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Sleep disordered breathing in patients with Prader-Willi syndrome: A multicenter study
Martino Pavone1, Valeria Caldarelli1,2, Sonia Khirani3,4
1Respiratory Unit, Bambino Ges, ù, Children's Hospital, Rome, Italy.
Pediatric Pulmonology
|April 9, 2015
Summary
Sleep disordered breathing (SDB) is common in patients with Prader-Willi syndrome (PWS). Hypotonia and facial features, not obesity, likely drive SDB in PWS patients, necessitating screening before growth hormone therapy.
Area of Science:
- Medical Research
- Genetics
- Pulmonology
Background:
- Sleep disordered breathing (SDB) is prevalent in Prader-Willi syndrome (PWS).
- Systematic screening for SDB is recommended, particularly before initiating growth hormone treatment in PWS patients.
- Understanding SDB's characteristics and management in PWS is crucial.
Purpose of the Study:
- To describe the baseline SDB.
- To outline therapeutic interventions for SDB.
- To analyze SDB in a large cohort of PWS patients.
Main Methods:
- Retrospective study design.
- Analysis of anthropometrics, polygraphy (PG), and gas exchange data.
- Inclusion of 88 PWS patients from three centers.
Main Results:
- High prevalence of SDB observed, with apnea-hypopnea index (AHI) ≥ 1.5 in 53% of children and ≥ 5 in 41% of adults.
- Mixed-obstructive apnea-hypopnea index (MOAHI) correlated significantly with SpO2 indexes, but not with anthropometrics (age, BMI, BMI z-score).
- Therapeutic interventions included growth hormone initiation (48 patients), upper airway surgery (9 patients), and noninvasive ventilation (16 patients).
Conclusions:
- Prader-Willi syndrome patients exhibit a high prevalence of SDB.
- The absence of a correlation between obesity and SDB suggests hypotonia and facial dysmorphic features are key contributors.
- Early SDB screening and management are vital for PWS patients, especially before growth hormone therapy.
Keywords:
Prader-Willi syndromegrowth hormone treatmentnoninvasive ventilationpolygraphysleep disordered breathingupper airway surgeryMore Related Videos
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