About this item:

65 Views | 31 Downloads

Author Notes:

Correspondence: Kristin P. Guilliams MD, Departments of Neurology and Pediatrics, Washington University School of Medicine, 660 S Euclid Ave Box 8111, St. Louis, MO 63112, Fax: 314-454-2523, Telephone: 314-454-6120, kristinguilliams@wustl.edu, Twitter: @kidsstroke2

The authors would like to acknowledge all IPSS investigators who contributed to the database, and Alexandra Linds for her coordination and organizational support of IPSS.

Disclosures: KPG receives support from NIH (K23 NS099472) to investigate mechanisms of stroke risk in children with sickle cell disease CKF receives support from NIH (KL2TR000143), Pediatric Epilepsy Research Foundation, and UCSF Benioff Children’s Hospital Pediatric Stroke Research Program to investigate pediatric stroke.

LCJ (R01NS096127) receives support from NIH to investigate mechanisms of stroke risk in children with sickle cell disease.


Research Funding:

Research was supported by The Auxilium Foundation and NIH (KPG K23 NS099472)


  • Science & Technology
  • Life Sciences & Biomedicine
  • Clinical Neurology
  • Peripheral Vascular Disease
  • Neurosciences & Neurology
  • Cardiovascular System & Cardiology
  • aspirin
  • child
  • headache
  • heparin
  • risk factor
  • Silent cerebral infarcts
  • Risk factors
  • Children
  • Anemia
  • Transfusion
  • Stenosis

Arteriopathy Influences Pediatric Ischemic Stroke Presentation, but Sickle Cell Disease Influences Stroke Management

Show all authors Show less authors


Journal Title:



Volume 50, Number 5


, Pages 1089-1094

Type of Work:

Article | Post-print: After Peer Review


Background and Purpose: Sickle cell disease (SCD) and arteriopathy are pediatric stroke risk factors that are not mutually exclusive. The relative contributions of sickled red blood cells and arteriopathy to stroke risk are unknown, resulting in unclear guidelines for primary and secondary stroke prevention when both risk factors are present. We hypothesized that despite similarities in clinical presentation and radiographic appearance of arteriopathies, stroke evaluation and management differ in children with SCD compared with those without SCD. Methods: We compared presentation and management of children with and without SCD enrolled in the IPSS (International Pediatric Stroke Study) with acute arterial ischemic stroke, according to SCD and arteriopathy status. Regression modeling determined relative contribution of SCD and arteriopathy in variables with significant frequency differences. Results: Among 930 childhood arterial ischemic strokes, there were 98 children with SCD, 67 of whom had arteriopathy, and 466 without SCD, 392 of whom had arteriopathy. Arteriopathy, regardless of SCD status, increased likelihood of hemiparesis (odds ratio [OR], 1.94; 95% CI, 1.46-2.56) and speech abnormalities (OR, 1.67; 95% CI, 1.29-2.19). Arteriopathy also increased likelihood of headache but only among those without SCD (OR, 1.89; 95% CI, 1.40-2.55). Echocardiograms were less frequently obtained in children with SCD (OR, 0.58; 95% CI, 0.37-0.93), but the frequency of identified cardiac abnormalities was similar in both groups (P=0.57). Children with SCD were less likely to receive antithrombotic therapy, even in the presence of arteriopathy (OR, 0.14; 95% CI, 0.08-0.22). Arteriopathy was associated with a significantly higher likelihood of antithrombotic therapy in children without SCD (OR, 5.36; 95% CI, 3.55-8.09). Conclusions: Arteriopathy, and not SCD status, was most influential of stroke presentation. However, SCD status influenced stroke management because children with SCD were less likely to have echocardiograms or receive antithrombotic therapy. Further work is needed to determine whether management differences are warranted.

Copyright information:

© 2019 American Heart Association, Inc.

Export to EndNote