Division of Pediatric Hematology/Oncology, Nationwide Children's Hospital, The Ohio State University, Columbus, Ohio, USA.
Biostatistics Resource at Nationwide Children's Hospital, Columbus, Ohio, USA.
Pediatr Hematol Oncol. 2023;40(5):433-445. doi: 10.1080/08880018.2023.2166634. Epub 2023 Jan 16.
Patients with sickle cell disease (SCD) have a high risk for venous thromboembolism which is associated with increased risk of mortality. Studies examining risk of pulmonary embolism (PE) in children with SCD are lacking. This study was conducted in children with SCD between 0-21 years of age using a nationwide administrative database in the United States- Pediatric Health Information System (PHIS) from January 2010 to June 2021. Diagnostic codes and imaging, procedure, and pharmaceutical billing codes were used to identify PE and potential clinical, demographic, and utilization risk factors. Logistic regression analyses were performed to assess association between risk factors and PE. We identified 22,631 unique patients with SCD with a median age of 10.8 years (range: <0.1-20.9). A total of 120 (0.53%) patients developed a PE with median age of 17.4 years (range: 6.6-20.9) at PE diagnosis. Patients with PE had longer hospitalization and more frequent ICU admissions than patients without PE ( < 0.001). Risk factors significantly associated with PE on multivariable analysis included older age, prior history of central venous line (CVL), acute chest syndrome, and apheresis. Mortality was not significantly different between those with and without PE. The prevalence of PE in hospitalized children with SCD was estimated to be 0.53%. Patients with PE had higher healthcare utilization characteristics. Factors significantly associated with PE suggest that the risk for PE in SCD may be related to the severity of disease state. Future trials are needed for risk stratification and PE prevention strategies in children with SCD.
镰状细胞病（SCD）患者发生静脉血栓栓塞的风险很高，这与死亡率增加有关。目前缺乏研究评估 SCD 儿童发生肺栓塞（PE）的风险。本研究在美国全国性管理数据库-儿科健康信息系统（PHIS）中纳入了 2010 年 1 月至 2021 年 6 月期间 0-21 岁的 SCD 患儿，使用诊断代码和影像学、程序及药物计费代码来识别 PE 和潜在的临床、人口统计学和利用风险因素。采用逻辑回归分析评估了危险因素与 PE 之间的关联。共纳入了 22631 例具有独特 SCD 特征的患者，其年龄中位数为 10.8 岁（范围：＜0.1-20.9）。共有 120 例（0.53%）患者发生了 PE，PE 诊断时的年龄中位数为 17.4 岁（范围：6.6-20.9）。与无 PE 患者相比，PE 患者的住院时间更长，更频繁地入住 ICU（ < 0.001）。多变量分析显示，PE 的显著危险因素包括年龄较大、既往中央静脉置管（CVL）史、急性胸部综合征和血浆置换。PE 组和非 PE 组患者的死亡率无显著差异。住院 SCD 患儿中 PE 的患病率估计为 0.53%。PE 患者的医疗保健利用特征更高。与 PE 显著相关的因素表明，SCD 中 PE 的风险可能与疾病严重程度有关。未来需要进行风险分层和 SCD 患儿 PE 预防策略的临床试验。
