Management of coronary artery aneurysms using abciximab in children with Kawasaki disease Evangelia Bachlava a , Sophia Loukopoulou a , Evangelos Karanasios b , George Chrousos a , Athanasios Michos a, ⁎ ,1 a First Department of Pediatrics, National and Kapodistrian University of Athens, “Aghia Sophia” Children's Hospital, Athens, Greece b Department of Cardiology, “Aghia Sophia” Children's Hospital, Athens, Greece abstract article info Article history: Received 18 April 2016 Accepted 19 June 2016 Available online 20 June 2016 Introduction: There are limited data regarding the possible benefits of abciximab in children with Kawasaki dis- ease (KD), who developed serious cardiac abnormalities non-responsive to standard treatment. Materials and methods: We retrospectively identified children with KD who were treated with abciximab from 2007 to 2015. Data regarding clinical course, treatment, echocardiographic data and follow-up at 1 and 6 months were retrieved. Results: During the study period, fifteen children were identified who were diagnosed with KD and were given abciximab. The median age at onset of symptoms was 11 months (range: 2 months–6 years). The median day of disease at admission was 10 days (range: 4–26 days) and the median day of administration of abciximab was 17 days (range: 9–40). Twelve children were diagnosed with complete and three with incomplete KD. An- eurysms were found in 8 children: 2 had ectatic coronary arteries and 5 presented with both ectasia and aneu- rysms. At 1 month follow-up, echocardiographic findings showed regression in the size of aneurysms in 11 children, resolution of the aneurysms or ectasia of coronary arteries in 3 children, while one child who could not take aspirin because of G6PD deficiency died. After 6 months of follow-up, echocardiographic findings showed resolution of coronary abnormalities in 12 (80%) children, whereas 2 children (13.3%) presented with significant regression of aneurysms. Conclusions: Abciximab may have an important role in the management of severe cardiac complications of KD, although prospective randomized controlled studies are needed to fully evaluate its role. © 2016 Published by Elsevier Ireland Ltd. Keywords: Kawasaki Abciximab Coronary Aneurysms Children Complication 1. Introduction Kawasaki disease (KD) is an acute systemic vasculitis that occurs predominantly in infants and young children. Although first reported in 1967 by T. Kawasaki, and the clinical symptoms of KD are well char- acterized, the causes and the pathogenesis of this disease are not yet known [1]. The most significant abnormality that results from acute Ka- wasaki disease is the development of coronary artery changes. If un- treated, approximately 15–25% of children develop coronary artery aneurysms, which may lead to myocardial infarction and death [2–4]. Patients who develop large coronary artery aneurysms have the greatest tendency to form thrombi within these aneurysms that could rupture, despite receiving standard lege artis treatment [1,5,6]. There have been cases of sudden death from myocardial infarction from a few months to many years after KD in young adults attributed to un- treated KD in childhood [7]. The American Heart Association currently recommends treatment with a single, high dose of intravenous globulin and high-dose aspirin during the acute phase of the illness [4]. This treatment regimen has re- duced the incidence of coronary artery aneurysms and ectasia to ap- proximately 5% [8]. Approximately 10–15% of patients with KD fail to respond to an ini- tial single dose of intravenous gamma globulin (IVIG) and oral aspirin, with persistent fever ≥ 36 h after completion of the initial IVIG infusion or after an initial response and a short period of being afebrile become febrile again [9–11]. Non-responders to initial therapy remain a chal- lenge, though several other additional treatments have been suggested [4,12]. These include the administration of a second dose of IVIG and corticosteroids or in children who fail multiple doses of IVIG and gluco- corticoids, may be considered for treatment with infliximab, cyclospor- ine, methotrexate or abciximab, even though the effectiveness of these agents is not well studied [4,12]. Abciximab, a platelet glycoprotein IIb/IIIa receptor inhibitor, has been shown to prevent thrombotic complications and promote vascular remodeling [5,13]. So far, limited data have been published regarding International Journal of Cardiology 220 (2016) 65–69 ⁎ Corresponding author at: First Department of Pediatrics, National and Kapodistrian University of Athens, Division of Infectious Diseases, “Aghia Sophia” Children's Hospital, Athens, Greece. E-mail address: amichos@med.uoa.gr (A. Michos). 1 Address: First Department of Pediatrics, National and Kapodistrian University of Athens Medical School, Division of Infectious Diseases, “Aghia Sophia” Children's Hospital, Thivon and Levadias Str., Goudi, 11526 Athens, Greece. http://dx.doi.org/10.1016/j.ijcard.2016.06.062 0167-5273/© 2016 Published by Elsevier Ireland Ltd. Contents lists available at ScienceDirect International Journal of Cardiology journal homepage: www.elsevier.com/locate/ijcard