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Purpose <p>Prader-Willi syndrome (PWS) is a rare genetic disorder with complex medical and psychiatric manifestations that may necessitate emergency department (ED) care. This study characterizes ED presentations among patients with PWS using a nationally representative cohort of ED presentations in the United States.</p> Methods <p>The National Emergency Department Sample (NEDS) was queried for patients with a coded diagnosis of PWS from Oct 2019 through Dec 2021. ED presentations for patients with PWS were matched to 5 non-PWS presentations based on age, sex, year, and hospital characteristics.</p> Results <p>Over 27 months there were 8,100 (95% CI 6,759 to 9,441) ED presentations for patients with PWS, or approximately 1 in 35,000 overall ED presentations. PWS patients were predominantly young (median age: 23 years), with notable comorbidities including septicemia, respiratory failure, and psychiatric conditions such as agitation and impulse-control disorders. Compared to matched control patients, PWS patients had higher odds of hospital admission (43.3% vs. 11.9%; adjusted odds ratio [aOR]: 6.0) and death (1.6% vs. 0.1%; aOR: 11.2) during their hospital encounter.</p> Conclusions <p>Individuals with PWS present to the emergency department with numerous medical and psychiatric complexities resulting in greater clinical complexity and mortality than matched control presentations. The rarity of PWS in the ED likely implies limited provider familiarity, which may exacerbate challenges in care delivery. These findings emphasize the need for increased awareness and education regarding PWS among emergency care providers and identify best practices for addressing the acute care needs of this vulnerable population.</p>

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Emergency Department Care for Patients With Prader-Willi Syndrome: A 2019–2021 National Emergency Department Sample Analysis

  • 期刊论文

  • 发表日期：

    2026年3月26日

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  • James Luccarelli,
  • Theresa V. Strong,
  • Thomas H. McCoy

摘要

Purpose

Prader-Willi syndrome (PWS) is a rare genetic disorder with complex medical and psychiatric manifestations that may necessitate emergency department (ED) care. This study characterizes ED presentations among patients with PWS using a nationally representative cohort of ED presentations in the United States.

Methods

The National Emergency Department Sample (NEDS) was queried for patients with a coded diagnosis of PWS from Oct 2019 through Dec 2021. ED presentations for patients with PWS were matched to 5 non-PWS presentations based on age, sex, year, and hospital characteristics.

Results

Over 27 months there were 8,100 (95% CI 6,759 to 9,441) ED presentations for patients with PWS, or approximately 1 in 35,000 overall ED presentations. PWS patients were predominantly young (median age: 23 years), with notable comorbidities including septicemia, respiratory failure, and psychiatric conditions such as agitation and impulse-control disorders. Compared to matched control patients, PWS patients had higher odds of hospital admission (43.3% vs. 11.9%; adjusted odds ratio [aOR]: 6.0) and death (1.6% vs. 0.1%; aOR: 11.2) during their hospital encounter.

Conclusions

Individuals with PWS present to the emergency department with numerous medical and psychiatric complexities resulting in greater clinical complexity and mortality than matched control presentations. The rarity of PWS in the ED likely implies limited provider familiarity, which may exacerbate challenges in care delivery. These findings emphasize the need for increased awareness and education regarding PWS among emergency care providers and identify best practices for addressing the acute care needs of this vulnerable population.