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PEDIATRICS is the official journal of the American Academy of Pediatrics. A monthly publication, it has been published continuously since 1948. PEDIATRICS is owned, published, and trademarked by the American Academy of Pediatrics, 141 Northwest Point Boulevard, Elk Grove Village, Illinois, 60007. Copyright © 2009 by the American Academy ofPediatrics. All rights reserved. Print ISSN: 0031-4005. Online ISSN: 1098-4275.
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Recognition of a Kawasaki Disease Shock Syndrome
John T. Kanegaye, MDa,b, Matthew S. Wilder, MDa, Delaram Molkara, MDa,c, Jeffrey R. Frazer, MDa,c, Joan Pancheri, RN, BSN, CCRCd, Adriana H. Tremoulet, MDa,e, Virginia E. Watson, MDa, Brookie M. Best,PharmD, MASa,f, Jane C. Burns, MDa,g Department of Pediatrics, School of Medicine, and fDepartment of Pharmacy, Skaggs School of Pharmacy and Pharmaceutical Sciences, University of California, San Diego, La Jolla, California; Divisions of bEmergency Medicine, cCardiology, eInfectious Diseases, and gAllergy, Immunology, and Rheumatology, and dCenter for Pediatric Clinical Research, Rady Children’sHospital San Diego, San Diego, California
The authors have indicated they have no ﬁnancial relationships relevant to this article to disclose.
What’s Known on This Subject
Although the cardiac complications of KD are well known, hemodynamic instability is unusual in the acute phase of illness, except as a complication of intravenous IVIG administration.
What This Study Adds
We haveobserved shock and hypotension with increasing frequency in newly diagnosed KD. Compared with hemodynamically normal KD, KDSS is associated with increased inﬂammation, platelet consumption, IVIG resistance, coronary artery abnormalities, mitral regurgitation, and myocardial dysfunction.
OBJECTIVE. We sought to deﬁne the characteristics that distinguish Kawasaki disease shock syndromefrom hemodynamically normal Kawasaki disease. METHODS. We collected data prospectively for all patients with Kawasaki disease who
www.pediatrics.org/cgi/doi/10.1542/ peds.2008-1871 doi:10.1542/peds.2008-1871
Key Words Kawasaki disease (mucocutaneous lymph node syndrome), shock, echocardiography, ventricular function Abbreviations KD—Kawasaki disease IVIG—intravenous immunoglobulin KDSS—Kawasakidisease shock syndrome
were treated at a single institution during a 4-year period. We deﬁned Kawasaki disease shock syndrome on the basis of systolic hypotension for age, a sustained decrease in systolic blood pressure from baseline of 20%, or clinical signs of poor perfusion. We compared clinical and laboratory features, coronary artery measurements, and responses to therapy and analyzed indicesof ventricular systolic and diastolic function during acute and convalescent Kawasaki disease.
RESULTS. Of 187 consecutive patients with Kawasaki disease, 13 (7%) met the deﬁni-
tion for Kawasaki disease shock syndrome. All received ﬂuid resuscitation, and 7 Accepted for publication Jan 12, 2009 (54%) required vasoactive infusions. Compared with patients without shock, paAddresscorrespondence to John T. Kanegaye, tients with Kawasaki disease shock syndrome were more often female and had larger MD, Division of Emergency Medicine, Rady Children’s Hospital San Diego, 3020 Children’s proportions of bands, higher C-reactive protein concentrations, and lower hemogloWay, MC 5075, San Diego, CA 92123-4282. bin concentrations and platelet counts. Evidence of consumptive coagulopathy was...