Исследовательский журнал клинической педиатрии

Sleep Disordered Breathing in Prader-Willi Syndrome: A Review

Jennifer F Ha

Objectives: PWS is a rare genetic disorder characterized by cognitive impairment, hypogonadism, morbid obesity due to hyperphagia and lack of satiety, and hypothalamic dysfunction. PWS is closely associated with SDB in many forms.

Data sources: A literature search was conducted on the PubMed, MedLine, CINAHL, Embase, Web of Science and Google Scholar databases based on the keywords “Prader Willi Syndrome”, “sleep disordered breathing”, “obstructive sleep apnea”.

Review method: Narrative review of the current literature.

Results: GH therapy was approved in 2000 for treatment of PWS and has been successful in promoting linear growth and improving muscular trophism and tone, with a consequent improvement in strength, physical activity and cardiorespiratory function as well as SDB. However it is not without its complications, in particular sudden deaths. Patients should be managed in the multidisciplinary team with regular polysomnogram especially in the first few weeks following initiation of the treatment. There are other conservative options that can be considered for persistence of SDB despite GH as well as surgical options.

Conclusion: PWS is a rare disorder associated with a variety of SDB. GH has the potential to positively impact on the frequency and severity of SDB in these children. Therefore, they will benefit from careful monitoring in a multidisciplinary setting.