GH use. The aim of this study was to determine the prevalence of SDB in children with PWS.
Methods: A retrospective study was undertaken of children with PWS aged 0–18 years who had sleep studies between September 2011 – May 2024. Data was collated on patient demographics, IGF-1 levels, GH doses, Non-Invasive Ventilation (NIV), and previous tonsillectomy and adenectomy (T&A) surgery.
Results: A total of 166 sleep studies (full polysomnography (CRSS)/overnight oxygen saturations/transcutaneous CO2 monitoring) were reviewed, for 53 children with PWS (26 males (49%)). The median age at the time of study was 3.9 (range 0.0-18.8) years. Overall, 48 (91%) of patients were on GH at time of study and 8 (15%) had previous/current NIV therapy. Univariate analysis showed BMI SDS to be significantly associated with all sleep study parameters with the exception of mean SpO2 (mixed + obstructive + hypopnoea (M/O/H) Apnoea Hypopnea Index (AHI)/hr R²=0.1, P = 0.0002; central AHI R²=0.03, P = 0.03; total AHI R²=0.04, P = 0.02, mean CO2kpa R²=0.1, P = 0.0001). Absolute IGF-1 was significantly associated with central AHI (R²=0.05, P = 0.01). Multivariate regression analysis showed an association between total AHI with central AHI and (M/O/H) AHI/hr (P < 0.0001), and GH use with IGF-1 (P = 0.0025). Patient sex correlated with mean CO2kpa (P < 0.0001).
Conclusions: SDB may be a consequence of GH therapy in children with PWS. BMI and IGF-1 are particularly associated with central AHI and other sleep parameters. Current guidance for monitoring of SDB in PWS is not clear and regular sleep screening is therefore recommended.
Volume 103
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Endocrine Abstracts
ISSN 1470-3947 (print) | ISSN 1479-6848 (online)
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