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

• Type of Material: Article
• Authors: Kristin P. Guilliams, Washington University; Fenella J. Kirkham, University College London; Susanne Holzhauer, Charite University; Steven Pavlakis, Icahn School of Medicine at Mount Sinai; Bryan Philbrook, Emory University; Catherine Amlie-Lefond, University of Washington; Michael J. Noetzel, Washington University; Nomazulu Dlamini, The Hospital for Sick Children; Mukta Sharma, University of Missouri; Jessica L. Carpenter, George Washington University; Christine K. Fox, University of California San Francisco; Marcela Torres, Cook Childrens Medical Center; Rebecca N. Ichord, University of Pennsylvania; Lori C. Jordan, Vanderbilt University; Michael D. Dowling, University of Texas Southwestern Medical Center
• Language: English
• Date: 2019-05-01
• Publisher: Lippincott Williams & Wilkins
• Publication Version: Author Accepted Manuscript (After Peer Review)
• Copyright Statement: © 2019 American Heart Association, Inc.
• Final Published Version (URL): http://dx.doi.org/10.1161/STROKEAHA.118.022800
• Title of Journal or Parent Work: Stroke
• Volume: 50
• Issue: 5
• Start Page: 1089
• End Page: 1094
• Grant/Funding Information: Research was supported by The Auxilium Foundation and NIH (KPG K23 NS099472)
• Keywords: aspirin; Neurosciences & Neurology; Children; Clinical Neurology; child; Science & Technology; Transfusion; headache; Anemia; Life Sciences & Biomedicine; risk factor; Risk factors; Cardiovascular System & Cardiology; Silent cerebral infarcts; heparin; Peripheral Vascular Disease; Stenosis
• Research Categories: Biology, Neuroscience; Health Sciences, Oncology; Health Sciences, Human Development; Biology, Cell

Abstract

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.