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Prevalence and Risk Factors for Pulmonary Embolism in Pediatric Sickle Cell Disease: A National Administrative
Natasha Bala1, Joseph Stanek1,2, Vilmarie Rodriguez1
1Division of Pediatric Hematology/Oncology, Nationwide Children's Hospital, The Ohio State University, Columbus, Ohio, USA.
Insights
Pulmonary embolism (PE) affects 0.53% of children with sickle cell disease (SCD), with older age and disease severity factors increasing risk. Further research is needed for PE prevention strategies in pediatric SCD patients.
Area of Science:
- Pediatric Hematology
- Cardiovascular Research
- Thrombosis and Hemostasis
Background:
- Sickle cell disease (SCD) patients face elevated risks of venous thromboembolism and mortality.
- Limited research exists on pulmonary embolism (PE) risk in pediatric SCD populations.
Purpose of the Study:
- To investigate the prevalence and risk factors of pulmonary embolism (PE) in children diagnosed with sickle cell disease (SCD).
Main Methods:
- Utilized the Pediatric Health Information System (PHIS) database (2010-2021) for nationwide US data.
- Identified PE and risk factors using diagnostic, imaging, procedure, and pharmaceutical billing codes.
- Employed logistic regression to analyze associations between risk factors and PE occurrence.
Main Results:
- Identified 22,631 pediatric SCD patients; 120 (0.53%) developed PE.
- PE occurred at a median age of 17.4 years, associated with longer hospital stays and ICU admissions.
- Significant PE risk factors included older age, central venous line history, acute chest syndrome, and apheresis.
Conclusions:
- The prevalence of PE in hospitalized children with SCD is 0.53%, with higher healthcare utilization observed in affected patients.
- Identified risk factors suggest PE risk in pediatric SCD is linked to disease severity.
- Further investigation is warranted for PE risk stratification and prevention in this vulnerable group.
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.
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