Prevalence and Risk Factors for Pulmonary Embolism in Pediatric Sickle Cell Disease: A National Administrative Database Study

Abstract

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 (p < 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.

Keywords:

• Child
• prevalence
• pulmonary embolism
• risk factors
• sickle cell anemia

Author contributions

All authors contributed to the study concept. NB and JS designed and performed the study and analyzed the data. NB wrote the manuscript which was critically reviewed by JS, VR and AV.

Disclosure statement

The authors have no relationships to disclose and no competing interests to declare.

Additional information

Funding

The author(s) reported there is no funding associated with the work featured in this article.