Children with Prader-Willi syndrome (PWS) face a multitude of potential health challenges including life-threatening obesity, endocrinopathies, behavioral and emotional dysregulation, developmental delays, and sleep disorders. In the current perspective piece, we provide a focused review of the condition's etiology and clinical findings, as well as a more in-depth discussion of sleep disorders frequently associated with PWS. In particular, we highlight and discuss difficult clinical scenarios frequently encountered by the pediatric sleep physician caring for this patient population, including diagnosis and treatment of complex sleep-related breathing disorders, considerations for sleep apnea surgery, the interplay between growth hormone and sleep apnea, diagnostic challenges in hypersomnia/narcolepsy, and current and emerging therapies for hypersomnia/narcolepsy.
View Article and Find Full Text PDFBackground Many barriers exist to the appropriate recognition and management of life-threatening adrenal crisis in the emergency department (ED). Clinical decision support (CDS) is a health information technology (IT) component that provides useful information to providers as healthcare is being delivered. We hypothesized that CDS incorporated into the electronic health record (EHR) could improve the recognition and management of adrenal crisis within the pediatric ED.
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