Luccarelli James, Strong Theresa V, Rubin Emily B, McCoy Thomas H
Massachusetts General Hospital, Boston, Massachusetts, USA.
Harvard Medical School, Boston, Massachusetts, USA.
Am J Med Genet A. 2025 May;197(5):e63980. doi: 10.1002/ajmg.a.63980. Epub 2025 Jan 11.
Prader-Willi syndrome (PWS) is a genetic disorder associated with baseline respiratory impairment caused by multiple contributing etiologies. While this may be expected to increase the risk of severe COVID-19 infections in PWS patients, survey studies have suggested paradoxically low disease severity. To better characterize the course of COVID-19 infection in patients with PWS, this study analyses the outcomes of hospitalizations for COVID-19 among patients with and without PWS. The National Inpatient Sample, an all-payors administrative claims database of hospitalizations in the United States, was queried for patients with a coded diagnosis COVID-19 in 2020 and 2021. Hospitalizations for patients with PWS compared to those for patients without PWS using Augmented Inverse Propensity Weighting (AIPW). There were 295 (95% CI: 228-362) COVID-19 hospitalizations for individuals with PWS and 4,112,400 (95% CI: 4,051,497-4,173,303) for individuals without PWS. PWS patients had a median age of 33 years compared to 63 for those without PWS. Individuals with PWS had higher baseline rates of obesity (47.5% vs. 28.4%). AIPW models show that PWS diagnosis is associated with increased hospital length of stay by 7.43 days, hospital charges by $80,126, and the odds of mechanical ventilation and in-hospital death (odds ratios of 1.79 and 1.67, respectively). PWS patients hospitalized with COVID-19 experienced longer hospital stays, higher charges, and increased risk of mechanical ventilation and death. These results suggest that PWS should be considered a risk factor for severe COVID-19, warranting continued protective measures and vaccination efforts. Further research is needed to validate coding for PWS and assess the impact of evolving COVID-19 variants and population immunity on this vulnerable population.
普拉德-威利综合征（PWS）是一种遗传性疾病，与多种病因导致的基线呼吸功能损害有关。虽然这可能会增加PWS患者发生严重新冠病毒感染的风险，但调查研究却显示疾病严重程度出人意料地低。为了更好地描述PWS患者的新冠病毒感染病程，本研究分析了有和没有PWS的患者因新冠病毒感染住院的结局。利用增强逆概率加权法（AIPW），将美国全国住院患者样本（一个涵盖所有支付方的住院行政索赔数据库）中2020年和2021年编码诊断为新冠病毒感染的患者进行了比较。有PWS的患者与没有PWS的患者的住院情况进行了对比。有PWS的个体有295例新冠病毒感染住院病例（95%置信区间：228 - 362），没有PWS的个体有4,112,400例（95%置信区间：4,051,497 - 4,173,303）。PWS患者的中位年龄为33岁，而没有PWS的患者为63岁。有PWS的个体肥胖基线率更高（47.5%对28.4%）。AIPW模型显示，PWS诊断与住院时间延长7.43天、住院费用增加80,126美元以及机械通气和院内死亡几率增加相关（优势比分别为1.79和1.67）。因新冠病毒感染住院的PWS患者住院时间更长、费用更高，机械通气和死亡风险增加。这些结果表明，PWS应被视为严重新冠病毒感染的一个风险因素，需要持续采取保护措施和开展疫苗接种工作。需要进一步研究以验证PWS的编码，并评估不断演变的新冠病毒变异株和群体免疫对这一弱势群体的影响。
