ABSTRACT
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. Overall, although there are many areas that need further research, sleep disorders remain a fruitful target for improving quality of life of children with PWS and their families.
ABSTRACT
Background 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. Methods We retrospectively analyzed the impact of electronic CDS on the management of patients with known adrenal insufficiency (AI) presenting to two pediatric ED locations over a 19-month period with symptoms suggestive of adrenal crisis. Outcome variables assessed included the frequency of hydrocortisone (HC) administration, appropriateness of HC dosing, and timing to HC order placement and administration. Results A total of 145 encounters were reviewed. When the electronic CDS was in place at the time of the ED visit, patients were nearly 3 times as likely to receive HC (p = 0.002). Among those patients who received HC, the presence of the CDS increased the likelihood of an appropriate 50-mg/m2 dose of HC being given from 20 to 53% (p = 0.02). However, the CDS did not significantly reduce the time from ED arrival to HC order placement (p = 0.36) or administration (p = 0.59). Conclusions The use of innovative health IT strategies, such as the electronic CDS, can improve the recognition and management of adrenal crisis among patients with AI presenting to the pediatric ED.